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International Classification of Diseases for Oncology
International Classification of Diseases for Oncology
from Wikipedia

The International Classification of Diseases for Oncology (ICD-O) is a domain-specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. This classification is widely used by cancer registries.

It is currently in its third revision (ICD-O-3). ICD-10 includes a list of morphology codes. They stem from ICD-O second edition (ICD-O-2) that was valid at the time of publication.

Axes

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The classification has two axes: topography and morphology.

Morphology

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The morphology axis addresses the microscopic structure (histology) of the tumor.

This axis has particular importance because the Systematized Nomenclature of Medicine ("SNOMED") has adopted the ICD-O classification of morphology. SNOMED has been changing continuously, and several different versions of SNOMED are in use. Accordingly, mapping of ICD-O codes to SNOMED requires careful assessment of whether entities are indeed true matches.

Topography

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The topography axis addresses the tumor's site in the body. It is standardized with the C section of ICD-10.

There were no changes in the topography axis between ICD-O-2 and ICD-O-3.

See List of ICD-10 codes#(C00–C97) Malignant Neoplasms for examples.

International Classification of Diseases for Oncology, Third Edition (ICD-O-3)

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5th Digit Behaviour Code for Neoplasms

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Morphology Codes (ICD-O-3)

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Source:[1]

800 Neoplasms, NOS

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M8000/0 Neoplasm, benign

M8000/1 Neoplasm, uncertain whether benign or malignant

  • Neoplasm, NOS
  • Tumor, NOS
  • Unclassified tumor, uncertain whether benign or malignant
  • Unclassified tumor, borderline malignancy

M8000/3 Neoplasm, malignant

  • Tumor, malignant, NOS
  • Malignancy
  • Cancer
  • Unclassified tumor, malignant
  • Blastoma, NOS

M8000/6 Neoplasm, metastatic

  • Neoplasm, metastatic
  • Tumor, metastatic
  • Tumor, secondary
  • Tumor embolus

M8000/9 Neoplasm, malignant, uncertain whether primary or metastatic

  • Unclassified tumor, malignant, uncertain whether primary or metastatic

M8001/0 Tumor cells, benign

M8001/1 Tumor cells, uncertain whether benign or malignant

  • Tumor cells, NOS

M8001/3 Tumor cells, malignant

M8002/3 Malignant tumor, small cell type

M8003/3 Malignant tumor, giant cell type

M8004/3 Malignant tumor, spindle cell type

  • Malignant tumor, fusiform cell type

M8005/0 Clear cell tumor, NOS

M8005/3 Malignant tumor, clear cell type

801–804 Epithelial Neoplasms, NOS

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805–808 Squamous Cell Neoplasms

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  • M8050/0 Papilloma, NOS (except papilloma of bladder M8120/1)
  • M8050/2 Papillary carcinoma in situ
  • M8050/3 Papillary carcinoma, NOS
  • M8051/0 Verrucous papilloma
  • M8051/3 Verrucous carcinoma, NOS
    • Condylomatous carcinoma
    • Verrucous squamous cell carcinoma
    • Verrucous epidermoid carcinoma
    • Warty carcinoma
  • M8052/0 Squamous cell papilloma, NOS
    • Squamous papilloma
    • Keratotic papilloma
  • M8052/2 Papillary squamous cell carcinoma, non-invasive
    • Papillary squamous cell carcinoma in situ
  • M8052/3 Papillary squamous cell carcinoma
    • Papillary epidermoid carcinoma
  • M8053/0 Squamous cell papilloma, inverted
  • M8060/0 Squamous papillomatosis
    • Papillomatosis, NOS
  • M8070/2 Squamous cell carcinoma in situ, NOS
    • Epidermoid carcinoma in situ, NOS
    • Intraepidermal carcinoma, NOS
    • Intraepithelial squamous cell carcinoma
  • M8070/3 Squamous cell carcinoma, NOS
    • Epidermoid carcinoma, NOS
    • Squamous carcinoma
    • Squamous cell epithelioma
  • M8070/6 Squamous cell carcinoma, metastatic, NOS
  • M8071/3 Squamous cell carcinoma, keratinizing, NOS
    • Squamous cell carcinoma, large cell, keratinizing
    • Epidermoid carcinoma, keratinizing
  • M8072/3 Squamous cell carcinoma, large cell, nonkeratinizing, NOS
    • Squamous cell carcinoma, non keratinizing, NOS
    • Epidermoid carcinoma, large cell, nonkeratinizing
  • M8073/3 Squamous cell carcinoma, small cell, nonkeratinizing
    • Epidermoid carcinoma, small cell, nonkeratinizing
  • M8074/3 Squamous cell carcinoma, spindle cell
    • Epidermoid carcinoma, spindle cell
    • Squamous cell carcinoma, sarcomatoid
  • M8075/3 Squamous cell carcinoma, adenoid
    • Squamous cell carcinoma, pseudoglandular
    • Squamous cell carcinoma, acantholytic
  • M8076/2 Squamous cell carcinoma in situ with questionable stromal invasion
    • Epidermoid carcinoma in situ with questionable stromal invasion
  • M8076/3 Squamous cell carcinoma, microinvasive
  • M8077/2 Squamous intraepithelial neoplasia, grade III
    • Cervical intraepithelial neoplasia, grade III (C53._)
      • CIN III, NOS (C53._)
      • CIN III with severe dysplasia (C53._)
    • Vaginal intraepithelial neoplasia, grade III (C52._)
      • VAIN III (C52._)
    • Vulvar intraepithelial neoplasia, grade III (C51._)
      • VIN III (C51._)
    • Anal intraepithelial neoplasia, grade III (C21.1)
      • AIN III (C21.1)
  • M8078/3 Squamous cell carcinoma with horn formation
  • M8080/2 Queyrat erythroplasia (C60._)
  • M8081/2 Bowen disease (C44._)
    • Intraepidermal squamous cell carcinoma, Bowen type (C44._)
  • M8082/3 Lymphoepithelial carcinoma
    • Lymphoepithelioma
    • Lymphoepithelioma-like carcinoma
    • Schmincke tumor (C11._)
  • M8083/3 Basaloid squamous cell carcinoma
  • M8084/3 Squamous cell carcinoma, clear cell type
  • M8090/1 Basal cell tumor (C44._)
  • (M8090/3) Basal cell carcinoma, NOS (C44._)
    • Basal cell epithelioma
    • Rodent ulcer
    • Pigmented basal cell carcinoma
  • M8091/3 Multifocal superficial basal cell carcinoma (C44._)
    • Multicentric basal cell carcinoma
  • M8092/3 Infriltrating basal cell carcinoma, NOS (C44._)
    • Infiltrating basal cell carcinoma, non-sclerosing
    • Infiltrating basal cell carcinoma, sclerosing
    • Basal cell carcinoma, morphoeic
    • Basal cell carcinoma, desmoplastic type
  • M8093/3 Basal cell carcinoma, fibroepithelial (C44._)
    • Fibroepithelioma of Pinkus type
    • Fibroepithelial basal cell carcinoma, Pinkus type
    • Pinkus tumor
    • Fibroepithelioma, NOS
  • M8094/3 Basosquamous carcinoma (C44._)
    • Mixed basal-squamous cell carcinoma
  • M8095/3 Metatypical carcinoma
  • M8096/0 Intraepidermal epithelioma of Jadassohn (C44._)
  • M8097/3 Basal cell carcinoma, nodular (C44._)
    • Basal cell carcinoma, micronodular
  • M8098/3 Adenoid basal carcinoma (C53._)
  • M8100/0 Trichoepithelioma (C44._)
    • Brooke tumor
    • Epithelioma adenoides cysticum
  • M8101/0 Trichofolliculoma (C44._)
  • M8102/0 Trichilemmoma (C44._)
  • M8102/3 Trichilemmocarcinoma (C44._)
    • Trichilemmal carcinoma
  • M8103/0 Pilar tumor (C44._)
    • Proliferating trichilemmal cyst
    • Proliferating trichilemmal tumor
  • M8110/0 Pilomatrixoma, NOS (C44._)
    • Calcifying epithelioma of Malherbe
    • Pilomatricoma, NOS
  • M8110/3 Pilomatrix carcinoma (C44._)
    • Pilomatrixoma, malignant
    • Pilomatricoma, malignant
    • Matrical carcinoma
  • M8120/0 Transitional cell papilloma, benign
    • Transitional papilloma
  • M8120/1 Urothelial papilloma, NOS
    • Papilloma of bladder (C67._)
    • Transitional cell papilloma, NOS
  • M8120/2 Transitional cell carcinoma in situ
    • Urothelial carcinoma in situ
  • M8120/3 Transitional cell carcinoma, NOS
    • Urothelial carcinoma, NOS
    • Transitional carcinoma
  • M8121/0 Schneiderian papilloma, NOS (C30.0, C31._)
    • Sinonasal papilloma, NOS
    • Sinonasal papilloma, exophytic
    • Sinonasal papilloma, fungiform
    • Transitional cell papilloma, inverted, benign
    • Transitional papilloma, inverted, benign
  • M8121/1 Transitional cell papilloma, inverted, NOS
    • Transitional papilloma, inverted, NOS
    • Schneiderian papilloma, inverted
    • Columnar cell papilloma
    • Cylindrical cell papilloma
    • Oncocytic Schneiderian papilloma
  • M8121/3 Schneiderian carcinoma (C30.0, C31._)
    • Cylindrical cell carcinoma
  • M8122/3 Transitional cell carcinoma, spindle cell
    • Transitional cell carcinoma, sarcomatoid
  • M8123/3 Basaloid carcinoma
  • M8124/3 Cloacogenic carcinoma (C21.2)
  • M8130/1 Papillary transitional cell neoplasm of low malignant potential (C67._)
    • Papillary urothelial neoplasm of low malignant potential
  • M8130/2 Papillary transitional cell carcinoma, non-invasive (C67._)
    • Papillary urothelial carcinoma, non-invasive
  • M8130/3 Papillary transitional cell carcinoma (C67._)
    • Papillary urothelial carcinoma
  • M8131/3 Transitional cell carcinoma, micropapillary ( C67._)
  • (M8140/0) Adenoma, NOS
  • M8140/1 Atypical adenoma
    • Bronchial adenoma, NOS (C34._)
  • M8140/2 Adenocarcinoma in situ, NOS
  • (M8140/3) Adenocarcinoma, NOS
  • M8140/6 Adenocarcinoma, metastatic, NOS
  • M8141/3 Scirrhous adenocarcinoma
    • Scirrhous carcinoma
    • Carcinoma with productive fibrosis
  • (M8142/3) Linitis plastica (C16._)
  • M8143/3 Superficial spreading adenocarcinoma
  • M8144/3 Adenocarcinoma, intestinal type (C16._)
    • Carcinoma, intestinal type
  • M8145/3 Carcinoma, diffuse type (C16._)
    • Adenocarcinoma, diffuse type
  • M8146/0 Monomorphic adenoma
  • M8147/0 Basal cell adenoma
  • M8147/3 Basal cell adenocarcinoma
  • M8148/2 Glandular intraepithelial neoplasia, grade III
    • Prostatic intraepithelial neoplasia, grade III (C61.9)
      • PIN III
  • M8149/0 Canalicular adenoma
  • M8150/0 Islet cell adenoma (C25._)
    • Islet cell tumor, benign
    • Nesidioblastoma
    • Islet cell adenomatosis
  • M8150/1 Islet cell tumor, NOS (C25._)
  • M8150/3 Islet cell carcinoma (C25._)
    • Islet cell adenocarcinoma
  • (M8151/0) Insulinoma, NOS (C25._)
    • Beta cell adenoma
  • M8151/3 Insulinoma, malignant (C25._)
    • Beta cell tumor, malignant
  • (M8152/0) Glucagonoma, NOS (C25._)
    • Alpha cell tumor, NOS
  • M8152/3 Glucagonoma, malignant (C25._)
    • Alpha cell tumor, malignant
  • (M8153/1) Gastrinoma, NOS
    • G cell tumor, NOS
    • Gastrin cell tumor
  • M8153/3 Gastinoma, malignant
    • G cell tumor, malignant
    • Gastrin cell tumor, malignant
  • M8154/3 Mixed islet cell and exocrine adenocarcinoma (C25._)
    • Mixed acinar-endocrine carcinoma
    • Mixed ductal-endocrine carcinoma
  • M8155/1 Vipoma, NOS
  • (M8155/3) Vipoma, malignant
  • M8156/1 Somatostatinoma, NOS
    • Somatostatin cell tumor, NOS
  • M8156/3 Somatostatinoma, malignant
    • somatostatin cell tumor, malignant
  • M8157/1 Enteroglucagonoma, NOS
  • M8157/3 Enteroglucagonoma, malignant
  • M8160/0 Bile duct adenoma (C22.1, C24.0)
    • Cholangioma
  • (M8160/3) Cholangiocarcinoma (C22.1, C24.0)
    • Bile duct carcinoma
    • Bile duct adenocarcinoma
  • M8161/0 (C22.1, C24.0)
  • M8161/3 Bile duct cystadenocarcinoma (C22.1, C24.0)
  • M8162/3 Klatskin tumor (C22.1, C24.0)
  • M8170/0 Liver cell adenoma (C22.0)
  • (M8170/3) Hepatocellular carcinoma, NOS (C22.0)
    • Liver cell carcinoma
    • Hepatocarcinoma
    • Hepatoma, malignant
    • Hepatoma, NOS
  • M8171/3 Hepatocellular carcinoma, fibrolamellar (C22.0)
  • M8172/3 Hepatocellular carcinoma, scirrhous (C22.0)
    • Sclerosing hepatic carcinoma
  • M8173/3 Hepatocellular carcinoma, spindle cell variant (C22.0)
    • Hepatocellular carcinoma, sarcomatoid
  • M8174/3 Hepatocellular carcinoma, clear cell type (C22.0)
  • M8175/3 Hepatocellular carcinoma, pleomorphic type
  • M8180/3 Combined hepatocellular carcinoma and cholangiocarcinoma (C22.0)
    • Mixed hepatocellular and bilde duct carcinoma
    • Hepatocholangiocarcinoma
  • M8190/0 Trabecular adenoma
  • M8190/3 Trabecular adenocarcinoma
    • Trabecular carcinoma
  • M8191/0 Embryonal adenoma
  • M8200/0 Eccrine dermal cylindroma (C44._)
    • Turban tumor
    • Cylindroma of skin
  • (M8200/3) Adenoid cystic carcinoma
    • Adenocystic carcinoma
    • Cylindroma, NOS (except cylindroma of skin M8200/0)
    • Adenocarcinoma, cylindroid
    • Bronchial adenoma, cylindroid (C34._)
  • M8201/2 Cribiform carcinoma in situ (C50._)
    • Ductal carcinoma in situ, cribiform type
  • M8201/3 Cribiform carcinoma, NOS
    • Ductal carcinoma, cribiform type
  • M8202/0 Microcystic adenoma (C25._)
  • M8204/0 Lactating adenoma
  • M8210/0 Adenomatous polyp, NOS
    • Polypoid adenoma
  • M8210/2 Adenocarcinoma in situ in adenomatous polyp
    • Adenocarcinoma in situ in tubular adenoma
    • Carcinoma in situ in adenomatous polyp
    • Adenocarcinoma in situ in polypoid adenoma
    • Adenocarcinoma in situ in a polyp, NOS
    • Carcinoma in situ in a polyp, NOS
  • M8210/3 Adenocarcinoma in adenomatous polyp
    • Adenocarcinoma in tubular adenoma
    • Carcinoma in adenomatous polyp
    • Adenocarcinoma in polypoid adenoma
    • Adenocarcinoma in a polyp, NOS
    • Carcinoma in a polyp, NOS
  • M8211/0 Tubular adenoma, NOS
  • M8211/3 Tubular adenocarcinoma
  • M8212/0 Flat adenoma
  • M8213/0 Serrated adenoma (C18._)
    • Mixed adenomatous and hyperplastic polyp
  • M8214/3 Parietal cell carcinoma (C16._)
    • Parietal cell adenocarcinoma
  • M8215/3 Adenocarcinoma of anal glands (C21.1)
    • Adenocarcinoma of anal ducts
  • M8220/0 Adenomatous polyposis coli (C18._)
    • Familial polyposis coli
    • Adenomatosis, NOS
  • M8220/3 Adenocarcinoma in adenomatous polyposis
  • M8221/0 Multiple adenomatous polyps
  • M8221/3 Adenocarcinoma in multiple adenomatous polyps
  • M8230/2 Ductal carcinoma in situ, solid type (C50._)
    • Intraductal carcinoma, solid type
  • M8230/3 Solid carcinoma, NOS
    • Solid carcinoma with mucin formation
    • Solid adenocarcinoma with mucin formation
  • M8231/3 Carcinoma simplex
  • (M8240/1) Carcinoid tumor of uncertain malignant potential
    • Carcinoid tumor, NOS, of appendix (C18.1)
    • Carcinoid, NOS, of appendix
    • Carcinoid tumor, argentaffin, NOS
    • Argentaffinoma, NOS
  • M8240/3 Carcinoid tumor, NOS (except of appendix M8240/1)
    • Carcinoid, NOS (except of appendix)
    • Typical carcinoid
    • Bronchial adenoma, carcinoid
  • M8241/3 Enterochromaffin cell carcinoid
    • Carcinoid tumor, argentaffin, malignant
    • Argentaffinoma, malignant
    • EC cell carcinoid
    • Serotonin producing carcinoid
  • M8242/1 Enterochromaffin-like cell carcinoid, NOS
    • ECL cell carcinoid, NOS
  • M8242/3 Enterochromaffin-like cell tumor, malignant
    • ECL cell carcinoid, malignant
  • M8243/3 Goblet cell carcinoid
    • Mucocarcinoid tumor
    • Mucinous carcinoid
  • M8244/3 Composite carcinoid
    • Combined carcinoid and adenocarcinoma
    • Mixed carcinoid-adenocarcinoma
  • M8245/1 Tubular carcinoid
  • M8245/3 Adenocarcinoid tumor
  • M8246/3 Neuroendocrine carcinoma, NOS
  • M8247/3 Merkel cell carcinoma (C44._)
    • Merkel cell tumor
    • Primary cutaneous neuroendocrine carcinoma
  • M8248/1 Apudoma
  • M8249/3 Atypical carcinoid tumor
  • M8250/1 Pulmonary adenomatosis (C34._)
  • M8250/3 Bronchiolo-alveolar adenocarcinoma, NOS (C34._)
    • Bronchiolo-alveolar carcinoma, NOS
    • Bronchiolar adenocarcinoma
    • Bronchiolar carcinoma
    • Alveolar cell carcinoma
  • M8251/0 Alveolar adenoma (C34._)
  • M8251/3 Alveolar adenocarcinoma (C34._)
    • Alveolar carcinoma
  • M8252/3 Bronchiolo-alveolar carcinoma, non-mucinous (C34._)
    • Bronchiolo-alveolar carcinoma, Club cell
    • Bronchiolo-alveolar carcinoma, type II pneumocyte
  • M8253/3 Bronchiolo-alveolar carcinoma, mucinous (C32._)
    • Bronchiolo-alveolar carcinoma, goblet cell type
  • M8254/3 Bronchiolo-alveolar carcinoma, mixed mucinous and non-mucinous (C34._)
    • Bronchiolo-alveolar carcinoma, Club cell and goblet cell type
    • Bronchiolo-alveolar carcinoma, type II pneumocyte and goblet cell type
    • Bronchiolo-alveolar carcinoma, indeterminate type
  • M8255/3 Adenocarcinoma combined with mixed subtypes
    • Adenomcarcinoma combined with other types of carcinoma
  • M8260/0 Papillary adenoma, NOS
    • Glandular papilloma
  • M8260/3 Papillary adenocarcinoma, NOS
    • Papillary carcinoma of thyroid (C73.9)
    • Papillary renal cell carcinoma (C64.9)
  • M8261/0 Villous adenoma, NOS
    • Villous papilloma
  • M8261/2 Adenocarcinoma in situ in villous adenoma
  • M8261/3 Adenocarcinoma in villous adenoma
  • M8262/3 Villous adenocarcinoma
  • M8263/0 Tubulovillous adenoma, NOS
    • villoglandular adenoma
    • Papillotubular adenoma
  • M8263/2 Adenocarcinoma in situ in tubulovillous adenoma
  • M8263/3 Adenocarcinoma in tubulovillous adenoma
    • Papillotubular adenocarcinoma
    • Tubulopapillary adenocarcinoma
  • M8264/0 Papillomatosis, glandular
    • Biliary papillomatosis (C22.1, C24.0)
  • M8270/0 Chromophobe adenoma (C75.1)
  • M8270/3 Chromophobe carcinoma (C75.1)
    • Chromophobe adenocarcinoma
  • (M8271/0) Prolactinoma (C75.1)
  • M8272/0 Pituitary adenoma, NOS (C75.1)
  • M8272/3 Pituitary carcinoma, NOS (C75.1)
  • M8280/0 Acidophil adenoma (C75.1)
    • Eosinophil adenoma
  • M8280/3 Acidophil carcinoma (C75.1)
    • Acidophil adenocarcinoma
    • Eosinophil carcinoma
    • Eosinophil adenocarcinoma
  • M8281/0 Mixed acidophil-basophil adenoma (C75.1)
  • (M8290/0) Oxyphilic adenoma
  • M8290/3 Oxyphilic adenocarcinoma
    • Oncocytic carcinoma
    • Oncocytic adenocarcinoma
    • Hurthlecell carcinoma (C73.9)
    • Hurthle cell adenocarcinoma
    • Follicular carcinoma, oxyphilic cell (C73.9)
  • M8300/0 Basophil adenoma (C75.1)
    • Mucoid cell adenoma
  • M8300/3 Basophil carcinoma (C75.1)
    • Basophil adenocarcinoma
    • Mucoid cell adenocarcinoma
  • M8310/0 Clear cell adenoma
  • M8310/3 Clear cell adenocarcinoma, NOS
    • Clear cell carcinoma
    • Clear cell adenocarcinoma, mesonephroid
  • M8311/1 Hypernephroid tumor
  • (M8312/3) Renal cell carcinoma, NOS (C64.9)
  • M8313/0 Clear cell adenofibroma (C56.9)
    • clear cell cystadenofibroma
  • M8313/1 Clear cell adenofibroma of borderline malignancy
    • Clear cell cystadenofibroma of borderline malignancy
  • M8313/3 Clear cell adenocarcinofibroma (C56.9)
    • Clear cell cystadenocarcinofibroma
  • M8314/3 Lipid-rich carcinoma (C50._)
  • M8315/3 Glycogen-rich carcinoma
  • M8316/3 Cyst-associated renal cell carcinoma (C64.9)
  • M8317/3 Renal cell carcinoma, chromophobe type (C64.9)
    • Chromophobe cell renal carcinoma
  • M8318/3 Renal cell carcinoma, sarcomatoid (C64.9)
    • Renal cell carcinoma, spindle cell
  • M8319/3 collecting duct carcinoma (C64.9)
    • Bellini duct carcinoma
    • Renal carcinoma, collecting duct type
  • M8320/3 Granular cell carcinoma
    • Granular cell adenocarcinoma
  • M8321/0 Chief cell adenoma (C75.0)
  • M8322/0 Water-clear cell adenoma (C75.0)
  • M8322/3 Water-clear cell adenocarcinoma (C75.0)
    • Water-celar cell carcinoma
  • M8323/0 Mixed cell adenoma
  • M8323/3 Mixed cell adenocarcinoma
  • M8324/0 Lipoadenoma
    • Adenolipoma
  • M8325/0 Metanephric adenoma (C64.9)
  • M8330/0 Follicular adenoma (C73.9)
  • M8330/1 Atypical follicular adenoma (C73.9)
  • M8330/3 Follicular adenocarcinoma, NOS (C73.9)
    • Follicular carcinoma, NOS
  • M8331/3 Follicular adenocarcinoma, well differentiated (C73.9)
    • Follicular carcinoma, well differentiated
  • M8332/3 Follicular adenocarcinoma, trabecular (C73.9)
    • Follicular carcinoma, trabecular
    • Follicular adenocarcinoma, moderately differentiated
    • FOlloicular carcinoma, moderately differentiated
  • M8333/0 Microfollicular adenoma, NOS (C73.9)
    • Fetal adenoma
  • M8333/3 Fetal adenocarcinoma
  • M8334/0 Macrofollicular adenoma (C73.9)
    • Colloid adenoma
  • M8335/3 Follicular carcinoma, minimally invasive (C73.9)
    • Follicular carcinoma, encapsulated
  • M8336/0 Hyalinizing trabecular adenoma (C73.9)
  • (M8337/3)Insular carcinoma (C73.9)
  • M8340/3 Papillary carcinoma, follicular variant (C73.9)
    • Papillary adenocarcinoma, follicular variant
    • Papillary and follicular adenocarcinoma
    • Papillary and follicular carcinoma
  • M8341/3 Papillary microcarcinoma (C73.9)
  • M8342/3 Papillary carcinoma, oxyphilic cell (C73.9)
  • M8343/3 Papillary carcinoma, encapsulated (C73.9)
  • M8344/3 Papillary carcinoma, columnar cell (C73.9)
    • Papillary carcinoma, tall cell
  • M8345/3 Medullary carcinoma with amyloid stroma (C73.9)
    • Parafollicular cell carcinoma
    • C cell carcinoma
  • M8346/3 Mixed medullary-follicular carcinoma (C73.9)
  • M8347/3 Mixed medullary-papillary carcinoma (C73.9)
  • M8350/3 Nonencapsulated sclerosing carcinoma (C73.9)
    • Nonencapsulated sclerosing adenocarcinoma
    • Nonencapsulated sclerosing tumor
    • Papillary carcinoma, diffuse sclerosing
  • M8360/1 Multiple endocrine adenomas
    • Endocrine adenomatosis
  • M8361/0 Juxtaglomerular tumor (C64.9)
    • Reninoma
  • M8370/0 Adrenal cortical adenoma, NOS (C74.0)
    • Adrenal cortical tumor, benign
    • Adrenal cortical tumor, NOS
  • M8370/3 Adrenal cortical carcinoma (C74.0)
    • Adrenal cortical adenocarcinoma
    • Adrenal cortical tumor, malignant
  • M8371/0 Adrenal cortical adenoma, compact cell (C74.0)
  • M8372/0 Adrenal cortical adenoma, pigmented (C74.0)
    • Black adenoma
    • Pigmented adenoma
  • M8373/0 Adrenal cortical adenoma, clear cell (C74.0)
  • M8374/0 Adrenal cortical adenoma, glomerulosa cell (C74.0)
  • M8375/0 Adrenal cortical adenoma, mixed cell (C74.0)
  • (M8380/0) Endometrioid adenoma, NOS
    • Endometrioid cystadenoma, NOS
  • M8380/1 Endometrioid adenoma, borderline malignancy
    • Endometrioid cystadenoma, borderline malignancy
    • Endometrioid tumor of low malignant potential
    • Atypical proliferative endometrioid tumor
  • M8380/3 Endometrioid adenocarcinoma, NOS
    • Endometrioid carcinoma, NOS
    • Endometrioid cystadenocarcinoma
  • M8381/0 Endometrioid adenofibroma, NOS
    • Endometrioid cystadenofibroma, NOS
  • M8381/1 Endometrioid adenofibroma, borderline malignancy
    • Endometrioid cystadenofibroma, borderline malignancy
  • M8381/3 Endometriod adenofibroma, malignant
    • Endometrioid cystadenofibroma, malignant
  • M8382/3 Endometrioid adenocarcinoma, secretory variant
  • M8383/3 Endometrioid adenocarcinoma, ciliated cell variant
  • M8384/3 Adenocarcinoma, endocervical type

M8390/0 Skin appendage adenoma (C44._)

  • Skin appendage tumor, benign
  • Adnexal tumor, benign

M8390/3 Skin appendage carcinoma (C44._)

  • Adnexal carcinoma

M8391/0 Follicular fibroma (C44._)

  • Trichodiscoma
  • Fibrofolliculoma
  • Perifollicular fibroma

M8392/0 Syringofibroadenoma (C44._)

M8400/0 Sweat gland adenoma (C44._)

  • Sweat gland tumor, benign
  • Hidradenoma, NOS
  • Syringadenoma, NOS

M8400/1 Sweat gland tumor, NOS (C44._)

M8400/3 Sweat gland adenocarcinoma (C44._)

  • Sweat gland carcinoma
  • Sweat gland tumor, malignant

M8401/0 Apocrine adenoma

  • Apocrine cystadenoma

M8401/3 Apocrine adenocarcinoma

M8402/0 Nodular hidradenoma (C44._)

  • Eccrine acrospiroma
  • Clear cell hidradenoma

M8402/3 Nodular hidradenoma, malignant (C44._)

  • Hidradenocarcinoma

M8403/0 Eccrine spiradenoma (C44._)

  • Spiradenoma, NOS

M8403/3 Malignant eccrine spiradenoma (C44._) M8404/0 Hidrocystoma (C44._)

  • Eccrine cystadenoma

M8405/0 Papillary hidradenoma

  • Hidradenoma papilliferum

M8406/0 Papillary syringadenoma (C44._)

  • Papillary syringocystadenoma
  • Syringocystadenoma papilliferum

M8407/0 Syringoma, NOS (C44._)

M8407/3 Sclerosing sweat duct carcinoma (C44._)

  • Syringomatous carcinoma
  • Microcystic adnexal carcinoma

M8408/0 Eccrine papillary adenoma (C44._)

M8408/1 Aggressive digital papillary adenoma (C44._)

M8408/3 Eccrine papillary adenocarcinoma (C44._)

  • Digital papillary adenocarcinoma

M8409/0 Eccrine poroma (C44._)

M8410/0 Sebaceous adenoma (C44._)

M8410/3 Sebaceous adenocarcinoma (C44._)

  • Sebaceous carcinoma

M8413/3 Eccrine adenocarcinoma (C44._)

M8420/0 Ceruminous adenoma (C44.2)

M8420/3 Ceruminous adenocarcinioma (C44.2)

  • Ceruminous carcinoma

M8430/1 Mucoepidermoid tumor

M8430/3 Mucoepidermoid carcinoma

M8440/0 Cystadenoma, NOS

  • Cystoma, NOS

M8440/3 Cystadenocarcinoma, NOS

M8441/0 Serous cystadenoma, NOS

  • Serous cystoma
  • Serous microcystic adenoma

M8441/3 Serous cystadenocarcinoma, NOS (C56.9)

  • Serous adenocarcinoma, NOS
  • Serous carcinoma, NOS

M8442/1 Serous cystadenoma, borderline malignancy (C56.9)

  • Serous tumor, NOS, of low malignant potential
  • Atypical proliferating serous tumor

M8443/0 Clear cell cystadenoma (C56.9)

M8444/1 Clear cell cystic tumor of borderline malignancy (C56.9)

  • Atypical proliferating clear cell tumor

M8450/0 Papillary cystadenoma, NOS (C56.9)

M8450/3 Papillary cystadenocarcinoma, NOS (C56.9)

  • Papillocystic adenocarcinoma

M8451/1 Papillary cystadenoma, borderline malignancy (C56.9)

M8452/1 Solid pseudo papillary tumor (C25._)

  • Papillary cystic tumor
  • Solid and papillary epithelial neoplasm
  • Solid and cystic tumor

M8452/3 Solid pseudopapillary carcinoma (C25._)

M8453/0 Intraductal papillary-mucinous adenoma (C25._)

M8453/1 Intraductal papillary-mucinous tumor with moderate dysplasia (C25._)

M8453/2 Intraductal papillary-mucinous carcinoma, non-invasive (C25._)

M8453/3 Intraductal papillary-mucinous carcinoma invasive (C25._)

M8454/0 Cystic tumor of atrio-ventricular node (C38.0)

M8460/0 Papillary serous cystadenoma, NOS (C56.9)

M8460/3 Papillary serous cystadenocarcinoma (C56.9)

  • Papillary serous adenocarcinoma
  • Micropapillary serous carcinoma

M8461/0 Serous surface papilloma (C56.9)

M8461/3 Serous surface papillary carcinoma (C56.9)

  • Primary serous papillary carcinoma of peritoneum (C48.1)

M8462/1 Serous papillary cystic tumor of borderline malignancy (C56.9)

  • Papillary serous cystadenoma, borderline malignancy
  • Papillary serous tumor of low malignant potential
  • Atypical proliferative papillary serous tumor

M8463/1 Serous surface papillary tumor of borderline malignancy (C56.9)

M8470/0 Mucinous cystadenoma, NOS (C56.9)

  • Mucinous cystoma
  • Pseudomucinous cystadenoma, NOS

M8470/1 Mucinous cystic tumor with moderate dysplasia (C25._)

M8470/2 Mucinous cystadenocarcinoma, non-invasive (C25._)

M8470/3 Mucinous cystadenocarcinoma/ NOS (C56.9)

  • Pseudomucinous adenocarcinoma
  • Pseudomucinous cystadenocarcinoma, NOS

M8471/0 Papillary mucinous cystadenoma, NOS (C56.9)

  • Papillary pseudomucinous cystadenoma, NOS

M8471/3 papillary mucinous cystadenocarcinoma (C56.9)

  • Papillary pseudomucinous cystadenocarcinoma

M8472/1 Mucinous cystic tumor of borderline malignancy (C56.9)

  • Mucinous cystadenoma, borderline malignancy
  • Pseudomucinous cystadenoma, borderline malignancy
  • Mucinous tumor, NOS, of low malignant potential
  • Atypical proliferative mucinous tumor

M8473/1 Papillary mucinous cystadenoma, borderline malignancy (C56.9)

  • Papillary pseudomucinous cystadenoma borderline malignancy
  • Papillary mucinous tumor of low malignant potential

M8480/0 Mucinous adenoma

M8480/3 Mucinous adenocarcinoma

  • Mucinous carcinoma
  • Colloid adenocarcinoma
  • Colloid carcinoma
  • Gelatinous adenocarcinoma
  • Gelatinous carcinoma
  • Mucoid adenocarcinoma
  • Mucoid carcinoma
  • Mucous adenocarcinoma
  • Mucous carcinoma
  • Pseudomyxoma peritonei with unknown primary site (C80.9)

M8480/6 Pseudomyxoma peritonei

M8481/3 Mucin-producing adenocarcinoma

  • Mucin-producing carcinoma
  • Mucin-secreting adenocarcinoma
  • Mucin-secreting carcinoma

M8482/3 Mucinous adenocarcinoma, endocervical type

M8490/3 Signet ring cell carcinoma

  • Signet ring cell adenocarcinoma

M8490/6 Metastatic signet ring cell carcinoma

  • Krukenberg tumor

850–854 Ductal, Lobular And Medullary Neoplasms

[edit]

M8500/2 Intraductal carcinoma, noninfiltrating, NOS

  • Intraductal adenocarcinoma, noninfiltrating, NOS
  • Intraductal carcinoma, NOS
  • Ductal carcinoma in situ, NOS (C50._)
  • DCIS, NOS
  • Ductal intraepithelial neoplasia 3
  • DIN 3

M8500/3 Infiltrating duct carcinoma, NOS (C50._)

  • Infiltrating duct adenocarcinoma
  • Duct adenocarcinoma, NOS
  • Duct carcinoma, NOS
  • Duct cell carcinoma
  • Ductal carcinoma, NOS

M8501/2 Comedocarcinoma, noninfiltrating (C50._)

  • Ductal carcinoma in situ, comedo type
  • DCIS, comedo type

M8501/3 Comedocarcinoma, NOS (C50._)

M8502/3 Secretory carcinoma of breast (C50._)

  • Juvenile carcinoma of breast

M8503/0 Intraductal papilloma

  • Duct adenoma, NOS
  • Ductal papilloma

M8503/2 Noninfiltrating intraductal papillary adenocarcinoma (C50._)

  • Noninfiltrating intraductal papillary carcinoma
  • Intraductal papillary adenocarcinoma, NOS
  • Intraductal papillary carcinoma, NOS
  • Ductal carcinoma in situ, papillary
  • DCIS, papillary

M8503/3 Intraductal papillary adenocarcinoma with invasion (C50._)

  • Infiltrating papillary adenocarcinoma
  • Infiltrating and papillary adenocarcinoma

M8504/0 Intracystic papillary adenoma

  • Intracystic papilloma

M8504/2 Noninfiltrating intracystic carcinoma

M8504/3 Intracystic carcinoma, NOS

  • Intracystic papillary adenocarcinoma

M8505/0 Intraductal papillomatosis, NOS

  • Diffuse intraductal papillomatosis

M8506/0 Adenoma of nipple (C50._)

  • Subareolar duct papillomatosis

M8507/2 Intraductal micropapillary carcinoma (C50._)

  • Ductal carcinoma in situ, micropapillary
  • Intraductal carcinoma, clinging

M8508/3 Cystic hypersecretory carcinoma (C50._)

M8510/3 Medullary carcinoma, NOS

  • Medullary adenocarcinoma

M8512/3 Medullary carcinoma with lymphoid stroma

M8513/3 Atypical medullary carcinoma (C50._)

M8514/3 Duct carcinoma, desmoplastic type

M8520/2 Lobular carcinoma in situ, NOS (C50._)

  • Lobular carcinoma, noninfiltrating
  • LCIS, NOS

M8520/3 Lobular carcinoma, NOS (C50._)

  • Lobular adenocarcinoma
  • Infiltrating lobular carcinoma, NOS

M8521/3 Infiltrating ductular carcinoma (C50._)

M8522/2 Intraductal carcinoma and lobular carcinoma in situ (C50._)

M8522/3 Infiltrating duct and lobular carcinoma (C50._)

  • Lobular and ductal carcinoma
  • Infiltrating duct and lobular carcinoma in situ
  • Intraductal and lobular carcinoma
  • Infiltrating lobular carcinoma and ductal carcinoma in situ

M8523/3 Infiltrating duct mixed with other types of carcinoma (C50._)

  • Infiltrating duct and cribiform carcinoma
  • Infiltrating duct and mucinous carcinoma
  • Infiltrating duct and tubular carcinoma
  • Infiltrating duct and colloid carcinoma

M8524/3 Infiltrating lobular mixed with other types of carcinoma (C50._)

M8525/3 Polymorphous low grade adenocarcinoma

  • Terminal duct adenocarcinoma

M8530/3 Inflammatory carcinoma (C50._)

  • Inflammatory adenocarcinoma

M8540/3 Paget disease, mammary (C50._)

  • Paget disease of breast

M8541/3 Paget disease and infiltrating duct carcinoma of breast (C50._)

M8542/3 Paget disease, extramammary (except Paget disease of bone)

M8543/3 Paget disease and intraductal carcinoma of breast (C50._)

M8550/0 Acinar cell adenoma

  • Acinar adenoma
  • Acinic cell adenoma

M8550/1 Acinar cell tumor

  • Acinic cell tumor

M8550/3 Acinar cell carcinoma

  • Acinic cell adenocarcinoma
  • Acinar adenocarcinoma
  • Acinar carcinoma

M8551/3 Acinar cell cystadenocarcinoma

M8560/0 Mixed squamous cell and glandular papilloma

M8560/3 Adenosquamous carcinoma

  • Mixed adenocarcinoma and squamous cell carcinoma
  • Mixed adenocarcinoma and epidermoid carcinoma

M8561/0 Adenolymphoma (C07._, C08._)

M8562/3 Epithelial-myoepithelial carcinoma

M8570/3 Adenocarcinoma with squamous metaplasia

M8571/3 Adenocarcinoma with cartilaginous and osseous metaplasia

  • Adenocarcinoma with cartilaginous metaplasia
  • Adenocarcinoma with osseous metaplasia

M8572/3 Adenocarcinoma with spindle cell metaplasia

M8573/3 Adenocarcinoma with apocrine metaplasia

  • Carcinoma with apocrine metaplasia

M8574/3 Adenocarcinoma with neuroendocrine differentiation

  • Carcinoma with neuroendocrine differentiation

M8575/3 Metaplastic carcinoma, NOS

M8576/3 Hepatoid adenocarcinoma

  • Hepatoid carcinoma

858 Thymic Epithelial Neoplasms

[edit]
  • M8580/0 Thymoma, benign (C37.9)
  • M8580/1 Thymoma, NOS (C37.9)
  • M8580/3 Thymoma, malignant, NOS (C37.9)
  • M8581/1 Thymoma, type A, NOS (C37.9)
  • Thymoma, spindle cell, NOS
  • Thymoma, medullary, NOS

M8581/3 Thymoma, type A, malignant (C37.9)

  • Thymoma, spindle cell, malignant
  • Thymoma, medullary, malignant

M8582/1 Thymoma, type AB, NOS (C37.9)

  • Thymoma, mixed type, NOS

M8582/3 thymoma, type AB, malignant (C37.9)

  • Thymoma, mixed type, malignant

M8583/1 Thymoma, type B1, NOS (C37.9)

  • Thymoma, lymphocyte-rich, NOS
  • Thymoma, lymphocytic, NOS
  • Thymoma, predominantly cortical, NOS
  • Thymoma, organoid, NOS

M8583/3 Thymoma, type B1, malignant (C37.9)

  • Thymoma, lymphocyte-rich, malignant
  • Thymoma, lymphocytic, malignant
  • Thymoma, predominantly cortical, malignant
  • Thymoma, organoid, malignant

M8584/1 Thymoma, type B2, NOS (C37.9)

  • Thymoma, cortical, NOS

M8584/3 Thymoma, type B2, malignant (C37.9)

  • Thymoma, cortical, malignant

M8585/1 Thymoma, type B3, NOS (C37.9)

  • Thymoma, epithelial, NOS
  • Thymoma, atypical, NOS

M8585/3 Thymoma, type B3, malignant (C37.9)

  • Thymoma, epithelial, malignant
  • Thymoma, atypical, malignant
  • Well differentiated thymic carcinoma

M8586/3 Thymic carcinoma, NOS (C37.9)

  • Thymoma, type C

M8587/0 Ectopic hamartomatous thymoma

M8588/3 Spindle epithelial tumor with thymus-like element

  • Spindle epithelial tumor with thymus-like differentiation
  • SETTLE

M8589/3 Carcinoma showing thymus-like element

  • Carcinoma showing thymus-like differentiation
  • CASTLE

859–867 Specialized gonadal neoplasms

[edit]

M8590/1 Sex cord-stromal tumor, NOS

  • Sex cord/gonadal stromal tumor, NOS
  • Testicular/ovarian stromal tumor

M8591/1 Sex cord-gonadal stromal tumor, incompletely differentiated

M8592/1 Sex cord-gonadal stromal tumor, mixed forms

M8593/1 Stromal tumor with minor sex cord elements

M8600/0 Thecoma, NOS

  • Theca cell tumor

M8600/3 Thecoma, malignant

M8601/0 Thecoma, luteinized

M8602/0 Sclerosing stromal tumor

M8610/0 Luteoma, NOS

  • Luteinoma

M8620/1 Granulosa cell tumor, NOS

  • adult type

M8620/3 Granulosa cell tumor, malignant

  • Granulosa cell carcinoma
  • Granulosa cell tumor, sarcomatoid

M8621/1 Granulosa cell-theca cell tumor

  • Theca cell-granulosa cell tumor

M8622/1 Granulosa cell tumor, juvenile

M8623/1 Sex cord tumor with annular tubules

M8630/1 Androblastoma/Arrhenoblastoma, benign

M8630/1 Androblastoma/Arrhenoblastoma, NOS

M8630/3 Androblastoma/Arrhenoblastoma, malignant

M8631/0 Sertoli-Leydig cell tumor, well differentiated

M8631/1 Sertoli-Leydig cell tumor of intermediate differentiation

M8631/3 Sertoli-Leydig cell tumor, poorly differentiated

M8632/1 Gynandroblastoma

M8633/1 Sertoli-Leydig cell tumor, retiform

M8634/1 Sertoli-Leydig cell tumor, intermediate differentiation, with heterologous elements

  • retiform, with heterologous elements

M8634/3 Sertoli-Leydig cell tumor, poorly differentiated, with heterologous elements

M8640/1 Sertoli tumor, NOS

  • Pick tubular adenoma
  • Sertoli cell adenoma
  • Tubular androblastoma, NOS
  • Testicular adenoma

M8640/3 Sertoli cell carcinoma (C62._)

M8641/0 Sertoli cell tumor with lipid storage

  • Folliculome lipidique (C56.9)
  • Tubular androblastoma with lipid storage
  • Lipid-rich Sertoli cell tumor

M8642/1 large cell calcifying Sertoli cell tumor

M8650/0 Leydig cell tumor, benign (C62._)

M8650/3 Leydig cell tumor, malignant

  • Interstitial cell tumor, malignant

M8660/0 Hilus cell tumor (C56.9)

  • Hilar cell tumor

M8670/0 Lipid cell tumor of ovary (C56.9)

  • Lipoid cell tumor of ovary
  • Steroid cell tumor, NOS
  • Masculinovoblastoma

M8670/3 Steroid cell tumor, malignant

M8641/0 Adrenal rest tumor

M8680/0 Paraganglioma, benign

M8680/1 Paraganglioma, NOS

M8680/3 paraganglioma, malignant

M8681/1 Sympathetic paraganglioma

M8682/1 Parasympathetic paraganglioma

M8683/0 Gangliocytic paraganglioma (C17.0)

M8690/1 Glomus jugulare tumor, NOS (C75.5)

  • Jugular/jugulotympanic paranglioma

M8691/1 Aortic body tumor (C75.5)

  • Aortic/aorticopulmonary paraganglioma

M8692/1 Carotid body tumor/paraganglioma (C75.4)

M8693/1 Extra-adrenal paraganglioma, NOS

  • Nonchromaffin paraganglioma, NOS
  • Chemodectoma

M8693/3 Extra-adrenal paraganglioma, malignant

  • Nonchromaffin paraganglioma, malignant

M8700/0 Pheochromocytoma, NOS (C74.1)

  • Adrenal medullary/chromaffin paraganglioma
  • Chromaffin tumor
  • Chromaffinoma

M8700/3 Pheochromocytoma, malignant (C74.1)

  • Adrenal medullary paraganglioma, malignant
  • Pheochromoblastoma

M8710/3 Glomangiosarcoma

  • Glomoid sarcoma

M8711/0 Glomus tumor, NOS

M8711/3 Glomus tumor, Malignant

M8712/0 Glomangioma

M8713/0 Glomangiomyoma

872–879 Nevi And Melanomas

[edit]

M8720/0 Pigmented nevus, NOS

M8720/2 Melanoma in situ

M8720/3 Malignant melanoma, NOS (except juvenile melanoma M8770/0)

M8721/3 Nodular melanoma

M8722/0 Balloon cell nevus

M8722/3 Balloon cell melanoma

M8723/0 Halo nevus

  • Regressing nevus

M8723/3 Malignant melanoma, regressing

M8725/0 Neuronevus

M8726/0 Magnocellular nevus (C69.4)

  • Melanocytoma, eyeball
  • Melanocytoma, NOS

M8727/0 Dysplastic nevus

M8727/0 dysplastic nevus

M8728/0 Diffuse melanocytosis

M8728/1 Meningeal melanocytoma (C70.9)

M8728/3 Meningeal melanomatosis (C70.9)

M8730/0 Nonpigmented nevus

  • Achromic nevus

M8740/0 Junctional nevus, NOS

  • Intraepidermal nevus

M8740/3 Malignant melanoma in junctional nevus

M8741/2 Precancerous melanosis, NOS

M8741/3 Malignant melanoma in precancerous melanosis

M8742/3 Lentigo maligna melanoma

  • Hutchinson melanotic freckle

M8743/3 Superficial spreading melanoma

M8744/3 Acral lentiginous melanoma, malignant

M8745/3 * Desmoplastic melanoma, malignant

M8746/3 Mucosal lentiginous melanoma

M8750/0 Intradermal nevus

  • Dermal nevus

M8760/0 compound nevus

  • Dermal and epidermal nevus

M8761/0 Small congenital nevus

M8761/1 Giant pigmented nevus, NOS

  • Intermediate and giant congenital nevus

M8761/3 Malignant melanoma in giant pigmented nevus/congenital melanocytic nevus

M78762/1 Proliferative dermal lesion in congenital nevus

M8770/0 Epithelioid and spindle cell nevus

  • Juvenile nevus
  • Juvenile melanoma
  • Spitz nevus
  • Pigmented spindle cell nevus of Reed

M8770/3 Mixed epithelioid and spindle cell melanoma

M8771/0 Epithelioid cell nevus

M8771/3 Epithelioid cell melanoma

M8772/0 spindle cell nevus, NOS

M8772/3 Spindle cell melanoma, NOS

M8773/3 Spindle cell melanoma, type A

M8774/3 Spindle cell melanoma, type B

M8780/0 Blue nevus, NOS

  • Jadassohn blue nevus

M8780/3 blue nevus, malignant

M8790/0 Cellular blue nevus

M8800/0 Soft tissue tumor, benign

M8800/3 Sarcoma, NOS

M8800/9 Sarcomatosis, NOS

M8801/3 Spindle cell sarcoma

M8802/3 Giant cell sarcoma (except of bone M9250/3)

  • Pleomorphic cell sarcoma

M8803/3 Small cell sarcoma

  • Round cell sarcoma

M8804/3 Epithelioid sarcoma

  • Epithelioid cell sarcoma

M8805/3 Undifferentiated sarcoma

M8806/3 Desmoplastic small round cell tumor

M8810/0 Fibroma, NOS

M8810/1 Cellular fibroma (C56.9)

M8810/3 Fibrosarcoma, NOS

M8811/0 Fibromyxoma

  • Myxoid fibroma
  • Myxofibroma, nos

M8811/3 Fibromyxosarcoma

M8812/0 Periosteal fibroma (C40._, C41._)

  • Periosteal sarcoma, NOS

M8813/0 Fascial fibroma

M8813/3 Fascial fibrosarcoma

M8814/3 Infantile fibrosarcoma

  • Congenital fibrosarcoma

M8815/0 Solitary fibrous tumor

  • Localized fibrous tumor

M8815/3 Solitary fibrous tumor, malignant

M8820/0 Elastofibroma

M8821/1 Aggressive fibromatosis

  • Extra-abdominal desmoid
  • Desmoid, NOS
  • Invasive fibroma

M8822/1 Abdominal fibromatosis

  • Abdominal desmoid
  • Mesenteric fibromatosis (C48.1)
  • Retroperitoneal fibromatosis (C48.0)

M8823/0 Desmoplastic fibroma

M8824/0 Myofibroma

M8824/1 Myofibromatosis

M8825/0 Myofibroblastoma

M8825/1 Myofibroblastic tumor, nos

  • Inflammatory myofibroblastic tumor

M8826/0 Angiomyofibroblastoma

M8827/1 Myobfibroblastic tumor, peribronchial (C34._)

  • congenital peribronchial myofibroblastic tumor

M8830/0 Benign fibrous histiocytoma

  • Fibrous histiocytoma, NOS
  • Fibroxanthoma, NOS
  • Xantofibroma

M8830/1 Atypical fibrous histiocytoma

  • Atypical fibroxanthoma

M8830/3 Malignant fibrous histiocytoma

  • Fibroxanthoma, malignant

M8831/0 Histiocytoma, NOS

  • Deep histiocytoma
  • Juvenile histiocytoma
  • Reticulohistiocytoma

M8832/0 Dermatofibroma, NOS (C44._)

  • Sclerosing hemangioma
  • Cutaneous histiocytoma
  • Subepidermal nodular fibrosis
  • Dermatofibroma lenticulare

M8832/3 Dermatofibrosarcoma, NOS (C44._)

  • Dermatofibrosarcoma protuberans, NOS

M8833/3 Pigmented dermatofibrosarcoma protuberans

  • Bednar tumor

M8834/1 Giant-cell fibroblastoma

M8835/1 Plexiform fibrohistiocytic tumor

M8836/1 Angiomatoid fibrous histiocytoma

M8840/0 Myxoma, NOS

M8840/3 Myxosarcoma

M8841/1 Angiomyxoma

  • Aggressive angiomyxoma

M8842/0 Ossifying fibromyxoid tumor

M8850/0 Lipoma, NOS

M8850/1 Atypical lipoma

  • Superficial well differentiated liposarcoma
  • Well differentiated liposarcoma of superficial soft tissue

M8850/3 Liposarcoma, NOS

  • Fibroliposarcoma

M8851/0 Fibrolipoma

M8851/3 Liposarcoma, well differentiated

  • Liposarcoma, differentiated
  • Lipoma-like liposarcoma
  • Sclerosing liposarcoma
  • Inflammatory liposarcoma

M8852/0 Fibromyxolipoma

  • Myxolipoma

M8852/3 Myxoid liposarcoma

  • Myxoliposarcoma

M8853/3 Round cell liposarcoma

M8854/0 Pleomorphic lipoma

M8854/3 Pleomorphic liposarcoma

M8855/3 Mixed liposarcoma

M8856/0 Intramuscular lipoma

  • Infiltrating lipoma/angiolipoma

M8857/0 Spindle cell lipoma

M8857/3 Fibroblastic liposarcoma

M8858/3 Dedifferentiated liposarcoma

M8860/0 Angiomyolipoma

M8861/0 Angiolipoma, NOS

M8862/0 Chondroid lipoma

M8870/0 Myelolipoma

M8880/0 Hibernoma

  • Fetal fat cell lipoma
  • Brown fat tumor

M8881/0 Lipoblastomatosis

  • Fetal lipoma, NOS
  • Fetal lipomatosis
  • Lipoblastoma

M8890/0 Leiomyoma, NOS

  • Fibroid uterus (C55.9)
  • Fibromyoma
  • Leiomyofibroma
  • Plexiform leiomyoma
  • Lipoleiomyoma

M8890/1 Leiomyomatosis, NOS

  • Intravascular leiomyomatosis

M8890/3 Leiomyosarcoma, NOS

M8891/0 Epithelioid leiomyoma

  • Leiomyoblastoma

M8891/3 Epithelioid leiomyosarcoma

M8892/0 Cellular leiomyoma

M8893/0 Bizarre leiomyoma

  • Symplastic/atypical/pleomorphic leiomyoma

M8894/0 Angiomyoma

  • Vascular leiomyoma
  • Angioleiomyoma

M8894/3 Angiomyosarcoma

M8895/0 Myoma

M8895/3 Myosarcoma

M8896/3 Myxoid leiomyosarcoma

M8897/1 Smooth muscle tumor of uncertain malignant potential

  • Smooth muscle tumor, NOS

M8898/1 Metastasizing leiomyoma

M8900/0 Rhabdomyoma, NOS

M8900/3 Rhabdomyosarcoma, NOS

  • Rhabdosarcoma

M8901/3 Pleomorphic rhabdomyosarcoma, adult type

  • Pleomorphic rhabdomyosarcoma, NOS

M8902/3 Mixed type rhabdomyosarcoma

  • Mixed embryonal rhabdomyosarcoma and alveolar rhabdomyosarcoma

M8903/0 Fetal rhabdomyoma

M8904/0 Adult rhabdomyoma

  • Glycogenic rhabdomyoma

M8905/0 Genital rhabdomyoma

M8910/3 Embryonal rhabdomyosarcoma, NOS

  • Embryonal rhabdomyosarcoma, pleomorphic
  • Sarcoma botryoides
  • Botryoid sarcoma

M8912/3 Spindle cell rhabdomyosarcoma

M8920/3 Alveolar rhabdomyosarcoma

M8921/3 Rhabdomyosarcoma with ganglionic differentiation

  • Ectomesenchymoma

893–899 Complex Mixed And Stromal Neoplasms

[edit]

M8930/0 Endometrial stromal nodule

M8930/3 Endometrial stromal sarcoma, NOS

  • Endometrial sarcoma, NOS
  • Endometrial stromal sarcoma, high grade

M8931/3 Endometrial stromal sarcoma, low grade

  • Endolymphatic stromal myosis
  • Endometrial stromatosis
  • Stromal endometriosis
  • Stromal myosis, NOS

M8932/0 Adenomyoma

  • Atypical polypoid adenomyoma

M8933/3 Adenosarcoma

M8934/3 Carcinofibroma

M8935/0 Stromal tumor, benign

M9835/1 Stromal tumor, NOS

M8935/3 Stromal sarcoma, NOS

M8936/0 Gastrointestinal stromal tumor, benign

  • GIST, benign

M8936/1 Gastrointestinal stromal tumor, NOS

  • GIST, NOS/uncertain malignant potential
  • Gastrointestinal autonomic nerve tumor (GANT)
  • Gastrointestinal pacemaker cell tumor

M8936/3 Gastrointestinal stromal sarcoma

  • GIST, malignant

M8940/0 Pleomorphic adenoma

M8940/3 Mixed tumor, malignant, NOS

M8941/3 Carcinoma in pleomorphic adenoma

M8950/3 Müllerian mixed tumor

M8951/3 Mesodermal mixed tumor

M8959/0 Benign cystic nephroma

M8959/1 Cystic partially differentiated nephroblastoma

M8959/3 Malignant cystic nephroma

  • Malignant multilocular cystic nephroma

M8960/1 Mesoblastic nephroma

M8960/3 Nephroblastoma, NOS

M8963/3 malignant rhabdoid tumor

  • Rhabdoid sarcoma
  • Rhabdoid tumor, NOS

M8964/3 Clear cell sarcoma of kidney

M8965/0 Nephrogenic adenofibroma

M8966/0 Renomedullary interstitial cell tumor

  • Renomedullary fibroma

M8967/0 Ossifying renal tumor

M8970/3 Hepatoblastoma

  • Embryonal hepatoma

M8971/3 Pancreatoblastoma

M8972/3 Pulmonary blastoma

  • Pneumoblastoma

M8973/3 Pleuropulmonary blastoma

M8974/1 Sialoblastoma

M8980/3 Carcinosarcoma, NOS

M8981/3 Carcinosarcoma, embryonal

M8982/0 Myoepithelioma

  • Myoepithelial tumor
  • Myoepithelial adenoma

M8982/3 Malignant myoepithelioma

  • Myoepithelial carcinoma

M8983/0 Adenomyoepithelioma

M8990/0 Mesencymoma, benign

M8990/1 Mesenchymoma, NOS

  • Mixed mesenchymal tumor

M8990/3 Mesenchymoma, malignant

  • Mixed mesenchymal sarcoma

M8991/3 Embryonal sarcoma

M9000/0 Brenner tumor, NOS

M9000/1 Brenner tumor, borderline malignancy

  • Brenner tumor, proliferating

M9000/3 Brenner tumor, malignant

M9010/0 Fibroadenoma, NOS

M9011/0 Intracanalicular fibroadenoma

M9012/0 Pericanalicular fibroadenoma

M9013/0 Adenofibroma, NOS

  • Cystadenofibroma, NOS
  • Papillary adenofibroma

M9014/0 Serous adenofibroma, NOS

  • Serous cystadenofibroma, NOS

M9014/1 Serous adenofibroma of borderline malignancy

  • Serous cystadenofibroma of borderline malignancy

M9014/3 Serous adenocarcinofibroma

  • Malignant serous adenofibroma
  • Serous cystadenocarcinofibroma
  • Malignant serous cystadenofibroma

M9015/0 Mucinous adenofibroma, NOS

  • Mucinous cystadenofibroma, NOS

M9015/1 Mucinous adenofibroma of borderline malignancy

  • Mucinous cystadenofibroma of borderline malignancy

M9015/3 Mucinous adenocarcinofibroma

  • Malignant mucinous adenofibroma
  • Mucinous cystadenocarcinofibroma
  • Malignant mucinous cystadenofibroma

M9016/0 Gian fibroadenoma

M9020/0 Phyllodes tumor, benign

  • Cystosarcoma phyllodes, benign

M9020/1 Phyllodes tumor, borderline

  • Cystosarcoma phyllodes, NOS
  • Phyllodes tumor, NOS

M9020/3 Phyllodes tumor, malignant

  • Cystosarcoma phyllodes, malignant

M9030/0 Juvenile fibroadenoma

M9040/0 Synovioma, benign

M9040/3 Synovial sarcoma, NOS

  • Synovioma, NOS
  • Synovioma, malignant

M9041/3 Synovial sarcoma, spindle cell

  • Synovial sarcoma, monophasic fibrous

M9042/3 Synovial sarcoma, epithelioid cell

M9043/3 Synovial sarcoma, biphasic

M9044/3 Clear cell sarcoma, NOS (except of kidney M9864/3)

  • Clear cell sarcoma, of tendons and aponeuroses
  • Melanoma, malignant, of soft parts

M9050/0 Mesothelioma, benign

M9050/3 Mesothelioma, malignant or NOS

M9051/0 Fibrous mesothelioma, benign

M9051/3 Fibrous mesothelioma, malignant or NOS

  • Spindled mesothelioma
  • Sarcomatoid mesothelioma
  • Desmoplastic mesothelioma

M9052/0 Epithelioid mesothelioma, benign

  • Well differentiated papillary mesothelioma, benign
  • mesothelial papilloma

M9052/3 Epithelioid mesothelioma, malignant or NOS

M9053/3 Mesothelioma, biphasic, malignant or NOS

M9054/0 Adenomatoid tumor, NOS

M9055/0 Multicystic mesothelioma, benign

  • Cystic mesothelioma, benign

M9055/1 cystic mesothelioma, NOS

M9060/3 Dysgerminoma

M9061/3 Seminoma, NOS

M9062/3 Seminoma, anaplastic

  • Seminoma with high mitotic index

M9063/3 Spermatocytic seminoma

  • Spermatocytoma

M9064/2 Intratubular malignant germ cells

  • Intratubular germ cell neoplasia

M9064/3 Germinoma

M9065/3 Germ cell tumor, nonseminomatous

M9070/3 Embryonal carcinoma, NOS

  • Embryonal adenocarcinoma

M9071/3 Yolk sac tumor

  • Endodermal sinus tumor
  • Polyvesicular vitelline tumor
  • Orchioblastoma
  • Embryonal carcinoma, infantile
  • Hepatoid yolk sac tumor

M9072/3 Polyembryoma

  • Embryonal carcinoma, polyembryonal type

M9073/1 Gonadoblastoma

  • Gonocytoma

M9080/0 Teratoma, benign

  • Adult cystic teratoma
  • Adult/cystic teratoma, NOS
  • Teratoma, differentiated
  • Mature teratoma

M9080/1 Teratoma, NOS

  • Solid teratoma

M9080/3 Teratoma, malignant, NOS

  • Embryonal teratoma
  • Teratoblastoma, malignant
  • Immature teratoma, malignant or NOS

M9081/3 Teratocarcinoma

  • Mixed embryonal carcinoma and teratoma

M9082/3 malignant teratoma, undifferentiated

  • Malignant teratoma, anaplastic

M9083/3 Malignant teratoma, intermediate

M9084/0 Dermoid cyst, NOS

  • Dermoid, NOS

M9084/3 Teratoma with malignant transformation

  • Dermoid cyst with malignant transformation or with secondary tumor

M9085/3 mixed germ cell tumor

  • Mixed teratoma and seminoma

M9090/0 Struma ovarii, NOS

M9090/3 Struma ovarii, malignant

M9091/1 Strumal carcinoid

  • Struma ovarii and carcinoid

M9100/0 Hydatidifrom mole, NOS

  • Hydatid mole
  • Complete hydatidiform mole

M9100/1 Invasive hydatidiform mole

  • Chorioadenoma /destruens
  • Chorioadenoma
  • Invasive mole, NOS
  • Malignant hydatidiform mole

M9100/3 Choriocarcinoma, NOS

  • Chorionepithelioma
  • Chorioepithelioma

M9101/3 Choriocarcinoma combined with other germ cell elements

  • combined with teratoma
  • combined with embryonal carcinoma

M9102/3 Malignant teratoma, trophoblastic

M9103/0 Partial hydatidiform mole

M9104/1 Placental site trophoblastic tumor

M9105/3 Trophoblastic tumor, epithelioid

M9110/0 Mesonephroma, benign

  • Mesonephric adenoma
  • Wolffian duct adenoma

M9110/1 Mesonephric tumor, NOS

  • Wolffian duct tumor

M9110/3 Mesonephroma, malignant

  • Mesonephric adenocarcinoma
  • Mesonephroma, NOS
  • Wolffian duct carcinoma

M9120/0 Hemangioma, NOS

M9120/3 Hemangiosarcoma

M9121/0 Cavernous hemangioma

M9122/0 Venous hemangioma

M9123/0 Racemose hemangioma

  • Arteriovenous hemangioma

M9124/3 Kupffer cell sarcoma

M9125/0 Epithelioid hemangioma

  • Histiocytoid hemangioma

M9130/0 Hemangioendothelioma, benign

M9130/1 Hemangioendothelioma, NOS

  • Angioendothelioma
  • Kaposiform hemangioendothelioma

M9130/3 Hemangioendothelioma, malignant

  • Hemangioendothelial sarcoma

M9131/0 Capillary hemangioma

  • Hemangioma simplex
  • Infantile/plexiform/juvenile hemangioma

M9132/0 Intramuscular hemangioma

M9133/1 Epithelioid hemangioendothelioma, NOS

M9133/3 Epithelioid hemangioendothelioma, malignant

  • Intravascular bronchial alveolar tumor

M9135/1 Endovascular papillary angioendothelioma

  • Dabska tumor

M9136/1 Spindle cell hemangioendothelioma

  • Spindle cell angioendothelioma

M9140/3 Kaposi's sarcoma

  • Multiple hemorrhagic sarcoma

M9141/0 Angiokeratoma

M9142/0 Verrucous keratotic hemangioma

M9150/0 Hemangiopericytoma, benign

M9150/1 Hemangiopericytoma, NOS

  • Hemangiopericytic meningioma

M9150/3 Hemangiopericytoma, malignant

M9160/0 angiofibroma, NOS

  • Juvenile angiofibroma
  • Fibrous papule of nose
  • Involuting nevus
  • Giant cell or cellular angiofibroma

M9161/0 Acquired tufted hemangioma

M9161/1 Hemangioblastoma

  • Angioblastoma

M9150/0 Hemangiopericytoma, benign

M9150/1 Hemangiopericytoma, NOS

M9170/0 Lymphangioma, NOS

  • Lymphangioendothelioma, NOS

M9170/3 Lymphangiosarcoma

  • Lymphangioendothelial sarcoma
  • Lymphangioendothelioma, malignant

M9171/0 Capillary lymphangioma

M9172/0 Cavernous lymphangioma

M9173/0 Cystic lymphangioma

  • Hygroma, NOS
  • Cystic Hygroma

M9174/0 Lymphangiomyoma

M9174/1 Lymphangiomyomatosis

  • Lymphangioleiomyomatosis

M9175/0 Hemolymphangioma

M9180/0 Osteoma, NOS

M9180/3 Osteosarcoma, NOS

  • Osteogenic sarcoma, NOS
  • Osteochondrosarcoma
  • Osteoblastic sarcoma

M9181/3 Chondroblastic osteosarcoma

M9182/3 Fibroblastic osteosarcoma

  • Osteofibrosarcoma

M9183/3 Telangeictatic osteosarcoma

M9184/3 Osteosarcoma in Paget disease of bone

M9185/3 Small cell osteosarcoma

  • Round cell osteosarcoma

M9186/3 Central osteosarcoma

  • Conventional central osteosarcoma
  • Medullary osteo sarcoma

M9187/3 Intraosseous well differentiated osteosarcoma

  • Intraosseous low grade osteosarcoma

M9191/0 Osteoid osteoma, NOS

M9192/3 Parosteal osteosarcoma

  • Juxtacortical osteosarcoma

M9193/3 Periosteal osteosarcoma

M9194/3 High grade surface osteosarcoma

M9195/3 Intracortical osteosarcoma

M9200/0 Osteoblastmoa, NOS

  • Giant osteoid osteoma

M9200/1 Aggressive osteoblastoma

M9210/0 Osteochondroma

M9210/1 Osteochondromatosis, NOS

  • Ecchondrosis

M9220/0 Chondroma, NOS

  • Enchondroma

M9220/1 Condromatosis, NOS

M9220/3 Chondrosarcoma, NOS

  • Fibrochondrosarcoma

M9221/0 Juxtacortical chondroma

  • Periosteal chondroma

M9221/3 Juxtacortical chondrosarcoma

  • Periosteal chondrosarcoma

M9230/0 Chondroblastoma, NOS

  • Chondromatous giant cell tumor
  • Codman tumor

M9230/3 Chondroblastoma, malignant

M9231/3 Myxoid chondrosarcoma

M9240/3 Mesenchymal chondrosarcoma

M9241/0 Chondromyxoid fibroma

M9242/3 Clear cell chondrosarcoma

M9243/3 Dedifferentiated chondrosarcoma

M9250/1 giant cell tumor of bone, NOS

  • Osteoclastoma, NOS

M9250/3 Giant cell tumor of bone, malignant

  • Osteoclastoma, malignant
  • Giant cell sarcoma of bone

M9251/1 Giant cell tumor of soft parts, NOS

M9251/3 Malignant giant cell tumor of soft parts

M9252/0 Tenosynovial giant cell tumor

  • Fibrous histiocytoma of tendon sheath
  • Giant cell tumor of tendon sheath

M9252/3 Malignant tenosynovial giant cell tumor

  • Giant cell tumor of tendon sheath, malignant

926 Miscellaneous bone tumors (C40._, C41._)

[edit]

M9260/3 Ewing's sarcoma/tumor

M9261/3 Adamantinoma of long bones

M9262/0 Ossifying fibroma

  • Fibro-osteoma
  • Osteofibroma

927–934 Odontogenic tumors C41._)

[edit]

M9270/0 Odontogenic tumor, benign

M9270/1 Odontogenic tumor, NOS

M9270/3 Odontogenic tumor, malignant

  • Odontogenic carcinoma/sarcoma
  • Primary intraosseous or ameloblastic carcinoma

M9271/0 Ameloblastic fibrodentinoma

  • Dentinoma

M9272/0 Cementoma, NOS

  • Periapical cemental dysplasia or cemento-osseus dysplasia

M9273/0 Cementoblastoma, benign

M9274/0 Cementifying fibroma

  • Cemento-ossifying fibroma

M9275/0 Gigantiform cementoma

  • Florid osseus dysplasia

M9280/0 Odontoma, NOS

M9281/0 Compound odontoma

M9282/0 Complex odontoma

M9290/0 Ameloblastic fibro-odontoma

  • Fibroameloblastic odontoma

M9290/3 Ameloblastic odontosarcoma

  • Ameloblastic fibrodentinosarcoma or fibro-odontosarcoma

M9300/0 Adenomatoid odontogenic tumor

  • Adenomeloblastoma

M9301/0 Calcifying odontogenic cyst

M9302/0 Odontogenic ghost cell tumor

M9310/0 Ameloblastoma, NOS

  • Adamantinoma, NOS (except of long bones M9261/3)

M9310/3 Ameloblastoma, malignant

  • Adamantinoma, NOS (except of long bones M9261/3)

M9311/0 Odontoameloblastoma

M9312/0 Squamous odontogenic tumor

M9320/0 Odontogenic myxoma

  • Odontogenic myxofibroma

M9321/0 Central odontogenic fibroma

  • Odontogenic fibroma, NOS

M9322/0 Peripheral odontogenic fibroma

M9330/0 Ameloblastic fibroma

M9330/3 Ameloblastic fibrosarcoma

  • Ameloblastic sarcoma
  • Odontogenic fibrosarcoma

M9340/0 Calcifying epithelial odontogenic tumor

  • Pindbord tumor

M9341/1 clear cell odontogenic tumor

M9342/3 Odontogenic carcinosarcoma

935–937 Miscellaneous tumors

[edit]

M9350/1 Craniopharyngioma

  • Rathke pouch tumor

M9351/1 Craniopharyngioma, adamantinomatous

M9352/1 Craniopharyngioma, papillary

M9360/1 Pinealoma

M9361/1 Pineocytoma

M9362/3 Pineoblastoma

  • Mixed pineal tumor
  • Mixed pineocytoma-pineoblastoma
  • Pineal parenchymal tumor of intermediate differentiation
  • Transitional pineal tumor

M9363/0 Melanotic neuroectodermal tumor

  • Retinal anlage tumor
  • Melanoameloblastoma
  • Melanotic progonoma

M9364/3 Peripheral neuroectodermal tumor

  • Neuroectodermal tumor, NOS

Peripheral primitive neuroectodermal tumor, NOS (PPNET)

M9365/3 Askin Tumor

M9370/3 Chordoma, NOS

M9371/3 Chondroid chordoma

M9372/3 Dedifferentiated chorcoma

M9373/0 Parachordoma

938–948 Gliomas

[edit]

M9380/3 Glioma, malignant

  • Glioma, NOS (except nasal glioma, not neoplastic)

M9381/3 Gliomatosis cerebri

M9382/3 Mixed glioma

M9383/1 Subepyndymoma

  • Subependymal glioma
  • Subependymal astrocytoma, NOS
  • Mixed subendymoma-ependymoma

M9384/1 Subependymal giant cell astrocytoma

M9390/0 Choroid plexus papilloma, NOS

M9390/1 Atypical choroid plexus papilloma

M9390/3 Choroid plexus carcinoma

  • Choroid plexus papilloma, anaplastic or malignant

M9391/3 Ependymoma, NOS

  • Epithelial / cellular / clear cell / tanycytic ependymoma

M9392/3 Ependymoma, anaplastic

  • Ependymoblastoma

M9393/3 Papillary ependymoma

M9394/1 Myxopapillary ependymoma

M9400/3 Astrocytoma, NOS

  • Astrocytic glioma
  • Astroglioma
  • Diffuse astrocytoma
  • Astrocytoma, low grade
  • Diffuse astocytoma, low grade
  • Cystic astrocytoma

M9401/3 Astrocytoma, anaplastic

M9410/3 Protoplasmic astrocytoma

M9411/3 Gemistocytic astrocytoma

  • Gemistocytoma

M9412/1 Desmoplastic infantile astrocytoma or ganglioglioma

M9413/0 dysembryoplastic neuroepithelial tumor

M9420/3 Fibrillary astrocytoma

  • Fibrous astrocytoma

M9421/1 Pilocytic astrocytoma

  • Piloid or Juvenile astrocytoma
  • Spongioblastoma, NOS

M9423/3 Polar spongioblastoma

  • Spongioblastoma polare
  • Primitive polar spongioblastoma

M9424/3 Pleomorphic xanthoastrocytoma

M9430/3 Astroblastoma

M9440/3 Glioblastoma, NOS

M9441/3 Giant cell glioblastoma

  • Monstrocellular sarcoma

M9442/1 gliofibroma

M9442/3 gliosarcoma

  • Glioblastoma with sarcomatous component

M9444/1 Chordoid glioma

  • Chordoid glioma of third ventricle

M9450/3 Oligodendroglioma, NOS

M9451/3 Oligodendroglioma, anaplastic

M9460/3 Oligodendroblastoma

M9470/3 Medullablastoma, NOS

  • Melanotic medulloblastoma

M9471/3 Desmoplastic nodular medulloblastoma

  • Desmoplastic medulloblastoma
  • Circumscribed arachnoidal cerebellar sarcoma

M9472/3 Medullomyoblastoma

M9473/3 Primitive neuroectodermal tumor, NOS

  • PNET, NOS
  • Central primitive neuroectodermal tumor, NOS (CPNET)
  • Supratentorial PNET

M9474/3 large cell medulloblastoma

M9480/3 Cerebellar sarcoma, NOS

M9490/0 Ganglioneuroma

M9490/3 Ganglioneuroblastoma

M9491/0 Ganglioneuromatosis

M9492/0 Gangliocytoma

M9493/0 Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos)

M9500/3 Neuroblastoma, NOS

  • Sympathicoblastoma
  • Central neuroblastoma

M9501/0 Medulloepithelioma, benign

  • Diktyoma, benign

M9501/3 Medulloepithelioma, NOS

  • Diktyoma, malignant

M9502/0 Teratoid medulloepithelioma, benign

M9502/3 Teratoid medulloepithelioma

M9503/3 Neuroepithelioma, NOS

M9504/3 Spongioneuroblastoma

M9505/1 Ganglioglioma, NOS

M9505/3 Ganglioglioma, anaplastic

M9506/1 Central neurocytoma

  • Neurocytoma
  • Cerebellar liponeurocytoma
  • Lipomatous medulloblastoma
  • Neurolipocytoma
  • Medullocytoma

M9507/0 Pacinian tumor

M9508/3 Atypical teratoid/rhabdoid tumor

M9510/0 Retinocytoma

M9510/3 Retinoblastoma, NOS

M9511/3 Retinoblastoma, differentiated

M9512/3 Retinoblastoma, undifferentiated

M9513/3 Retinoblastoma, diffuse

M9514/1 Retinoblastoma, spontaneously regressed

M9520/3 Olfactory neurogenic tumor

M9521/3 Olfactory neurocytoma

  • Esthesioneurocytoma

M9522/3 Olfactory neuroblastoma

  • Esthesioneuroblastoma

M9523/3 Olfactory neuroepithelioma

  • Esthesio neuroepithelioma

M9530/0 Meningioma, NOS

  • Microcystic
  • Secretory
  • Lymphoplasmacyte-rich
  • Metaplastic

M9530/1 Meningiomatosis, NOS

  • Diffuse
  • Multiple meningiomas

M9530/3 Meningioma, malignant

  • Anaplastic
  • Leptomeningeal sarcoma
  • Meningeal sarcoma
  • Meningothelial sarcoma

M9531/0 Meningothelial meningioma

  • Endotheliomatous meningioma
  • Syncytial meningioma

M9532/0 Fibrous meningioma

  • Fibroblastic meningioma

M9533/0 Psammomatous meningioma

M9534/0 Angiomatous meningioma

M9537/0 Transitional meningioma

M9538/1 Clear cell meningioma

  • Chordoid

M9538/3 Papillary meningioma

  • Rhabdoid

M9539/1 Atypical meningioma

M9539/3 Meningeal sarcomatosis

M9540/0 Neurofibroma, NOS

M9540/1 Neurofibromatosis, NOS

  • Multiple neurofibromatosis
  • Von Recklinghausen disease (except of bone)

M9540/3 Malignant peripheral nerve sheath tumor

  • MPNST, NOS
  • MPNST with glandular differentiation
  • Epithelioid
  • with mesenchymal differentiation
  • Melanotic
  • Melanotic psammomatous

M9541/0 Melanotic neurofibroma

M9550/0 Plexiform neurofibroma

  • Plexiform neuroma

M9560/0 Neurilemoma, NOS

  • Schwannoma, NOS
  • Neurinoma
  • Acoustic neuroma
  • Pigmented or melanotic schwannoma
  • Plexiform / cellular / degenerated / ancient / psammomatous

M9560/1 Neurinomatosis

M9561/3 Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation

  • MPNST with rhabdomyoblastic differentiation
  • Triton tumor, malignant
  • Malignant schwannoma with rhabdomyoblastic differentiation

M9562/0 Neurothekeoma

  • Nerve sheath myxoma

M9570/0 Neuroma, NOS

M9571/0 Perineurioma, NOS

M9571/3 Perineurioma, malignant

  • Perineural MPNST

M9560/0 Schwannoma, NOS

M9560/0 Neurinoma

M9560/0 Acoustic neuroma

M9570/0 Neuroma, NOS

M9580/0 Granular cell tumor/myoblastoma, NOS

M9580/3 Granular cell tumor/myoblastoma, malignant

M9581/3 Alveolar soft part sarcoma

M9582/0 Granular cell tumor of the sellar region (C75.1)

[edit]

M9590/3 Malignant Lymphoma, NOS

  • Lymphoma, NOS
  • Microglioma (C71._)

M9591/3 Malignant lymphoma, non-Hodgkin, NOS

  • Non-Hodgkin's lymphoma, NOS
  • B cell lymphoma, NOS
  • Malignant lymphoma, non-cleaved cell, NOS
  • Malignant lymphoma, diffuse, NOS
  • Malignant lymphoma, lymphocytic, intermediate differentiation, nodular
  • Malignant lymphoma, small cell, noncleaved, diffuse
  • Malignant lymphoma, undifferentiated cell, non-Burkitt
  • Malignant lymphoma, undifferentiated cell type, NOS
  • Lymphosarcoma, NOS
  • Lymphosarcoma, diffuse
  • Reticulum cell sarcoma, NOS
  • Reticulum cell sarcoma, diffuse
  • Reticulosarcoma, NOS
  • Reticulosarcoma, diffuse
  • Malignant lymphoma, small cleaved cell, diffuse
  • Malignant lymphoma, lymphocytic, poorly differentiated, diffuse
  • Malignant lymphoma, small cleaved cell, NOS
  • Malignant lymphoma, cleaved cell, NOS

M9596/3 Composite Hodgkin and non-Hodgkin lymphoma

M9650/3 Hodgkin lymphoma, NOS

M9651/3 Hodgkin lymphoma, lymphocyte rich

M9652/3 Mixed cellularity classical Hodgkin lymphoma, NOS

  • Hodgkin lymphoma, mixed cellularity, NOS

M9653/3 Lymphocyte-depleted classical Hodgkin lymphoma, NOS

M9654/3 Hodgkin lymphoma, lymphocyte depletion, diffuse fibrosis

M9655/3 Hodgkin lymphoma, lymphocyte depletion, reticular

M9659/3 Nodular lymphocyte predominant Hodgkin lymphoma

  • Hodgkin lymphoma, lymphocyte predominance, nodular
  • Hodgkin paragranuloma, NOS
  • Hodgkin paragranuloma, nodular

M9661/3 Hodgkin granuloma

M9662/3 Hodgkin sarcoma

M9663/3 Nodular sclerosis classical Hodgkin lymphoma

  • Hodgkin lymphoma, nodular sclerosis, NOS

M9664/3 Hodgkin lymphoma, nodular sclerosis, cellular phase

  • Classical Hodgkin lymphoma, nodular sclerosis, cellular phase

M9665/3 Hodgkin lymphoma, nodular sclerosis, grade 1

  • Classical Hodgkin lymphoma, nodular sclerosis grade 1
  • Hodgkin disease, nodular sclerosis, lymphocyte predominance
  • Hodgkin disease, nodular sclerosis, mixed cellularity

M9667/3 Hodgkin lymphoma, nodular sclerosis, grade 2

  • Classical Hodgkin lymphoma, nodular sclerosis, grade 2
  • Hodgkin disease, nodular sclerosis, lymphocyte depletion
  • Hodgkin disease, nodular sclerosis, syncytial variant

967–969 Mature B-cell Lymphomas

[edit]

M9670/3 Malignant lymphoma, small B lymphocytic, NOS (see also M9823/3)

  • Small lymphocytic lymphoma
  • Malignant lymphoma, small lymphocytic, NOS
  • Malignant lymphoma, lymphocytic, well differentiated, diffuse
  • Malignant lymphoma, lymphocytic, NOS
  • Malignant lymphoma, lymphocytic, diffuse, NOS
  • Malignant lymphoma, small cell, NOS
  • Malignant lymphoma, small lymphocytic, diffuse
  • Malignant lymphoma, small cell diffuse

M9671/3 Malignant lymphoma, lymphoplasmacytic (see also M9761/3)

  • Lymphoplasmacytic lymphoma
  • Malignant lymphoma, lymphoplasmacytoid
  • Immunocytoma
  • Malignant lymphoma, plasmacytoid
  • Plasmacytic lymphoma

M9673/3 Mantle cell lymphoma

  • Includes all variants: blastic, pleomorphic, small cell
  • Mantle zone lymphoma
  • Malignant zone lymphoma
  • Malignant lymphoma, lymphocytic, intermediate differentiation, diffuse
  • Malignant lymphoma, centrocytic
  • Malignant lymphomatous polyposis

M9675/3 Malignant lymphoma, mixed small and large cell, diffuse (see also M9690/3)

  • Malignant lymphoma, mixed lymphocytic-histiocytic, diffuse
  • Malignant lymphoma, mixed cell type, diffuse
  • Malignant lymphoma, centroblastic-centrocytic, NOS
  • Malignant lymphoma, centroblastic-centrocytic, diffuse

M9678/3 Primary effusion lymphoma

M9679/3 Mediastinal large B-cell lymphoma (C38.3)

M9680/3 Malignant lymphoma, large B-cell, diffuse, NOS

  • Diffuse large B-cell lymphoma, NOS
  • Malignant lymphoma, large cell, NOS
  • Malignant lymphoma, large B-cell, NOS
  • Malignant lymphoma, histiocytic, NOS
  • Malignant lymphoma, histiocytic, diffuse
  • Malignant lymphoma, large cell, cleaved and noncleaved
  • Malignant lymphoma, large cell, diffuse, NOS
  • Malignant lymphoma, large cleaved cell, NOS
  • Malignant lymphoma, large cell, cleaved, NOS
  • Malignant lymphoma, large cell, noncleaved, diffuse or NOS
  • Malignant lymphoma, large B-cell, diffuse, centroblastic, NOS
  • Malignant lymphoma, large B-cell, centroblastic, NOS or diffuse
  • Intravascular large B-cell lymphoma
  • Angioendotheliomatosis
  • Angiotropic lymphoma
  • T-cell rich large B-cell lymphoma
  • Histiocyte-rich large B-cell lymphoma
  • Anaplastic large B-cell lymphoma

M9684/3 Malignant lymphoma, large B-cell, diffuse, immunoblastic, NOS

  • Malignant lymphoma, immunoblastic, NOS
  • Immunoblastic sarcoma
  • Malignant lymphoma, large cell, immunoblastic
  • Plasmablastic lymphoma

M9687/3 Burkitt lymphoma, NOS (see also M9826/3)

  • Includes all variants
  • Malignant lymphoma, undifferentiated
  • Malignant lymphoma, small noncleaved
  • Burkitt-like lymphoma

M9689/3 Splenic marginal zone lymphoma

M9690/3 Follicular lymphoma, NOS (see also M9675/3)

  • Malignant lymphoma, follicular/follicle center, NOS

M9691/3 Follicular lymphoma, grade 2

  • Follicular lymphoma, small cleaved cell

M9698/3 Follicular lymphoma, grade 3

  • Malignant lymphoma, large cell/centroblastic, follicular, NOS

M9699/3 Marginal zone B-cell lymphoma, NOS

970–971 Mature T- and NK-cell Lymphomas

[edit]

M9700/3 Mycosis fungoides

  • Pagetoid reticulosis

M9701/3 Sezary syndrome

  • Sezary disease

M9702/3 Mature T-cell lymphoma, NOS

  • Peripheral T-cell lymphoma, NOS
  • T-cell lymphoma, NOS
  • Peripheral T-cell lymphoma, pleomorphic small/medium/large cell/T-zone lymphoma
  • Lymphoepithelioid lymphoma
  • Lennert lymphoma

M9705/3 Angioimmunoblastic T-cell lymphoma

  • Peripheral T-cell lymphoma, AILD (Angioimmunoblastic lymphadenopathy with dysproteinemia)

M9708/3 Subcutaneous panniculitis-like T-cell lymphoma

M9709/3 Cutaneous T-cell lymphoma, NOS (C44._)

  • Cutaneous lymphoma, NOS

M9714/3 Anaplastic large cell lymphoma, T cell and Null cell type

  • Anaplastic large cell lymphoma, CD30+/NOS

M9716/3 Hepatosplenic (gamma-delta) cell lymphoma

M9717/3 Intestinal T-cell lymphoma

M9718/3 Primary cutaneous CD 30+ T-cell lymphoproliferative disorder (C44._)

M9719/3 NK/T-cell lymphoma, nasal and nasal type

972 Precursor Cell Lymphoblastic Lymphoma

[edit]

M9727/3 Precursor cell lymphoblastic lymphoma, NOS (see also M9835/3)

M9728/3 Precursor B-cell lymphoblastic Lymphoma (see also M9836/3)

M9729/3 Precursor T-cell lymphoblastic Lymphoma (see also M9837/3)

M9731/3 Plasmacytoma, NOS

M9732/3 Multiple myeloma (C42.1)

M9733/3 Plasma cell leukemia (C42.1)

  • Plasmacytic leukemia

M9734/3 Plasmacytoma, extramedullary (not occurring in bone)

M9740/1 Mastocytoma, NOS or Extracutaneous mastocytoma

  • Mast cell tumor, NOS

M9740/3 Mast cell sarcoma

  • Malignant mast cell tumor
  • Malignant mastocytoma

M9741/3 Malignant mastocytosis

M9742/3 Mast cell leukemia (C42.1)

975 Neoplasms of Histiocytes and Accessory Lymphoid Cells

[edit]

M9750/3 Malignant histiocytosis

M9751/1 Langerhans cell histiocytosis, NOS

M9752/1 Langerhans cell histiocytosis, unifocal

  • Langerhans cell granulomatosis, unifocal
  • Langerhans cell histiocytosis, mono-ostotic
  • Eosinophilic granuloma

M9753/1 Langerhans cell histiocytosis, multifocal

  • Langerhans cell histiocytosis, poly-ostotic

M9754/3 Langerhans cell histiocytosis, disseminated

  • Langerhans cell histiocytosis, generalized
  • Letterer-Siwe disease
  • Acute progressive histiocytosis X

M9755/3 Histiocytic sarcoma

  • True histiocytic lymphoma

M9756/3 Langerhans cell sarcoma

M9757/3 Dendritic cell sarcoma, NOS

M9758/3 Follicular dendritic cell sarcoma/tumor

M9760/3 Immunoproliferative disease, NOS

M9761/3 Waldenstrom macroglobulinemia (C42.0) (see also M9671/3)

M9762/3 Heavy chain disease, NOS

  • Alpha heavy chain disease
  • Mu heavy chain disease
  • Gamma heavy chain disease
  • Franklin disease

M9764/3 Immunoproliferative small intestinal disease (C17._)

  • Mediterranean lymphoma
  • Primary small intestinal extranodal marginal zone lymphoma

M9765/1 Monoclonal gammopathy of undetermined significance

  • MGUS
  • Monoclonal gammopathy, NOS

(M9766/1) Angiocentric immunoproliferative lesion

M9767/1 Angioimmunoblastic lymphadenopathy

M9768/1 T-gamma lymphoproliferative disease

M9769/1 Immunoglobulin deposition disease

  • Systemic light chain disease
  • Primary amyloidosis

980–994 Leukemias

[edit]

980) Leukemias, NOS

[edit]

M9800/3 Leukemia, NOS

M9801/3 Acute leukemia, NOS

  • Blast cell leukemia
  • Undifferentiated leukemia
  • Stem cell leukemia

M9805/3 Acute biphenotypic leukemia

(982–983) Lymphoid leukemias (C42.1)

[edit]

M9820/3 Lymphoid leukemia, NOS

M9823/3 B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (see also M9670/3)

  • Chronic lymphocytic leukemia, B-cell type (includes all variants of BCLL)
  • Chronic lymphocytic leukemia
  • Chronic lymphoid leukemia
  • Chronic lymphatic leukemia

M9826/3 Burkitt cell Leukemia (see also M9687/3)

  • Acute lymphoblastic leukemia, mature B-cell type

M9827/3 Adult T-cell leukemia/lymphoma (HTLV-1 positive) includes all variants

  • Adult T-cell lymphoma/leukemia

M9831/1 T-cell large granular lymphocytic leukemia

  • T-cell large granular lymphocytosis
  • NK-cell large granular lymphocytic leukemia
  • Large granular lymphocytosis, NOS

M9832/3 Prolymphocytic leukemia, NOS

M9833/3 B-cell prolymphocytic leukemia

M9834/3 T-cell prolymphocytic leukemia

M9835/3 Precursor cell lymphoblastic leukemia, NOS (see also M9727/3)

  • Precursor cell lymphoblastic leukemia, not phenotyped
  • Acute lymphoblastic leukemia, NOS (see also M9727/3)
  • Acute lymphoblastic leukemia, precursor-cell type
  • Acute lymphoblastic leukemia-lymphoma, NOS
  • Acute lymphocytic leukemia
  • Acute lymphoid leukemia
  • Acute lymphatic leukemia
  • Acute lymphoblastic leukemia, L2 type, NOS
  • FAB L2

M9836/3 Precursor B lymphoblastic leukemia (see also M9728/3)

  • Pro-B ALL
  • Common precursor B ALL
  • Pre-B ALL
  • Pre-pre-B ALL
  • Common ALL
  • c-ALL

M9837/3 Precursor T lymphoblastic leukemia (see also M9729/3)

  • Pro-T ALL
  • Pre-T ALL
  • Cortical T ALL
  • Mature T ALL

984–993 Myeloid Leukemias (C42.1)

[edit]

(9840–9849) Erythroleukemias (FAB-M6)

M9840/3 Acute myeloid leukemia, M6 type

(9850–9859) Lymphosarcoma cell leukemia (9860–9869) Myeloid (Granulocytic) Leukemias

M9860/3 Myeloid leukemia, NOS

  • Non-lymphocytic leukemia, NOS
  • Granulocytic leukemia, NOS
  • Myelogenous leukemia, NOS
  • Myelomonocytic leukemia, NOS
  • Myelocytic leukemia, NOS
  • Eosinophilic leukemia
  • Monocytic leukemia, NOS

M9861/3 Acute myeloid leukemia, NOS (FAB or WHO type not specified (see also M9930/3)

  • Acute myelogenous leukemia
  • Acute non-lymphocytic leukemia
  • Acute granulocytic leukemia
  • Acute myelogenous leukemia
  • Acute myelocytic leukemia

M9863/3 Chronic myeloid leukemia, NOS

M9866/3 Acute promyelocytic leukemia t(15;17)(q22;q11–12) * Acute promyelocytic leukemia, PML/RAR-alpha

  • Acute myeloid leukemia, t(15:17(q22;q11–12)
  • Acute promyelocytic leukemia, NOS

FAB-M3 (includes all variants)

M9867/3) Acute myelomonocytic leukemia

M9870/3 Basophilic leukemia or Acute basophilic leukemia

M9871/3 Acute myeloid leukemia with abnormal marrow eosinophils (includes all variants)

M9872/3 Acute myeloid leukemia, minimally differentiated (FAB type M0)

  • Acute myeloblastic leukemia]

M9873/3 Acute myeloid leukemia, without maturation (FAB type M1)

M9874/3 Acute myeloid leukemia, with maturation (FAB M2), NOS

M9875/3 Chronic myelogenous leukemia BCR/ABL positive

  • Philadelphia chromosome (Ph1 positive)
  • t(9;22)(q34;q11)
  • Chronic granulocytic leukemia (BCR/ABL positive)/(Ph1 positive)/t(9;22)(q34;q11)

M9876/3 Atypical chronic myelogenous leukemia BCR/ABL negative

  • Atypical chronic myeloid leukemia (BCR/ABL negative)/(Ph1 negative)

M9891/3 Acute monoblastic and monocytic leukemia

  • Monoblastic leukemia, NOS
  • FAB M5 (includes all variants)

M9895/3 Acute myeloid leukemia multilineage dysplasia

  • AML with/without prior myelodysplastic syndrome

M9896/3 AML with t(8;21)(q22;q22), AML1/ETO

  • FAB M2 with t(8;21)(q22;q22), AML1/ETO

M9897/3 AML with 11q23 (MLL) abnormalities

  • AML, MLL

M9910/3 Acute megakaryoblastic leukemia, NOS

  • Megakaryocytic leukemia
  • (FAB-M7)

M9920/3 Acute myeloid leukemia and myelodysplastic syndrome, therapy related, NOS Therapy-related acute myeloid leukemia, alkylating agent/epipodophyllotoxin related

M9930/3 Chloroma or Myeloid sarcoma (see also M9861/3)

  • Granulocytic sarcoma

M9931/3) Acute panmyelosis with myelofibrosis (C42.1)

  • Acute panmyelosis, NOS
  • Acute myelofibrosis
  • Acute myelosclerosis, NOS

994 Other Leukemias (C42.1)

[edit]

M9940/3 Hairy cell leukemia (C42.1)

  • Hairy cell leukemia variant
  • Leukemic reticuloendotheliosis

M9945/3 Chronic myelomonocytic, leukemia, NOS

  • Type 1
  • Type 2

M9946/3 Juvenile myelomonocytic leukemia

M9948/3 Aggressive NK cell leukemia

995–996 Chronic Myeloproliferative Disorders (C42.1)

[edit]

M9950/3 Polycythemia vera

  • Polycythemia rubra vera
  • Proliferative polycythemia

M9960/1 Chronic Myeloproliferative disease, NOS

M9961/3 Myelosclerosis with myeloid metaplasia

  • Chronic idiopathic myelofibrosis
  • Myelofibrosis as a result of myeloproliferative disease
  • Agnogenic myeloid metaplasia
  • Megakaryocytic myelosclerosis
  • Myelofibrosis with myeloid metaplasia

M9962/3 Essential thrombocytemia

  • Idiopathic thrombocythemia
  • Essential/idiopathic hemorrhagic thrombocythemia

M9963/3 Chronic neutrophilic leukemia

M9964/3 Chronic eosinophilic leukemia / hypereosinophilic syndrome

997 Other Haematologic Disorders

[edit]

M9970/1 Lymphoproliferative disease/disorder, NOS

M9975/3 Myeloproliferative disease, NOS

M9980/3 Chronic myelomonocytic leukemia or Refractory anemia

M9982/3 Refractory anemia with ringed sideroblasts

  • with sideroblasts

M9983/3 Refractory anemia with excess blasts

  • RAEB
  • RAEB I
  • RAEB II

M9985/3 Refractory cytopenia with multilineage dysplasia

M9986/3 Myelodysplastic syndrome associated with isolated del(5q) chromosome abnormality

M9989/3 Myelodysplastic syndrome, NOS

See also

[edit]

References

[edit]
[edit]
Revisions and contributorsEdit on WikipediaRead on Wikipedia
from Grokipedia
The International Classification of Diseases for Oncology (ICD-O) is a specialized coding system developed by the (WHO) to classify neoplasms based on their anatomical site (), histological type (morphology), behavior (benign, borderline/uncertain, in situ, or malignant), and degree of differentiation (grading). It serves as the global standard for data collection, enabling consistent recording of cancer diagnoses in reports, tumor registries, and epidemiological studies to facilitate incidence tracking, , and mortality statistics. First introduced in 1976 by the WHO, ICD-O evolved from earlier systems like the Manual of Tumor Nomenclature and Coding (MOTNAC) established by the in 1951, addressing the need for a unified framework beyond the general (ICD). The system's multi-axial structure includes a axis derived from the codes for malignant neoplasms (e.g., C00-C96 for primary sites), extended to cover benign and uncertain tumors, while the morphology axis employs five-digit alphanumeric codes (e.g., M-8000/3 for unspecified malignant ) with a slash-separated behavior digit (/0 for benign, /1 for borderline, /2 for , /3 for malignant). A separate one-digit code for grading (1-4, indicating differentiation level) further refines histological details. The current version, ICD-O-3, was published in 2000, with subsequent revisions including ICD-O-3.1 in 2013 (updating morphology codes for hematopoietic and lymphoid neoplasms to align with the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues) and ICD-O-3.2 in 2019 (incorporating further refinements for neurologic and other tumor types). Widely adopted by national and international cancer registries—such as those under the International Agency for Research on Cancer (IARC) and the European Network of Cancer Registries (ENCR)—ICD-O supports over 25 years of standardized data worldwide, with training resources available in 14 languages to promote global interoperability. In relation to the broader ICD framework, ICD-O's elements have been integrated into ICD-11's chapter on neoplasms and , enhancing compatibility for future applications while maintaining its role as the primary tool for legacy and specialized cancer coding.

History and Development

Origins and First Edition

The development of the International Classification of Diseases for Oncology (ICD-O) began in the late 1960s as a collaborative effort between the (WHO) and the International Agency for Research on Cancer (IARC), building on earlier WHO initiatives from the 1950s to improve cancer registration and classification. In 1968, IARC was specifically tasked by WHO to recommend revisions for the neoplasm chapter in the forthcoming ninth revision of the (ICD-9), which laid the groundwork for a specialized oncology extension. This collaboration addressed the limitations of general disease classifications in capturing detailed tumor characteristics, aiming to create a uniform system for international cancer data collection. A key influence on ICD-O was the American Cancer Society's Manual of Tumor Nomenclature and Coding (MOTNAC), first published in 1951 and revised in 1968, which provided foundational codes for tumor morphology and served as a precursor for standardizing descriptions. Prominent epidemiologist Calum S. Muir played a pivotal role in these origins; after joining IARC in 1966 as head of its unit, he co-founded the International Association of Cancer Registries (IACR) in 1966 to promote global cancer registration standards and actively contributed to the early development of ICD-O, including serving as an editor for its inaugural edition. Muir's work emphasized the need for precise, comparable data across registries, drawing from his experience in establishing cancer registration programs in and . The first edition, ICD-O-1, was published by WHO in 1976 as an offshoot of ICD-9, integrating a axis derived from ICD-9's malignant chapter to denote anatomic sites and a morphology axis adapted from established systems like the (SNOMED) to describe cell type, behavior, and grade. This dual-axis structure allowed for alphanumeric coding that distinguished between benign, , malignant, and uncertain s, marking a significant advancement over prior fragmented approaches. The primary purpose of ICD-O-1 was to standardize coding for population-based cancer registries, enabling consistent recording of incidence, , and data to support epidemiological research and planning worldwide.

Subsequent Editions and Updates

The second edition of the International Classification of Diseases for Oncology (ICD-O-2) was released in 1990 by the (WHO), building upon the first edition by aligning its topography codes more closely with the chapter of the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) to enhance consistency in global cancer reporting. This edition expanded the morphology code set, introducing additional terms for tumor histology while maintaining the dual-axis structure for site and characteristics, which facilitated broader adoption in cancer registries from 1992 onward. Subsequent interim additions included North American-specific lymphoma terms (1995–2000) and leukemia terms (1998–2000), marked for transitional use to reflect evolving pathological understandings without altering the core framework. The third edition (ICD-O-3) was published in 2000 by WHO, representing a comprehensive revision that significantly expanded classifications for hematopoietic and lymphoid s to align with the Revised European-American classification and the WHO's initial framework for these tumors. This update refined behavior codes, introducing a standardized fifth-digit system (/0 for benign, /1 for uncertain, /2 for , /3 for malignant primary, /6 for metastatic, and /9 for malignant uncertain primary/metastatic) to better capture tumor aggressiveness and site-specific malignancy. ICD-O-3 was dedicated to Dr. Calum Muir (1930–1995), a pivotal editor of the second edition and advocate for international cancer registry standards, whose contributions advanced coding worldwide. Interim updates to ICD-O-3 addressed emerging classifications, with the first revision (ICD-O-3.1) released in 2011 by the International Agency for Research on Cancer (IARC) and WHO (published 2013), focusing on morphology updates for lymphoid neoplasms to incorporate terms from the 2008 WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, such as new codes for B-cell and T-cell lymphomas without altering or core behavior schemes. The second revision (ICD-O-3.2), published in 2019 by IARC/WHO and recommended for implementation from 2020, introduced terms including codes for NTRK gene fusions (e.g., 8990/3 for NTRK-rearranged spindle cell neoplasm) and other fusion-positive entities, reflecting advances in precision oncology while ensuring compatibility with prior editions. Preparation for the fourth edition (ICD-O-4) commenced around 2021 under the IACR/WHO ICD-O , synchronized with the ongoing rollout of the fifth edition of the WHO Classification of Tumours (Blue Books), which began publication in 2021 and continues as of 2025 with volumes such as Endocrine Organs released in 2025. The consultation period for ICD-O-4 closed in May 2025, and as of November 2025, it awaits final publication, anticipating structural enhancements such as a fifth digit in morphology codes for greater specificity in molecular subtypes, with an international formed to guide global registry implementation and software adaptations.

Purpose and Applications

Role in Oncology Coding and Registries

The for Oncology (ICD-O) serves as the primary standard for coding the (anatomic site) and morphology (histologic type) of neoplasms in population-based cancer registries worldwide. It is extensively utilized by programs such as the Surveillance, Epidemiology, and End Results (SEER) program in the United States and the International Agency for Research on Cancer (IARC) databases, where it enables the systematic documentation of tumor characteristics derived mainly from reports. This standardized approach has been the cornerstone for cancer data collection for over 50 years, ensuring consistency in how tumors are classified across diverse healthcare settings. By providing a uniform coding framework, ICD-O facilitates meaningful comparisons of cancer incidence, rates, and temporal trends on a global scale. Registries employing ICD-O contribute to international datasets, such as those compiled by IARC's Global Cancer Observatory, allowing researchers to analyze variations in cancer patterns across regions and populations. For instance, this standardization underpins studies like the CONCORD program, which tracks worldwide outcomes for major cancers using registry data coded with ICD-O.70179-7/fulltext) Such comparability is essential for identifying risk factors, evaluating interventions, and informing policy decisions. ICD-O is mandated for reporting cancer cases to the (WHO) and various national health systems, integrating seamlessly with broader mortality and morbidity surveillance efforts. In the United States, for example, SEER requires its use for all reportable cases diagnosed since January 1, 2001, with updates like ICD-O-3.2 applied from 2021 onward. In pathology laboratories, ICD-O supports precise coding of diagnoses, aiding in the translation of microscopic findings into structured data for clinical documentation and research. Despite its strengths, ICD-O has notable limitations, as it is not designed for clinical staging of neoplasms; for that purpose, systems like the TNM classification are recommended instead. This focus on descriptive coding rather than prognostic staging ensures its role remains centered on epidemiological and registry applications.

Integration with Broader ICD System

The International Classification of Diseases for Oncology (ICD-O) functions as a specialized supplement to the broader (ICD) system, extending its capabilities for neoplasm coding while maintaining compatibility for global health data aggregation. Developed by the (WHO) and the International Agency for Research on Cancer (IARC), ICD-O builds upon the ICD's framework to address the unique needs of , particularly in distinguishing histological types and behaviors of tumors. ICD-O's topography axis directly aligns with the ICD-10 Chapter II (Neoplasms, codes C00-D48), adopting the same four-character alphanumeric structure to denote anatomic sites, but extends it to provide consistent coding for both malignant and non-malignant tumors, including greater site specificity for benign and borderline conditions not emphasized in . This mapping ensures seamless integration, as ICD-O topography codes can be converted to equivalents for general reporting, while excluding categories for secondary neoplasms and lymph node involvement to focus on primary sites. In contrast, the morphology axis is unique to ICD-O, employing five-digit codes (M-8000/0 to M-9989/3) to specify histological types, which the general ICD lacks, enabling detailed tumor characterization beyond mere site-based classification. A key differentiator is ICD-O's inclusion of behavior codes, appended as a fifth digit after the morphology code (/0 for benign, /1 for borderline or uncertain, /2 for , /3 for malignant primary, /6 for metastatic), which allow for nuanced distinctions in tumor aggressiveness and type—features absent in , where neoplasms are broadly categorized without histological or behavioral granularity. This enhancement supports precise data but requires mapping back to ICD categories for broader applications, such as distinguishing from invasive malignancies. In morbidity and mortality statistics, the general ICD serves as the standard for overall health coding and epidemiological reporting, while ICD-O is reserved for specialized contexts, such as cancer registries, where its detailed codes facilitate targeted analysis of incidence, survival, and treatment outcomes without disrupting aggregate ICD-based data flows. Updates to ICD-O are synchronized with ICD revisions to preserve ; for example, the third edition (ICD-O-3, published in 2000) was explicitly designed to harmonize with , and later revisions like ICD-O-3.2 (2019) incorporate nomenclature changes aligned with evolving ICD standards. With the transition to , integration has deepened: 's Chapter 2 (Neoplasms) retains the and ICD-O anatomical subdivisions at the three-character level, while fully incorporating ICD-O's morphology, grading, and behavior elements into its foundation, with post-coordination allowing combined codes for enhanced detail; an dedicated ICD-O view is also available in the browser for direct access. This evolution ensures continued compatibility, enabling oncology data from ICD-O to map fluidly into for comprehensive global health surveillance.

Classification Axes

Topography Axis

The topography axis in the (ICD-O) provides a standardized system for coding the anatomic site of origin of neoplasms, enabling precise identification of tumor locations across various body systems. This axis is essential for distinguishing the primary site of a tumor, which supports accurate in cancer registries and facilitates epidemiological studies on cancer incidence and distribution. Unlike other classification components, the topography axis focuses exclusively on spatial localization, independent of the tumor's cellular characteristics. Topography codes consist of four alphanumeric characters, typically structured as a letter followed by three digits, such as for breast, unspecified. These codes are derived from the malignant section (C00-C80) of the , 10th Revision (). This structure is used for all neoplasms, with the behavior (benign, , malignant, etc.) specified separately in the morphology code to enhance site specificity for purposes while maintaining compatibility with the broader ICD framework. The hierarchy of topography codes is organized anatomically, progressing from sites in the head and neck (C00-C14, covering , oral cavity, and ) through various organ systems to hematopoietic and reticuloendothelial tissues (C42), ill-defined sites (C76), and unknown primary sites (C80). This sequential arrangement allows for systematic categorization, starting with cranial and facial regions and extending to systemic or unspecified locations, which aids in compiling comprehensive cancer statistics. The primary purpose of the topography axis is to specify the site of the primary for tracking cancer patterns, incidence rates, and outcomes in population-based registries, thereby supporting and research. Notably, it excludes coding for metastatic or secondary sites, which are instead designated using M codes in supplementary systems to avoid conflation with primary tumor data.

Morphology Axis

The Morphology Axis of the for (ICD-O) provides a standardized system for classifying the histological type and microscopic structure of neoplasms, enabling precise description of tumor cell characteristics independent of their anatomic location. This axis is essential for coding, , and registry , as it captures the cellular and tissue-level features that define tumor and guide clinical . Morphology codes in ICD-O are structured as five-digit alphanumeric identifiers, ranging from 8000/0 to 9989/3, where the first four digits specify the histologic type (e.g., 8140 for ) and the fifth digit, separated by a slash, denotes the behavior code (/0 for benign, /1 for uncertain, /2 for , /3 for malignant). For instance, the code 8140/3 indicates a malignant . These codes encompass over 1,000 terms covering a wide spectrum of neoplasms, from epithelial carcinomas to mesenchymal tumors and hematopoietic malignancies. The morphology classification is derived from the World Health Organization's (WHO) International Histological Classification of Tumours series, particularly the "Blue Books," and the Systematized Nomenclature of Medicine (SNOMED) neoplasm section, with historical roots in the American Cancer Society's Manual of Tumor Nomenclature and Coding (MOTNAC) from 1951 and the Systematized Nomenclature of Pathology (SNOP) from 1965. This foundation ensures compatibility with broader pathological standards, and SNOMED has adopted the ICD-O morphology codes for enhanced interoperability in medical informatics. Updates to the Morphology Axis, such as those in ICD-O-3.1 (2013) and ICD-O-3.2 (2019), incorporate advances in , including cytogenetic and genetic criteria like translocations (e.g., t(9;22) in chronic myeloid leukemia) and mutations (e.g., in ), reflecting revisions from the WHO's 5th edition tumor classifications. These enhancements allow for more nuanced coding of molecularly defined entities, particularly in hematopoietic and lymphoid neoplasms, without altering the core five-digit structure.

Behavior and Grade Codes

The behavior codes in the International Classification of Diseases for Oncology (ICD-O) are represented by a fifth digit appended to the morphology code, indicating the neoplastic of the tumor. These codes were introduced in the first edition of ICD-O in as part of the multi-axial system to classify neoplasms by site, , and , and have been refined in subsequent editions to align with evolving pathological understanding. The possible values are /0 for benign tumors, /1 for borderline or uncertain whether benign or malignant, /2 for (non-invasive) neoplasms, /3 for malignant (invasive) neoplasms, and /6 for metastatic neoplasms (used rarely for sites of origin unknown). These behavior codes align with the standard classification in oncology and pathology of neoplasms as benign, malignant, and borderline (also known as intermediate or uncertain), based on their biological behavior, growth characteristics, invasiveness, and potential for metastasis. All malignant neoplasms are assigned the default code of /3 unless specified otherwise, facilitating the compilation of cancer incidence statistics stratified by type. Grade codes in ICD-O provide a separate one-digit assessment of tumor differentiation, applied after the morphology and behavior codes to describe how closely tumor cells resemble normal cells of the tissue of origin. The values range from 1 for well-differentiated (low-grade) tumors, 2 for moderately differentiated, 3 for poorly differentiated, and 4 for undifferentiated or anaplastic (high-grade) tumors, with 9 indicating unknown or not applicable. These codes emphasize histologic grading rather than proliferative activity, supporting prognostic and epidemiological analyses. In the third edition (ICD-O-3), behavior codes for hematopoietic and lymphoid neoplasms were expanded, with all lymphomas and leukemias classified as malignant (/3) to reflect their inherent neoplastic potential and improve reportability in cancer registries. This update, effective from , addressed prior ambiguities in borderline designations for conditions like certain myeloproliferative disorders, ensuring consistent coding for statistical purposes. The full morphology code integrates the behavior digit (e.g., 8000/3), enabling precise tumor characterization across the classification system.

Topography Coding Details

Anatomic Site Categories

The axis in the for (ICD-O) organizes anatomic sites into categories that reflect major organ systems and body regions, enabling precise localization of neoplasms for cancer registration and . These categories draw directly from the ICD-10's Chapter II codes for malignant neoplasms (C00-D48), but ICD-O extends their application to all tumor types, including benign and lesions, while adding subsite detail not always present in ICD-10. This alignment facilitates integration with broader diagnostic systems, though ICD-O emphasizes oncology-specific precision, such as distinguishing subsites within organs. The major categories encompass primary sites across the body, with codes structured as a letter (C or D) followed by two digits and a subsite digit after a decimal (e.g., C15.2). Hematopoietic and lymphoid tissues receive a dedicated category (C42), reflecting their unique diffuse nature in . Ill-defined sites are grouped separately to handle cases where precise localization is unavailable. Special considerations apply to overlapping or paired sites, such as long bones of the lower limb (C40.2), which may involve bilateral coding rules in registries.
CategoryCode RangeDescriptionSubsite Example
Lip, oral cavity, and pharynxC00-C14Encompasses the lips, tongue, gums, other mouth parts, major salivary glands, tonsils, oropharynx, nasopharynx, and hypopharynx.C00.0 (External upper lip)
Digestive organsC15-C26Includes the esophagus, stomach, small and large intestines, liver, gallbladder, pancreas, and other digestive structures.C15.2 (Cardia of stomach)
Respiratory system and intrathoracic organsC30-C39Covers nasal cavity, middle ear, sinuses, larynx, trachea, bronchi, lungs, pleura, thymus, heart, and mediastinum.C34.0 (Main bronchus)
Bones, joints, and articular cartilageC40-C41Addresses bones and joints of the limbs, head, trunk, and other/unspecified skeletal sites.C40.2 (Long bones of lower limb)
Mesothelial and soft tissueC45Includes sites specific to mesotheliomas such as pleura, peritoneum, pericardium, and tunica vaginalis.C45.0 (Pleura)
Kaposi sarcomaC46Sites of Kaposi sarcoma including skin, palate, gastrointestinal tract, lung, and lymph nodes.C46.0 (Skin)
Peripheral nerves and autonomic nervous systemC47Covers sympathetic and parasympathetic nerves and ganglia, peripheral nerves of head, neck, and other sites.C47.0 (Sympathetic nervous system)
PeritoneumC48Includes peritoneum, retroperitoneum, and specified parts of peritoneum.C48.1 (Specified part of peritoneum)
Connective and soft tissueC49Encompasses connective and soft tissue of head, face, neck, upper/lower limbs, thorax, abdomen, pelvis, and other sites.C49.0 (Connective tissue of head, face and neck)
SkinC44Specifies skin of the eyelid, scalp, face, neck, trunk, upper/lower limbs, and other/unspecified areas.C44.2 (Skin of ear and external auricular canal)
BreastC50Details quadrants and regions of the female and male breast, including nipple and areola.C50.1 (Central portion of breast)
Female genital organsC51-C58Includes vulva, vagina, cervix uteri, corpus uteri, uterus (NOS), placenta, and ovary.C53.1 (Exocervix)
Male genital organsC60-C63Covers penis, prostate, testis, epididymis, and other male genital sites.C61.9 (Prostate gland)
Urinary organsC64-C68Encompasses kidney, renal pelvis, ureter, bladder, and urethra.C65.9 (Renal pelvis)
Eye and central nervous systemC69-C72Includes eye (orbit, retina, choroid), meninges, brain, spinal cord, and cranial nerves.C71.0 (Cerebrum)
Endocrine glands and related structuresC73-C75Addresses thyroid gland, adrenal gland, pituitary, pineal gland, and other endocrine sites.C73.9 (Thyroid gland)
Lymphoid, hematopoietic, and related tissueC42Specifies bone marrow, spleen, thymus, lymph nodes, and other hematopoietic sites.C42.1 (Bone marrow)
Other and ill-defined sitesC76-C80Covers unspecified sites of head/neck, thorax, abdomen, pelvis, limbs, and unknown primary.C76.0 (Head, face, and neck)

Coding Rules and Conventions

Coding rules for in the (ICD-O) emphasize assigning the most precise anatomic site based on available clinical and pathological information to ensure accurate tumor registration and epidemiological analysis. Coders must select the most specific subsite possible, even if the tumor extends to adjacent areas, using all diagnostic details from medical records such as imaging, reports, and physician notes. For instance, a tumor originating in the upper lobe is coded as C34.1 rather than the broader C34.9 for lung not otherwise specified (NOS). This rule promotes granularity in for cancer . When the primary site cannot be identified despite thorough review, the code C80.9 (unknown primary site) is used as a default, applicable to cases where metastases are present but the origin remains undetermined. This code serves as a last resort and is not intended for tumors with any identifiable location, even if ill-defined. In ambiguous situations, such as overlapping lesions or conflicting diagnostic evidence, may guide site assignment; for example, a melanoma-like morphology might direct coding to sites (C44) if no other site is documented, though explicit clinical site descriptions take precedence over histology-suggested locations. Conventions for paired organs and address symmetry in anatomic structures to enhance data precision in registries. For paired sites like the , the external upper is specifically coded as C00.0, distinguishing it from internal or subsites. is indicated separately in registry data items (e.g., 1 for right, 2 for left, 9 for not applicable) rather than embedded in the ; for tumors, a like C50.1 ( and ) would pair with 1 to specify the right . These conventions apply to organs such as lungs, kidneys, and ovaries, ensuring bilateral cases are distinguished without altering the core structure. Updates across ICD-O editions have refined topography coding for emerging clinical needs. The third edition (ICD-O-3), published in 2000 and updated through 2019, retained the four-character format (C followed by three digits with decimal) but incorporated alignments, adding specificity for sites like the (C48.0-C48.2) to better capture intra-abdominal malignancies previously grouped under broader categories. Common errors in topography coding often stem from conflating surface origins, such as misassigning squamous cell carcinomas from skin (C44) to mucosal sites (C00-C14) or vice versa, which can distort incidence statistics for epithelial tumors. Another frequent issue involves overusing NOS or overlapping codes (.8 or .9) when specific subsites are inferable from , leading to loss of detail. To mitigate these, coders should pathologic descriptions carefully. Validation of topography assignments relies on standardized tools like the SEER Site/ Validation Lists, which flag invalid combinations (e.g., certain histologies incompatible with specific sites) to promote consistency across registries. Updated annually, these lists, now integrated into the Cancer PathCHART system, require review for non-listed pairings, ensuring data quality for research and policy. For cases diagnosed on or after January 1, 2025, the 2025 Cancer PathCHART ICD-O-3 Site Morphology Validation List supersedes prior versions, incorporating new codes and behaviors.

Morphology Coding System

Code Structure and Principles

The morphology codes in the for (ICD-O) follow a standardized alphanumeric format of M-XXXX/X, where the prefix "M-" identifies the morphology axis, XXXX represents a four-digit numeric code specifying the histological term (ranging from 8000 to 9989), and the suffix "/X" denotes a single-digit behavior code (0 for benign, 1 for uncertain, 2 for , or 3 for malignant). This structure facilitates the precise description of a neoplasm's cellular origin and biologic potential within tumor registries and reporting. The foundational principles of the morphology coding system emphasize and specificity to promote accurate and consistent . Codes are organized hierarchically, with broad categories like 8000/0 (, NOS) serving as defaults only when more detailed diagnoses are unavailable; registrars and are required to select the most specific code possible based on available diagnostic information, avoiding NOS terms unless necessary. Synonyms for histological terms are systematically included in the to accommodate variations in nomenclature and ensure comprehensive coverage. The system is derived directly from the World Health Organization's (WHO) International Histological Classification of Tumours series, with updates incorporating advances in , such as new insights from molecular and genetic studies. In the ICD-O-3 edition, there are approximately 1,100 defined morphology codes, providing a robust framework for classifying diverse while allowing for expansion in subsequent revisions like ICD-O-3.2. Morphology codes are assigned by pathologists or certified cancer registrars, relying on key features from reports including microscopic cellular patterns, gross tumor characteristics, and ancillary tests like . This assignment process prioritizes conceptual understanding of tumor differentiation and type to support epidemiological analysis, , and standardized data collection across global registries.

Behavior Code Integration

In the International Classification of Diseases for Oncology (ICD-O), the behavior code is integrated into the morphology classification as the fifth digit of the five-digit morphology code, forming a complete histological designation that specifies both the cell type and the neoplasm's biological activity. For instance, the code 8140/3 denotes , not otherwise specified (NOS), with malignant behavior, where /3 indicates invasiveness and potential to metastasize. This appended structure allows for a unified representation of and behavior, enabling precise coding from reports without requiring separate axes for these elements. Defaults for behavior are often implied by the morphological term itself; for example, terms like "" or "" default to /3 (malignant), while "" typically defaults to /0 (benign), though coders may override based on explicit pathological descriptions to ensure accuracy. Special rules govern behavior coding for certain neoplasm types to maintain consistency across classifications. Hematopoietic and lymphoid neoplasms are uniformly assigned /3 (malignant) behavior in ICD-O-3, regardless of specific subtype, reflecting their inherent malignant potential as per the (WHO) classification. For epithelial neoplasms, in situ lesions—confined to the without invasion—are coded with /2 () behavior, distinguishing them from invasive malignancies (/3). These rules ensure standardized application, particularly in registries where behavior determines reportability. Historical developments have refined behavior code integration. The second edition (ICD-O-2) introduced the /1 (borderline or uncertain malignant potential) code to address neoplasms with intermediate behavior, such as certain ovarian or tumors, which were previously ambiguously coded as /0 or /3. The third edition (ICD-O-3) further standardized this for lymphomas by aligning morphology codes with the 2001 WHO Classification of Hematopoietic and Lymphoid Tissues, consolidating terms and ensuring all such neoplasms receive /3 behavior to reflect updated pathological understanding. In cancer statistics and , behavior codes serve as a critical filter for incidence and mortality reporting; only /2 () and /3 (malignant) neoplasms are generally reportable to population-based registries, excluding most /0 (benign) and /1 (borderline) cases except for specific sites like the . This integration facilitates global comparisons of cancer burden by distinguishing clinically significant tumors. Although grading—assessed via a separate one-digit indicating differentiation (e.g., 1 for well-differentiated, 3 for poorly differentiated)—is distinct from , the two are often paired in practice; for example, high-grade tumors frequently imply /3 and poor differentiation, aiding in prognostic assessments.

Epithelial Neoplasms

Neoplasms NOS and Epithelial NOS

In the for (ICD-O-3), the Neoplasms NOS category, designated by codes 8000-8005, serves as a foundational set for classifying tumors lacking identifiable histological specificity. These codes apply to s where the tissue of origin or cellular characteristics remain undetermined, allowing for benign (/0), uncertain (/1), or malignant (/3) behaviors. For instance, code 8000/0 denotes a benign , while 8000/3 indicates a malignant without further subtype details, and 8000/6 is reserved for metastatic sites of unspecified malignant s. Similarly, 8001/3 codes malignant tumor cells of unknown type, and specialized variants like 8002/3 (malignant tumor, small cell type) or 8003/3 (malignant tumor, type) capture basic morphological features when more precise is unavailable. This category emphasizes provisional coding, with registrars encouraged to update to specific morphologies upon additional diagnostic information. The Epithelial NOS category, spanning codes 8010-8041, addresses carcinomas of epithelial origin that defy further histological differentiation. Code 8010/3 represents NOS, a broad term for malignant epithelial tumors without specified subtype, applicable across various sites such as or when reports lack detail on glandular or squamous features. In situ variants are captured under 8010/2 (, NOS), while 8011/3 (epithelioma, malignant) denotes general malignant epithelial growths. More descriptive yet still non-specific codes include 8020/3 (, undifferentiated type, NOS), used for tumors showing no , and 8012/3 (large cell , NOS) for those with large, undifferentiated cells. Additional codes like 8031/3 ( ) or 8041/3 (, NOS) incorporate rudimentary cytological traits, such as cell size or shape, without implying lineage-specific diagnoses. These codes integrate behavior modifiers, including /6 for metastatic epithelial tumors of unknown subtype. Guidelines in ICD-O-3 stress the use of these NOS codes only as a temporary measure when is unknown or non-specific, urging avoidance in favor of more precise alternatives to enhance data accuracy for and . For example, a describing "metastatic " without primary site or type details would employ 8000/6, but subsequent might reclassify it to a specific code like 8140/3 (, NOS). In the third edition, updates to this range were limited, primarily adding terms like 8014/3 (large cell with rhabdoid ) to reflect emerging morphological recognitions, while reinforcing the principle of maximal specificity through validation lists that restrict invalid site- combinations. The overall framework promotes conceptual clarity by prioritizing histological detail over generic categorization, ensuring NOS usage supports rather than hinders tumor registry integrity.

Squamous, Basal, and Transitional Cell Neoplasms

The squamous, basal, and transitional cell neoplasms category in the for (ICD-O) encompasses morphology codes 8050 through 8139, which classify tumors arising from specific epithelial tissues characterized by their and origin. These codes are part of the broader morphology axis in ICD-O-3, where the first four digits denote the histological type, and the fifth digit indicates (/0 for benign, /1 for uncertain borderline, /2 for , /3 for malignant, /6 for metastatic). Squamous neoplasms (codes 8050-8089) primarily originate from lining the skin and mucosal surfaces, basal cell neoplasms (8090-8119) derive from basal cells of the or adnexal structures, and transitional cell neoplasms (8120-8139) emerge from the , most commonly the urothelium of the urinary tract. This grouping emphasizes non-glandular epithelial tumors, distinguishing them from glandular variants covered elsewhere. Further refinements in the 2024 ICD-O-3.2 update included new terms for HPV-associated squamous cell s (e.g., 8085/3) and specific urothelial variants (e.g., 8070/3 pure squamous carcinoma of urothelial tract, 8120/3). Squamous cell neoplasms, coded under 8050-8089, include a range of lesions from benign papillomas to invasive , often exhibiting keratinization and intercellular bridges as key histological features. Representative examples include 8051/3 for verrucous carcinoma, a well-differentiated, locally invasive tumor with a warty appearance typically affecting oral or genital mucosa, and 8070/3 for , NOS, the most common malignant form arising in sun-exposed or aerodigestive tract mucosa, characterized by squamous cells with keratin pearl formation. Other variants, such as 8072/3 large cell keratinizing and 8074/3 spindle cell , highlight degrees of differentiation and stromal interaction, with the latter showing sarcomatoid features. These tumors frequently present with malignant behavior (/3), though forms like 8070/2 are coded separately to reflect non-invasive intraepithelial neoplasia. Basal cell neoplasms, assigned codes 8090-8119, typically originate from the basal layer of the or hair follicle adnexa, predominantly in the skin, and are known for their slow growth and low metastatic potential despite local invasiveness. The prototypical example is 8090/3 , NOS, accounting for the majority of non-melanoma skin cancers, featuring nests of uniform basaloid cells with peripheral palisading and retraction artifact, often linked to exposure. Variants such as 8091/3 multifocal superficial basal cell carcinoma describe superficial multicentric growth patterns, while 8094/3 basosquamous carcinoma combines basaloid and squamous differentiation, indicating a more aggressive clinical course. Benign counterparts like 8090/0 basal cell papilloma are rare and confined to specific sites, but most lesions in this group are coded as malignant (/3) due to their propensity for local recurrence. Transitional cell neoplasms, coded 8120-8139, arise from the urothelium lining the urinary bladder, ureters, and , reflecting the unique capable of stretching. Key examples include 8120/3 transitional cell carcinoma, NOS (also termed urothelial carcinoma), the predominant malignancy of the urinary tract, displaying papillary or flat growth patterns with atypical transitional cells invading the . Specialized variants encompass 8130/3 papillary transitional cell carcinoma, often presenting as exophytic frond-like projections, and 8120/2 for transitional cell carcinoma in situ, a flat high-grade lesion confined to the . Low-malignant-potential tumors, such as 8130/1 papillary transitional cell neoplasm of low malignant potential, indicate less aggressive behavior with minimal . These neoplasms are overwhelmingly malignant (/3) when invasive, with behavior codes integrating site-specific prognostic implications, such as multifocality in the bladder. The ICD-O-3 edition, published in 2000 and updated through ICD-O-3.2 in 2019, introduced or refined several codes in this category to better capture histological variants and molecular associations, such as 8051/3 for verrucous carcinoma to distinguish its indolent progression from conventional squamous types. Behavior code integration remains consistent across these groups, with /2 reserved for lesions to facilitate staging and treatment planning, while malignant /3 codes dominate due to the invasive nature of most clinically significant tumors. This classification supports standardized cancer registry data collection, emphasizing histological precision for epidemiological and research purposes.

Adenomas, Adenocarcinomas, and Glandular Variants

Adenomas and represent a major category of epithelial neoplasms characterized by glandular differentiation, classified within the morphology codes 8140–8499 of the for Oncology, Third Edition (ICD-O-3). These codes encompass benign (typically coded with behavior /0 or /1), such as adenoma NOS (8140/0), and malignant adenocarcinomas (behavior /3), exemplified by adenocarcinoma NOS (8140/3), which is the most common histological type across various organs. The subrange 8140–8389 primarily covers adenomas and conventional adenocarcinomas, while 8390–8499 includes specialized glandular variants like cystic, mucinous, and serous tumors, such as mucinous cystadenocarcinoma (8470/3). Key variants within this category include ductal adenocarcinoma (8500/3), which features glandular structures resembling normal ducts and is prevalent in sites like the breast and ; lobular carcinoma (8520/3), distinguished by its infiltrative, non-cohesive pattern and common in tissue; and medullary carcinoma (8510/3), noted for its lymphoid stroma and association with genetic mutations in organs such as the breast and . Papillary adenocarcinoma (8260/3) and ous adenocarcinoma (8480/3) are also prominent, with the former showing finger-like projections and the latter abundant extracellular production. These neoplasms are most frequently encountered in glandular tissues of the (e.g., colorectal ), breast (e.g., ductal and lobular types), and ovaries (e.g., mucinous and serous cystadenocarcinomas). In ICD-O-3, released in 2000, refinements were introduced to better capture subtypes like papillary and mucinous variants, enhancing diagnostic precision for registry reporting and research. Updates in subsequent revisions, such as ICD-O-3.2 (effective 2021), further specified site-specific applications, including for lung mucinous adenocarcinomas (8253/3).

Skin Appendage and Specialized Epithelial Neoplasms

The International Classification of Diseases for Oncology (ICD-O) assigns morphology codes in the range 8390–8589 to and specialized epithelial neoplasms, encompassing tumors derived from adnexal structures of the skin, as well as more complex epithelial proliferations involving mucoepidermoid, ductal, lobular, and thymic origins. These codes integrate histological subtypes with behavior designations (/0 for benign, /1 for borderline or uncertain, /2 for , /3 for malignant, and /6 for metastatic), reflecting the diverse biological potential of these lesions, which often arise from glandular or thymic epithelial elements and exhibit a predominance of benign or low-grade behaviors compared to more aggressive epithelial carcinomas. This grouping distinguishes them from general glandular neoplasms by emphasizing specialized differentiation patterns, such as those mimicking or thymic components. Adnexal and skin appendage neoplasms (codes 8390–8429) primarily involve tumors of sweat glands, sebaceous glands, and hair follicles, typically occurring in the or subcutis of the skin (topography C44). These are often benign or borderline lesions, though malignant counterparts exist; for instance, 8410/0 denotes , a well-circumscribed proliferation of lipid-laden epithelial cells, while 8410/3 represents sebaceous , characterized by infiltrative growth and . Similarly, sweat gland tumors include 8400/0 for sweat gland and 8400/3 for sweat gland , highlighting the spectrum from indolent cystic or papillary (e.g., 8402/0 nodular hidradenoma) to rare aggressive variants like 8407/3 sclerosing sweat duct carcinoma, which shows ductal differentiation with sclerosis. These neoplasms are specialized in that they recapitulate embryonic development, often presenting as slow-growing nodules with low metastatic potential unless high-grade features are present. Mucoepidermoid and cystic neoplasms (codes 8430–8499) feature mixtures of mucous, epidermoid, and intermediate cells, frequently forming cystic structures in salivary glands, thyroid, or other mucosal sites. A representative example is 8430/3 mucoepidermoid carcinoma, graded by cystic component and mitoses, which can behave indolently in low-grade forms but aggressively in high-grade ones with solid growth. Cystic variants, such as 8440/0 cystadenoma, NOS (benign) and 8440/3 cystadenocarcinoma, NOS (malignant), emphasize papillary or serous/mucinous linings, while borderline tumors like 8472/1 mucinous cystic tumor of borderline malignancy underscore the uncertain behavior common in this category. These lesions derive from specialized epithelial linings of ducts or cysts, with a notable propensity for encapsulation in benign cases, distinguishing them from diffuse glandular adenocarcinomas. Ductal, lobular, and complex epithelial neoplasms (codes 8500–8579) cover intricate breast-like or salivary ductal patterns, including in situ and invasive forms, often in (C50) or salivary gland (C07–C08) topographies. Key examples include 8500/3 infiltrating duct carcinoma, NOS, the most common invasive subtype with tubular differentiation, and 8520/2 , a borderline proliferation confined to lobules without stromal . Complex variants like 8560/3 exhibit cribriform or tubular patterns with basement membrane material, typically arising in salivary glands and showing perineural despite an otherwise indolent course. Metaplastic forms, such as 8575/3 metaplastic , NOS, incorporate squamous or spindle cell elements, reflecting epithelial-mesenchymal transitions but remaining classified here due to their epithelial origin. These tumors are characterized by their architectural complexity and variable hormone responsiveness, with many borderline or lesions managed conservatively. Thymic neoplasms (codes 8580–8589) represent specialized epithelial tumors of the (C37), blending epithelial and lymphoid components in a mediastinal context. Thymomas, such as 8580/1 , NOS (borderline), are encapsulated with varying lymphocytic infiltration, while malignant subtypes like 8580/3 , malignant, exhibit capsular invasion. Refined classifications include 8586/3 thymic , NOS, a frankly invasive squamous or undifferentiated tumor with poor , and 8588/3 spindle epithelial tumor with thymus-like element, a rare pediatric variant with low-grade behavior. These lesions are unique for their association with autoimmune disorders like in benign forms, highlighting thymic epithelial specialization.

Mesenchymal and Soft Tissue Neoplasms

Soft Tissue Tumors NOS and Fibromatous Neoplasms

The Soft Tissue Tumors NOS and Fibromatous Neoplasms category in the for (ICD-O-3) addresses neoplasms arising from connective and soft tissues that lack specific differentiation or primarily exhibit fibrous characteristics. These are classified using morphology codes ranging from 8800 to 8839, with myxomatous variants incorporated under 8840 to 8849, allowing for precise histological documentation in cancer registries. This grouping facilitates the coding of undifferentiated or fibrous-predominant tumors, emphasizing their site-independent morphology while integrating behavior codes to denote benign (/0), borderline or uncertain (/1), or malignant (/3) potential. Codes 8800 through 8804 pertain to soft tissue tumors not otherwise specified (NOS), including sarcomas of undetermined type. For instance, 8800/3 designates sarcoma, NOS, or soft tissue sarcoma, representing malignant mesenchymal tumors without identifiable lineage, often used when diagnostic material is limited or the tumor shows no distinct features. Benign counterparts, such as 8800/0 for soft tissue tumor, benign, apply to non-invasive lesions, while 8800/1 covers uncertain cases. These codes underscore the importance of behavior integration, as undifferentiated sarcomas like 8801/3 (spindle cell sarcoma) or 8802/3 (giant cell sarcoma) are inherently malignant and require aggressive management. ICD-O-3 updates refined these to exclude outdated terms, promoting consistency in reporting. Fibromatous neoplasms, spanning codes 8810 to 8839, encompass tumors dominated by fibroblastic proliferation, ranging from indolent to aggressive entities. Representative examples include 8810/0 for , NOS, a benign, well-circumscribed fibrous growth commonly arising in subcutaneous tissues, and 8810/3 for , a malignant tumor characterized by spindle-shaped fibroblasts in a collagenous stroma, historically overdiagnosed but now reserved for specific molecularly confirmed cases. Intermediate behaviors are clarified in ICD-O-3, such as 8811/1 for (desmoid-type), a locally invasive but non-metastasizing often linked to APC mutations, highlighting the system's evolution to incorporate borderline categories for better prognostic stratification. Other fibromatous types, like 8821/1 for abdominal fibromatosis, further illustrate the mix of behaviors within this range. Myxomatous neoplasms, integrated via codes 8840 to 8849, feature mucoid or gelatinous matrix due to abundant extracellular mucopolysaccharides, often overlapping with fibromatous elements. Key terms include 8840/0 for , NOS, a typically intracardiac or intramuscular, and 8840/3 for myxosarcoma, its malignant analog with potential for . ICD-O-3 delineates these to distinguish pure myxoid from mixed forms, such as 8841/1 for aggressive angiomyxoma, an uncertain borderline tumor predominantly in pelvic sites of females, emphasizing histological and behavioral nuance for registry accuracy. Overall, this category's codes support a spectrum of behaviors, with ICD-O-3 clarifications enhancing across global tumor classifications. ICD-O-3.2 (2020) introduced updates including new codes for malignant variants like ossifying fibromyxoid tumor (8842/3).

Lipomatous, Myomatous, and Mixed Stromal Neoplasms

Lipomatous neoplasms encompass tumors originating from adipose (fat) tissue and are assigned morphology codes 8850–8889 in the ICD-O-3 classification. These include both benign and malignant entities, with the behavior code indicating malignancy (/3 for malignant, /0 for benign). Representative benign examples include lipoma, NOS (8850/0), a common subcutaneous tumor composed of mature adipocytes, and fibrolipoma (8851/0). Malignant variants, such as liposarcoma, NOS (8850/3), represent the most frequent soft tissue sarcoma in adults and exhibit diverse subtypes like myxoid liposarcoma (8852/3), characterized by a chicken-wire vascular pattern and round cell liposarcoma (8853/3), known for its aggressive behavior. Dedifferentiated liposarcoma (8858/3) often arises from well-differentiated forms and shows non-lipogenic sarcomatous components. These tumors frequently occur in deep soft tissues of the extremities or retroperitoneum and are diagnosed based on histological features like lipoblasts and chromosomal abnormalities such as MDM2 amplification. ICD-O-3.2 (2020) added 8859/3 for myxoid pleomorphic liposarcoma. Myomatous neoplasms involve tumors of smooth or striated muscle origin and are coded 8890–8929, reflecting their mesenchymal derivation with integrated behavior codes to denote benignity (/0), uncertainty (/1), or malignancy (/3). Leiomyoma, NOS (8890/0), exemplifies a benign tumor, commonly presenting as uterine fibroids that are hormone-dependent and affect up to 70% of women by age 50, often managed conservatively or surgically due to symptoms like heavy menstrual bleeding. Its malignant counterpart, leiomyosarcoma, NOS (8890/3), arises de novo or from malignant transformation and is marked by nuclear atypia, mitoses exceeding 10 per 10 high-power fields, and necrosis; it predominantly affects the uterus or gastrointestinal tract with poor prognosis if metastatic. Epithelioid leiomyosarcoma (8891/3) features rounded cells with eosinophilic cytoplasm, while rhabdomyosarcoma variants like embryonal (8910/3) occur in children and show strap cells. These neoplasms are site-specific, with uterine leiomyomas being the most prevalent benign mesenchymal tumors in that organ. Mixed stromal neoplasms combine elements of connective tissue, muscle, or epithelial components and fall under codes 8930–8999, emphasizing their heterogeneous and behavior coding for clinical relevance. Endometrial stromal sarcoma, low grade (8931/3), is a key malignant example, typically with uniform spindle cells infiltrating , often associated with JAZF1-SUZ12 fusion; high-grade variants (8930/3) exhibit more and YWHAE-NUTM2 fusions, leading to worse outcomes. Benign counterparts include endometrial stromal nodule (8930/0), a circumscribed uterine mass without invasion. Adenomyoma (8932/0) represents a mixed glandular and stromal proliferation, commonly in the or . Perivascular epithelioid cell tumors (PEComas), such as benign (8860/0) in the , feature and markers, while malignant PEComa (8714/3) shows TSC1/2 alterations (ICD-O-3.2 update). These tumors are frequently gynecologic, with endometrial stromal neoplasms showing positivity and targeted therapies like for advanced cases. Fibroepithelial neoplasms, coded 9000–9039, involve intermixed fibrous stroma and epithelial elements, often in or gynecologic sites, with behavior codes distinguishing benign, borderline, and malignant forms. , NOS (9010/0), is a common benign tumor in young women, featuring pericanalicular or intracanalicular patterns without . (9020 series) spans the spectrum: benign (9020/0), borderline (9020/1), and malignant (9020/3), characterized by leaf-like stromal projections and epithelial slits; malignant cases show sarcomatous overgrowth, high mitotic activity (>10/10 HPF), and 5-year recurrence rates up to 40%, treated with wide excision. These neoplasms are site-specific, predominantly affecting , where phyllodes tumors comprise 0.3–1% of all fibroepithelial lesions and require stromal grading for .

Vascular, Osseous, and Chondromatous Neoplasms

The International Classification of Diseases for Oncology (ICD-O) categorizes vascular neoplasms under codes 9120-9169 for blood vessel tumors and 9170-9179 for lymphatic vessel tumors, encompassing a spectrum of benign, borderline, and malignant lesions arising from and pericytic cells. These include common benign entities such as NOS (9120/0), characterized by proliferations of vascular channels often seen in soft tissues or , and (9121/0), which features dilated blood-filled spaces. Malignant counterparts, like or (9120/3), represent aggressive endothelial malignancies with poor prognosis, frequently associated with sites such as the liver or soft tissues, while (9133/3) denotes a borderline to low-grade malignant tumor with epithelioid endothelial cells. Lymphatic variants, such as (9170/3), are rare and typically post-radiation or post-mastectomy malignancies involving lymphatic . Osseous neoplasms, coded 9180-9195, primarily involve bone-forming tissues and range from benign osteoma NOS (9180/0), a compact or cancellous bone overgrowth often incidental in the skull or sinuses, to highly malignant osteosarcoma NOS (9180/3), the most common primary bone cancer in adolescents, producing osteoid matrix and exhibiting rapid growth. Subtypes include chondroblastic osteosarcoma (9181/3), blending cartilaginous and bony elements, and parosteal osteosarcoma (9192/3), a surface-based low-grade variant with better outcomes. Chondromatous neoplasms, under 9200-9243, cover cartilage-derived tumors such as osteochondroma (9210/0), a benign exophytic growth with a cartilage cap that may undergo malignant transformation to chondrosarcoma (9220/3), a slow-growing but locally invasive malignancy graded by cellularity and atypia. Dedifferentiated chondrosarcoma (9243/3) arises from preexisting benign lesions and carries a dismal prognosis due to high-grade sarcomatous components. These classifications aid in standardizing pathology reporting for prognostic and therapeutic decisions. Giant cell tumors, assigned codes 9250-9259, typically exhibit borderline behavior (/1) and involve multinucleated in a stromal background, with NOS (9250/1) commonly affecting the epiphyses of long bones in young adults and rarely metastasizing despite local aggressiveness. Malignant progression to , malignant (9250/3), occurs in less than 10% of cases, often post-radiation. Odontogenic neoplasms (9270-9349) originate from tooth-forming apparatuses, including benign NOS (9310/0), a locally invasive tumor with follicular or plexiform patterns, and malignant odontogenic (9270/3), encompassing aggressive variants like ameloblastic . Primary intraosseous (9270/3) arises de novo in the without epithelial origin. Miscellaneous bone tumors (9260-9269) include (9260/3), a small round cell malignancy driven by EWSR1-FLI1 fusion, predominantly in children and affecting diaphyses of long bones, and of long bones (9261/3), a low-grade tumor mimicking epithelial differentiation in the . These codes facilitate precise epidemiological tracking and multidisciplinary management in .

Synovial-like, Mesothelial, and Miscellaneous Bone Tumors

The synovial-like tumors in ICD-O are classified under morphology codes 9040-9049 and encompass malignant neoplasms that mimic synovial tissue differentiation, primarily affecting soft tissues but occasionally involving through extension or primary osseous variants. These tumors are characterized by a biphasic of epithelial and spindle cell components, with a characteristic t(X;18) translocation involving SS18 and genes. (9040/3) is the prototypical example, presenting as a firm, lobulated mass most commonly in the extremities of young adults, with monophasic spindle cell (9041/3), epithelioid (9042/3), biphasic (9043/3), and clear cell (9044/3) subtypes distinguished by their histological composition and prognostic implications, where biphasic forms often show better outcomes with . Mesothelial tumors, coded 9050-9059, represent neoplasms arising from mesothelial cells lining serosal surfaces such as the pleura, peritoneum, and pericardium, with a strong association to asbestos exposure in malignant cases. Mesothelioma (9050/3), the hallmark lesion, is a malignant tumor subdivided into epithelioid (9052/3), sarcomatoid or desmoplastic (9051/3), and biphasic (9053/3) variants based on cellular morphology and stromal composition, each carrying distinct survival rates—epithelioid forms generally responding better to chemotherapy. The ICD-O-3 classification was updated in its third edition to refine pleural mesothelioma coding, incorporating histological subtypes for improved registry accuracy, while the 2021 WHO classification and subsequent ICD-O-3.2 revisions introduced a specific in situ code (9050/2) for early lesions and emphasized BAP1 immunohistochemistry for diagnostic confirmation in 40-60% of cases. Benign counterparts, such as well-differentiated papillary mesothelioma (9054/0), are rare and typically peritoneal, contrasting with the aggressive diffuse malignant forms. Miscellaneous bone tumors under codes 9350-9379 include a diverse group of mesenchymal-derived lesions affecting the skeletal system, ranging from uncertain behavior to overtly malignant entities. Craniopharyngiomas (9350/1), benign or borderline epithelial tumors of the sellar region with adamantinomatous (9351/1) and papillary (9352/1) subtypes, feature calcified cysts and wet , often causing endocrine dysfunction due to proximity to the pituitary, and are coded with uncertain behavior (/1) reflecting their locally invasive nature despite histological benignity. Chordomas (9370/3), malignant neoplasms of notochordal remnants, arise in axial s like the or clivus, exhibiting physaliphorous cells in a myxoid stroma and brachyury positivity, with chondroid (9371/3) and dedifferentiated (9372/3) variants showing poorer due to aggressive local growth and potential. These codes facilitate precise topographic-morphologic correlation in bone registries, emphasizing mesenchymal differentiation without overlap into vascular or chondromatous categories.

Germ Cell, Gonadal, and Trophoblastic Neoplasms

Germ Cell Tumors

Germ cell tumors in the for (ICD-O) are neoplasms originating from primordial germ cells, which are pluripotent cells capable of differentiating into various tissue types. These tumors are classified under morphology codes 9060-9097 and encompass a spectrum of entities including dysgerminomas and seminomas (9060-9065), tumor (9071/3) and polyembryoma (9072/3), teratomas (9080-9085), and dermoid cysts (9084/0). Dysgerminomas (9060/3) and seminomas (9061/3) represent the most common pure tumors, characterized by uniform cells resembling primordial germ cells, while tumors (9071/3), also known as endodermal sinus tumors, feature Schiller-Duval bodies and produce . Teratomas (9080) include mature (benign, /0) and immature (malignant, /3) variants, often containing elements from multiple germ layers such as , , and , and dermoid cysts (9084/0) are typically benign cystic teratomas with skin-like appendages. These tumors primarily arise in gonadal sites, such as the (C56.9) and testis (C62.), but can also occur extragonadally in locations including the (C38.), retroperitoneum (C48.0), and pineal region. Extragonadal germ cell tumors often present in midline structures and may be associated with poorer compared to gonadal counterparts. Behavior codes vary across the category: benign (/0) for mature forms like dermoid cysts, uncertain or borderline (/1) for some immature or mixed lesions, and malignant (/3) for most subtypes, which predominate in adults and include aggressive entities like choriocarcinoma components in mixed tumors (9100/3). Malignant behavior is particularly common in post-pubertal cases, with seminomas and dysgerminomas showing and favorable outcomes when localized. In ICD-O-3, refinements were introduced for variants, classifying them under 9080/3 with a sixth-digit grade (1-3) to indicate the degree of immaturity and neuroepithelial differentiation, which correlates with malignant potential and guides therapeutic decisions such as adjuvant chemotherapy for higher-grade lesions. This update enhances precision in coding mixed tumors, which may combine elements like embryonal (9070/3) and components, reflecting their heterogeneous . Overall, the classification supports standardized reporting for and treatment planning, emphasizing the pluripotency of s in tumor histogenesis.

Gonadal and Trophoblastic Neoplasms

The International Classification of Diseases for Oncology (ICD-O) categorizes gonadal and trophoblastic neoplasms under morphology codes for sex cord-stromal tumors (8590–8679), trophoblastic tumors (9100–9105), and mesonephric tumors (9110), encompassing tumors derived from sex cord-stromal components of the ovaries and testes, as well as placental trophoblastic elements. These neoplasms are distinct from germ cell tumors, which originate from primordial and are classified separately (e.g., under codes 9060–9097). Gonadal sex cord-stromal tumors often exhibit hormone production, leading to clinical manifestations such as excess or , while trophoblastic tumors are frequently associated with gestational events and elevated (hCG) levels. Sex cord-stromal gonadal tumors, coded 8590–8679, include entities such as (e.g., 8620/1 for adult type, benign or uncertain behavior; 8620/3 for malignant variants), which typically arise in the and are characterized by follicular patterns and estrogen secretion that can cause or . Other examples encompass Sertoli-Leydig cell tumors (e.g., 8610/1, often androgen-producing, leading to ) and thecomas (e.g., 8700/0, estrogenic fibromas). These tumors generally have low malignant potential but can recur years after initial , with staging influencing ; for instance, stage I cases show over 90% 5-year . Mesonephromas, under 9110 (e.g., 9110/3 for malignant mesonephroma, also known as mesonephric ), are rare Wolffian duct-derived tumors primarily in the or , exhibiting clear cell or tubular patterns without prominent hormone production. Trophoblastic neoplasms, coded 9100–9105, represent gestational or non-gestational placental-site tumors, with (9100/3) being the most aggressive, often following or , and marked by high hCG levels facilitating early detection and responsiveness (cure rates exceeding 90% in low-risk cases). Placental site trophoblastic tumors (9104/1, uncertain behavior) produce lower hCG and , presenting with irregular bleeding; they are less chemosensitive than choriocarcinomas. Hydatidiform moles (9100/0 benign; 9100/1 invasive) serve as precursors, with complete moles showing diploid androgenetic karyotypes and partial moles triploidy. These tumors are gestational in origin, underscoring the need for post-pregnancy monitoring. Paragangliomas and glomus tumors (8680–8719) in gonadal contexts, such as extra-adrenal pheochromocytomas (8693/3 malignant paraganglioma), arise from chromaffin cells and may secrete catecholamines, causing ; gonadal sites are uncommon but included when not involving the . Glomus tumors (8711/0 benign; 8711/3 malignant) feature vascular patterns and are typically benign, with rare gonadal occurrences. Behavior codes (/0 benign, /1 uncertain, /3 malignant) guide reporting, emphasizing multidisciplinary management due to endocrine effects.

Nervous System Neoplasms

Gliomas and Neuroepithelial Neoplasms

Gliomas and neuroepithelial neoplasms encompass a diverse group of primary tumors originating from glial cells or primitive neuroectodermal precursors, classified in the for Oncology (ICD-O) under morphology codes 9380-9529. These tumors are primarily intraparenchymal and include both low-grade and high-grade , with behavioral codes indicating malignancy (/3) for most adult forms. The classification emphasizes histological features while integrating molecular markers in later updates to refine subtypes and prognosis. The glioma subgroup (codes 9380-9489) covers tumors derived from , , ependymal cells, and mixed glial elements. Representative examples include (e.g., 9400/3 astrocytoma, NOS; 9421/3 pilocytic astrocytoma; 9440/3 , NOS), (e.g., 9450/3 , NOS; 9451/3 anaplastic oligodendroglioma), and (e.g., 9391/3 , NOS; 9392/3 anaplastic ependymoma). Glioblastomas, the most aggressive form, account for approximately 50% of all gliomas and are graded as WHO grade IV, correlating with poor survival outcomes averaging 12-15 months. Mixed gliomas (9382/3) and gliomatosis cerebri (9381/3) represent diffuse infiltrative patterns, often requiring additional molecular testing for precise categorization. Neuroblastomas and related primitive tumors (codes 9490-9499) primarily affect pediatric populations and arise from derivatives, though central variants are included. Key entities include 9490/3 ganglioneuroblastoma and 9500/3 , NOS, which exhibit small round blue cell morphology and are typically malignant (/3). These tumors often occur in the or sympathetic chain but can involve CNS sites, with staging influenced by the International Neuroblastoma Risk Group system rather than ICD-O grading alone. The neuroepithelial neoplasms subgroup (codes 9500-9529) includes embryonal and primitive tumors such as and (PNETs), though some overlap with 9470-9474 codes for cerebellar variants. Prominent examples are 9470/3 and 9506/3 , NOS, both highly malignant and common in children, comprising about 20% of pediatric brain tumors. Olfactory neuroblastomas (9522/3 ) represent sinonasal neuroepithelial malignancies with intermediate behavior potential. Grading in ICD-O-3 aligns with the (WHO) classification for tumors, using a separate one-digit code (1-4) to indicate differentiation and aggressiveness, integrated since the 2007 WHO edition. For gliomas, WHO grades I-II denote low-grade tumors (e.g., 9421/1 pilocytic , grade 1), while grades III-IV indicate high-grade malignancies (e.g., 9440/3 , grade 4), with concordance rates exceeding 90% when molecular features are considered. This integration facilitates standardized reporting in cancer registries, emphasizing histological-mitotic activity, , and vascular proliferation for grade assignment. Updates to ICD-O-3, particularly in the revision and ICD-O-3.2 (effective January 1, 2021), incorporate molecular subtypes to address limitations in purely histological classification. For instance, IDH-mutant gliomas are distinguished with codes like 9445/3 , IDH-mutant (grade 4), reflecting better compared to IDH-wildtype counterparts, with survival differences up to 20-30 months. Additional markers such as 1p/19q codeletion for oligodendrogliomas (9451/3) and H3 K27M mutations for diffuse midline gliomas (9385/3) enable subtype-specific coding, improving epidemiological tracking and eligibility. These revisions stem from the 2016 and 2021 WHO classifications, ensuring ICD-O remains aligned with advancing .

Meningiomas, Nerve Sheath, and Granular Cell Tumors

The for (ICD-O) categorizes meningiomas, nerve sheath tumors, and granular cell tumors within morphology codes 9530-9589, primarily under nervous system neoplasms, reflecting their origin from meningeal, peripheral , or specialized granular cell elements. These tumors are predominantly extra-axial or peripheral, distinguishing them from intra-axial glial neoplasms, and are coded based on histological features and behavior, with most exhibiting benign (/0) or borderline (/1) behavior, while malignant (/3) variants are uncommon. Meningiomas (codes 9530-9539) arise from arachnoid cap cells in the and represent the most common primary intracranial tumors in adults. The code 9530/0 designates meningioma, NOS, a typical benign variant characterized by whorls and psammoma bodies on . Atypical meningioma, coded as 9539/1, features increased mitotic activity and , classifying it as borderline with higher recurrence risk. Malignant forms, such as 9530/3 (malignant meningioma) or 9538/3 (papillary meningioma), are rare, comprising less than 5% of cases and showing anaplastic features like high mitoses and . The ICD-O-3.2 update, aligned with the 2016 WHO classification, refined coding for atypical meningioma to better capture intermediate-grade behavior, improving prognostic stratification in registries. Nerve sheath tumors (codes 9540-9579) originate from Schwann cells, perineurial cells, or fibroblasts in peripheral nerves, often presenting as solitary or multiple lesions in neurofibromatosis-associated cases. , NOS (9540/0), is a benign, unencapsulated tumor with mixed cellularity, commonly linked to NF1 . (9560 series, typically /0) forms encapsulated masses with Antoni A and B patterns, frequently affecting like the . Malignant peripheral nerve sheath tumors (MPNST; 9540/3) are aggressive sarcomas arising de novo or from benign precursors, accounting for about 10% of sarcomas and showing heterogeneous genetics like NF1 mutations. These tumors are mostly benign, with malignancy rare outside syndromic contexts. Granular cell tumors (codes 9580-9589) derive from Schwann cell precursors or other neural crest elements, featuring abundant lysosomal granules on microscopy, and can occur in soft tissues or sellar regions. The code 9580/0 applies to granular cell tumor, NOS, a benign, slow-growing lesion often in the head and neck or tongue. Malignant granular cell tumor (9580/3) is exceptional, defined by metastasis or marked atypia, with fewer than 2% of cases showing aggressive behavior. Specialized variants include 9582/0 for granular cell tumor of the sellar region, typically benign and pituitary-adjacent. Overall, these neoplasms are overwhelmingly benign, with malignant progression infrequent and linked to size or site.

Hematopoietic and Lymphoid Neoplasms

Lymphomas and Plasma Cell Tumors

The International Classification of Diseases for Oncology, Third Edition (ICD-O-3), categorizes lymphomas and plasma cell tumors within the morphology codes 9590/3 to 9769/3, encompassing a range of lymphoid malignancies that primarily affect solid lymphoid tissues such as lymph nodes and extranodal sites. These codes reflect a major revision in ICD-O-3 to align with the (WHO) Classification of Tumours of Haematopoietic and Lymphoid Tissues, revised 4th edition (2016), incorporating updates for precise histopathological diagnosis and behavior coding, with all terms designated as malignant (/3). This alignment facilitates standardized reporting in cancer registries by grouping neoplasms based on , such as B-cell, T-cell, and NK-cell origins, while distinguishing them from leukemic presentations. Subsequent updates, including those aligning with the 2016 WHO classification, further refined codes to incorporate molecular and genetic features. Lymphomas are subdivided into Hodgkin lymphoma (codes 9650/3–9669/3), non-Hodgkin lymphomas (codes 9670/3–9719/3), and precursor B-cell lymphoblastic lymphoma (9728/3) and precursor T-cell lymphoblastic lymphoma (9729/3). Hodgkin lymphoma includes entities like nodular sclerosis classical Hodgkin lymphoma (9663/3) and mixed cellularity classical Hodgkin lymphoma (9652/3), characterized by Reed-Sternberg cells. Non-Hodgkin lymphomas are further delineated by mature B-cell types (9670/3–9699/3), such as diffuse large B-cell lymphoma (9680/3) and follicular lymphoma (9690/3–9691/3), and mature T- and NK-cell types (9700/3–9719/3), including anaplastic large cell lymphoma (9714/3) and peripheral T-cell lymphoma, NOS (9702/3). Precursor B-cell lymphoblastic lymphoma (9728/3) and precursor T-cell lymphoblastic lymphoma (9729/3) represent immature lymphoid neoplasms, often site-specific to lymph nodes or skin. Nonspecific lymphoma codes (9590/3–9599/3) are used when further subclassification is not possible. Plasma cell tumors fall under codes 9730/3–9739/3, including plasmacytoma, NOS (9731/3), plasma cell myeloma (9732/3), and extramedullary plasmacytoma (9734/3), which denote neoplasms derived from terminally differentiated B-cells producing monoclonal immunoglobulins. These are distinguished by their systemic or localized involvement, with multiple myeloma representing widespread bone marrow infiltration. Additional categories include mast cell neoplasms (9740/3–9741/3, e.g., systemic mastocytosis with an associated hematological neoplasm, 9741/3) and histiocytic/ neoplasms (9750/3–9759/3, such as histiocytic sarcoma, 9750/3). Immunoproliferative diseases occupy codes 9760/3–9769/3, covering entities like lymphoplasmacytic lymphoma (9671/3, cross-referenced) and heavy chain diseases (9762/3). Updates to ICD-O-3 introduced or refined over 40 terms in these ranges to better capture molecular and immunophenotypic features, enhancing epidemiological tracking without altering the core /3 malignant behavior.

Leukemias and Myeloproliferative Disorders

Leukemias and myeloproliferative disorders in the (ICD-O) are classified under morphology codes ranging from 9800 to 9989, encompassing and proliferative conditions originating from hematopoietic cells in the . These neoplasms are uniformly assigned the behavior code /3 to denote , reflecting their invasive and life-threatening nature, and are topographically coded to C42.1, indicating involvement of the as the primary site. This classification facilitates standardized reporting in cancer registries, enabling epidemiological tracking and clinical correlations across global datasets. The category begins with codes 9800-9809 for unspecified leukemias (leukemia, NOS), which serve as a default for cases lacking further differentiation, such as 9800/3 for leukemia, NOS, and 9801/3 for acute undifferentiated leukemia. These codes are crucial for initial registrations when diagnostic precision is pending, ensuring all cases are captured without delay. Lymphoid leukemias fall under 9820-9839, capturing neoplasms derived from lymphoid precursors, including 9820/3 for lymphoid leukemia, NOS, and 9823/3 for chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), a common indolent malignancy. Other examples include 9831/3 for T-cell large granular lymphocytic leukemia and 9837/3 for T-lymphoblastic leukemia/lymphoma, NOS, highlighting the spectrum from acute lymphoblastic to chronic forms. This grouping aligns with immunophenotypic and genetic features, aiding in targeted therapies. Myeloid leukemias, coded 9840-9939, represent the largest subgroup and include acute and chronic variants from myeloid lineage cells. Notable codes are 9860/3 for , NOS; 9861/3 for (AML), NOS, the most frequent in adults; and 9863/3 for chronic myeloid leukemia (CML), NOS, often associated with the BCR-ABL fusion. Additional specifics encompass 9866/3 for with PML::RARA translocation and 9910/3 for , emphasizing subtype-driven prognoses and treatments like all-trans for APL. Other leukemias in 9940-9949 cover less common or hybrid entities, such as 9940/3 for , characterized by BRAF mutations, and 9945/3 for (CMML), NOS, which bridges myelodysplastic and myeloproliferative features. Juvenile myelomonocytic leukemia is designated 9946/3, underscoring pediatric-specific presentations. These codes integrate morphological, cytogenetic, and molecular criteria for precise categorization. Myeloproliferative disorders, under 9950-9969, denote chronic conditions with excessive production of one or more myeloid cell lines, all malignant (/3). Key examples include 9950/3 for , driven by JAK2 mutations; 9960/3 for , NOS; 9961/3 for ; and 9962/3 for . These classifications support monitoring for progression to and guide therapies like JAK inhibitors. Additional hematologic conditions in 9970-9979, such as 9975/3 for myelodysplastic/myeloproliferative neoplasm, unclassifiable, address overlapping syndromes not fitting prior categories. Myelodysplastic syndromes (MDS) are classified in 9980-9989, with 9980/3 for MDS with single lineage dysplasia and 9989/3 for MDS, unclassifiable, reflecting ineffective hematopoiesis and risk of transformation to AML. This structure, derived from the World Health Organization's collaborative framework, ensures interoperability with codes like C92 for myeloid leukemias.

Other Hematologic Neoplasms

The category of other hematologic neoplasms in the (ICD-O) includes rare entities involving histiocytes, s, immunoproliferative processes, and myelodysplastic changes, primarily coded under morphology codes 9740–9999, excluding those classified as lymphomas or leukemias. These neoplasms represent outliers in hematopoietic and lymphoid tissue , often requiring specialized immunohistochemical confirmation for accurate and coding. Histiocytic and accessory cell neoplasms (9750–9759) are particularly emphasized, as they arise from monocyte-macrophage or lineages and exhibit distinct morphological features such as large cells with abundant cytoplasm and grooved nuclei. Histiocytic neoplasms encompass a spectrum from localized to disseminated diseases. (LCH), coded as 9751/3 when reportable (multifocal or disseminated), involves clonal proliferation of Langerhans-type dendritic cells, typically expressing CD1a, langerin (CD207), and ; it is reportable as malignant when multifocal or disseminated, affecting , , and other sites in children and adults. , coded 9750/3 and synonymous with true histiocytic , is an aggressive malignancy of mature histiocytes, characterized by pleomorphic cells positive for , , and , often presenting in s or soft tissues with poor . Additional rare histiocytic-derived tumors include (9756/3), a high-grade with malignant features, and interdigitating dendritic cell (9757/3), involving S100-positive cells from paracortical regions; both were added for specificity in cases diagnosed from 2001 onward. Fibroblastic reticular cell tumor (9759/3), recognized since 2010, represents another dendritic cell variant with spindle-shaped cells in nodal stroma. Immunoproliferative diseases (9760–9769) capture disorders with dysregulated immunoglobulin production outside classic neoplasms. Immunoproliferative disease, NOS (9760/3), serves as a broad code for unspecified entities, while heavy chain diseases (9762/3) include alpha (9762/3), gamma (9762/3), and mu (9762/3) variants, characterized by production of truncated heavy chains leading to lymphoplasmacytic infiltrates in mucosal sites. (9761/3), an indolent B-cell with IgM paraproteinemia, overlaps here but is distinguished by involvement and hyperviscosity symptoms. These codes facilitate tracking of rare gammopathies not fitting categories. Myelodysplastic syndromes (MDS), coded 9980–9989, denote ineffective hematopoiesis with cytopenias and dysplastic marrow changes, reportable as malignant neoplasms in ICD-O-3 since January 1, 2001. Key subtypes include refractory anemia (9980/3), refractory anemia with ring sideroblasts (9981/3), refractory anemia with excess blasts (9982/3), and therapy-related MDS, NOS (9987/3), the latter linked to prior chemotherapy or radiation exposure. MDS with 5q deletion syndrome (9986/3) highlights a specific cytogenetic abnormality conferring distinct clinical features, such as anemia responsive to lenalidomide. These codes underscore the premalignant potential of MDS, with progression risks to acute myeloid leukemia. A significant update in the 2011 ICD-O-3.1 revision incorporated blastic plasmacytoid dendritic cell neoplasm (BPDCN, 9727/3), a rare, aggressive malignancy of precursors, often initially cutaneous with +, CD56+, and CD123+ expression, and high relapse rates despite intensive therapy. This addition refined classification for this orphan disease, previously coded under ambiguous terms like agranular + NK-cell .

Updates and Future Directions

ICD-O-3 Updates and Implementation

The third edition of the (ICD-O-3), published in 2000, has undergone targeted refinements to incorporate advances in tumor and systems. In 2013, an update known as ICD-O-3.1 was released, focusing exclusively on the morphology codes for hematopoietic and lymphoid neoplasms (with in some registries starting in 2011). This revision introduced 38 new codes to better align with the 2008 (WHO) Classification of Tumours of Haematopoietic and Lymphoid Tissues, enabling more precise coding of , mature B-cell, T-cell, and NK-cell lymphomas, among others. A further update, ICD-O-3.2, was issued in 2019 to reflect emerging molecular and histological insights. This version included revisions to codes for breast tumors (such as updates to mucinous and metaplastic carcinomas) and neoplasms (including refinements for gliomas and meningiomas). These changes enhanced the system's ability to capture molecularly defined entities without overhauling the core structure. Implementation of these updates has been phased, with mandatory adoption varying by registry network. For the Surveillance, Epidemiology, and End Results (SEER) program, the 2011 ICD-O-3.1 hematopoietic codes became required for cases diagnosed on or after January 1, 2011, while the North American Association of Central Cancer Registries (NAACCR) enforced them progressively through 2014. ICD-O-3.2 was mandated for SEER and NAACCR cases starting in 2020, with annual refinements to ensure consistency. In 2024, the Cancer PathCHART initiative—developed by the Cancer Pathology Coding Histology And Registration Terminology group—integrated molecular markers into ICD-O-3 coding for select sites, such as brain tumors with specific genetic alterations, providing a searchable validation list for site-morphology combinations. Adoption has not been without hurdles, particularly in training cancer registrars to apply the nuanced lymphoid codes in ICD-O-3.1 and the molecular terms in ICD-O-3.2, which required updated educational modules and software adjustments. posed another issue, as converting pre-update historical data often resulted in loss of , with specific new morphology codes mapping imprecisely to older equivalents. Despite these, ICD-O-3 updates have become the global standard, utilized in cancer registries across more than 100 countries for standardized tumor reporting and epidemiological analysis.

Introduction to ICD-O-4

The for Oncology, Fourth Edition (ICD-O-4), represents the next evolution in the standardized coding system for neoplasms, developed under the auspices of the (WHO) and the International Agency for Research on Cancer (IARC). Its creation aligns closely with the fifth edition of the WHO Classification of Tumours (Blue Books), incorporating updated histological and molecular classifications to reflect advances in since the third edition (ICD-O-3). Development of ICD-O-4 began with consultations from 2021 to 2025, involving global cancer registries and experts to ensure harmonization with emerging diagnostic criteria. A draft was released in 2024 for public comment, with feedback periods closing on November 1, 2024, and extended to May 31, 2025. As of November 2025, the draft is under review by expert groups including the NAACCR ICD-O , with no specific release date announced yet. The NAACCR ICD-O and IACR played pivotal roles in reviewing submissions and refining the classification to address gaps in prior versions. Key anticipated changes in ICD-O-4 include an expanded 5-digit morphology code structure, where the alphanumeric codes provide greater specificity for histological subtypes, including cases previously grouped under broader terms and new entities with non-zero fifth digits. The axis will feature updated site codes to incorporate novel anatomical locations identified in recent epidemiological data. Additionally, the edition integrates more molecular and genomic terminology, such as expanded references to fusion genes, to support precision medicine by enabling coding of genetically defined neoplasms. These enhancements aim to address emerging conditions, including therapy-related malignancies like those associated with CAR-T cell therapies, thereby improving cancer surveillance and research interoperability. ICD-O-4 is expected to be released in late 2025 or 2026 as an online PDF, with NAACCR and IACR leading implementation efforts through standardized training and validation tools for registries worldwide. To facilitate adoption, transition guidelines will include mapping protocols from ICD-O-3 codes, ensuring continuity in historical data analysis while accommodating the new precision-oriented framework. This builds on recent ICD-O-3 refinements without overlapping their scope.

References

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