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Vulvectomy
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 | This article needs more reliable medical references for verification or relies too heavily on primary sources. (January 2022) |
| Vulvectomy | |
|---|---|
 A 3 in 1 incision vulvectomy | |
| ICD-9-CM | 71.5–71.6 |
Vulvectomy refers to a gynecological procedure in which the vulva is partly or completely removed. The procedure is usually performed as a last resort in certain cases of cancer,[1] vulvar dysplasia, vulvar intraepithelial neoplasia,[2] or as part of female genital mutilation. Although there may be severe pain in the groin area after the procedure, for a number of weeks, sexual function is generally still possible but limited.[3]
Types
[edit]A simple vulvectomy can be either complete (more than 80% of the vulvar area) or partial (less than 80% of vulvar area). It removes the skin and superficial subcutaneous tissues. A radical vulvectomy is the same with regard to complete or partial, however, includes removal of skin and deep subcutaneous tissue. An inguinofemoral lymphadenectomy may be performed along with a radical vulvectomy (whether partial or complete) on one or both sides if spread of a cancer is suspected.[4]
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A simple partial vulvectomy is the least severe, only removing the affected portion of the vulva.
A "skinning vulvectomy" is the removal of the top layer of vulvar skin (the external female genital organs, including the clitoris, vaginal lips and the opening of the vagina). In this case skin grafts from other parts of the body may be needed to cover the area. There are two types of skinning vulvectomy, the "partial" and the "total". The objective of the first is the preservation of the cosmetic and functional integrity of the vulva in younger and sexually active patients, in whom a steady increase in the incidence of vulvar intraepithelial neoplasia has been observed in the last decade.[when?] The objective of the latter is the removal of the entire vulva with total skin graft replacement in patients with an entire vulvar cancer involvement. "Modified radical vulvectomy" involves the removal of vulva containing cancer and some of the normal tissue around it.
See also
[edit]References
[edit]- ^ Fuh KC and Berek JS (2012) Current management of vulvar cancer. Hematol Oncol Clin North Am. 26(1):45–62. doi:10.1016/j.hoc.2011.10.006
- ^ Hillemans P, Wang X, Staehle S, Michels W, Dannecker C (2006) Evaluation of different treatment modalities for vulvar intraepithelial neoplasia (VIN): CO(2) laser vaporization, photodynamic therapy, excision and vulvectomy. Gynecol Oncol. 100:271–5. doi:10.1016/j.ygyno.2005.08.012
- ^ Aerts L et al (2012) Sexual, psychological, and relational functioning in women after surgical treatment for vulvar malignancy: a literature review. J. Sex. Med. 9(2):361–71. doi:10.1111/j.1743-6109.2011.02520.x
- ^ American Medical Association Current Procedural Terminology (CPT) Professional Edition 2015 ISBN 162202026X
Vulvectomy
View on Grokipediafrom Grokipedia
Definition and Overview
Procedure Description
Vulvectomy is a surgical procedure to excise part or all of the vulva, the external female genitalia encompassing the labia majora, labia minora, clitoris, and vestibular tissues.[3] The surgery is conducted under general anesthesia to render the patient unconscious and insensate to pain.[1][8]
The patient is positioned in the lithotomy or split-leg stance, with legs separated and elevated in stirrups to optimize exposure of the perineal region.[1] The surgeon marks the resection margins, typically 1-2 cm of healthy tissue surrounding the lesion, and performs incisions circumferentially or in a tailored pattern around the vulva to encompass the targeted area.[2] Dissection proceeds through the skin, dermis, subcutaneous fat, and, in more extensive cases, deeper structures including underlying muscles or portions of the vagina and perineum, ensuring complete removal while preserving vital anatomy where possible.[2][8]
Hemostasis is meticulously secured to control bleeding, followed by wound closure. Primary closure with sutures is employed for smaller defects; larger excisions necessitate reconstruction via skin grafts from donor sites like the thigh or flaps mobilized from adjacent tissues to cover the raw area, restore contour, and facilitate healing.[1][2] Drains may be placed temporarily to evacuate serous fluid and prevent hematoma formation.[3] The procedure duration varies from 1 to 4 hours depending on extent and concurrent interventions.[1]
Anatomical Considerations
The vulva consists of the mons pubis, labia majora, labia minora, clitoris, vulvovaginal vestibule (including the urethral and vaginal orifices), vestibular bulbs, and associated glands such as the Bartholin and Skene glands. These structures overlie the perineal body and are bounded laterally by the groin creases, superiorly by the pubic symphysis, and inferiorly by the anus. The skin of the labia majora is thicker and hair-bearing, transitioning to thinner, mucous membrane-like epithelium on the labia minora and vestibule, which influences surgical margins and reconstruction techniques to preserve cosmesis and function.[9][10] Vascular supply to the vulva derives primarily from branches of the internal pudendal artery (arising from the internal iliac artery), including the posterior labial arteries that perfuse the labia, vestibule, and perineum, supplemented by the external pudendal artery (from the femoral) for the mons pubis and labia majora. Venous drainage parallels the arterial supply via the internal and external pudendal veins, forming a rich plexus that contributes to hemostatic challenges during excision but supports wound healing post-resection. Innervation is provided mainly by the pudendal nerve (S2-S4), which supplies sensory fibers to the labia, clitoris (via its dorsal branch), and vestibule, as well as motor innervation to the bulbospongiosus and ischiocavernosus muscles; the anterior mons and labia majora receive contributions from the ilioinguinal and genitofemoral nerves.[11][10][9] Lymphatic drainage from the vulva proceeds to the superficial inguinal lymph nodes (primarily), with deeper structures like the clitoris also draining to deep inguinal and external iliac nodes, a pathway that necessitates careful consideration in vulvectomy to achieve oncologic clearance while mitigating risks of lymphedema, particularly with concurrent inguinofemoral lymphadenectomy. Surgical approaches must account for the vulva's proximity to the urethra, vagina, and rectum, as well as its embedding in the superficial perineal pouch above the urogenital diaphragm, to avoid inadvertent injury to continence mechanisms or pelvic floor integrity. The clitoral neurovascular bundle, located dorsally beneath Buck's fascia, requires precise dissection in procedures involving its removal to minimize sexual dysfunction.[9][12][13]Historical Development
Early Surgical Approaches
The initial surgical interventions for vulvar carcinoma in the late 19th and early 20th centuries were limited to local excision or cauterization of visible lesions, approaches that overlooked the frequent lymphatic spread to inguinal nodes and resulted in high recurrence rates exceeding 50% in many series.[14] Electrocoagulation of the vulva emerged around 1922 as a more aggressive local method, aiming to destroy tissue in situ while preserving some anatomy, but it carried risks of incomplete ablation and subsequent metastasis, with long-term control rates below 30% in reported cases.[15] These techniques reflected the era's limited understanding of vulvar cancer's propensity for regional dissemination, prioritizing symptom palliation over curative intent.[16] A paradigm shift occurred with the introduction of radical vulvectomy, an en bloc resection combining vulvar excision with inguinal-femoral lymphadenectomy to address lymphatic pathways comprehensively. French surgeon A. Basset described this integrated approach in 1912, advocating dissection from the vulva to groin nodes in continuity to minimize skip metastases.[17] German gynecologist Walter Stoeckel advanced separate incision techniques for node sampling in 1930, reducing wound complications while maintaining oncologic radicality.[14] These methods laid the groundwork for standardized radical surgery, though operative mortality approached 10-15% due to extensive tissue disruption and infection risks in the pre-antibiotic era.[18] American gynecologist Frederick J. Taussig refined the procedure in the 1930s and 1940s, reporting on over 100 cases by 1940 with a focus on butterfly incisions extending from the groin above the inguinal ligament at a 60-degree angle to encompass deep pelvic nodes when indicated.[16] Taussig's series demonstrated 5-year survival rates of approximately 40-50% for node-negative patients, a marked improvement over prior local therapies, attributing success to wide margins (at least 2 cm) and bilateral node clearance regardless of laterality.[19] British surgeon Stanley Way further popularized variations in the 1940s, performing 75 radical vulvectomies between 1943 and 1949 with reported postoperative mortality of 14.6%, emphasizing meticulous hemostasis and drainage to mitigate lymphedema and wound breakdown.[18] These early radical approaches, while morbid—often involving urinary diversion and prolonged healing—established surgery as the cornerstone for curative intent in invasive disease.[20]Evolution to Conservative Techniques
The radical en bloc vulvectomy, popularized by Fredrick Taussig and Stanley Way in the 1940s, became the standard treatment for vulvar cancer following earlier poor outcomes with local excisions, which yielded 5-year survival rates of only 15-25%.[16] This approach involved extensive removal of the entire vulva in a butterfly-shaped incision combined with bilateral inguinofemoral lymphadenectomy, improving survival to 60-70% by the mid-20th century through comprehensive resection of primary tumors and regional nodes.[16] [20] However, it was associated with substantial morbidity, including wound breakdown rates exceeding 50%, chronic lymphedema in up to 65% of cases, prolonged hospitalization, and significant psychosexual dysfunction due to loss of vulvar anatomy and function.[21] [16] By the 1970s, recognition of these complications prompted initial shifts toward less invasive strategies for early-stage disease. DiSaia proposed wide local excision for small lesions, while Morris introduced unilateral groin dissection for lateralized tumors in 1977, aiming to tailor surgery to tumor location and extent without sacrificing oncologic efficacy.[16] In 1979, DiSaia and Hacker advanced modified radical vulvectomy with reduced resection margins of 1-2 cm, which the Gynecologic Oncology Group later validated in 1992 as sufficient for local control.[21] A pivotal innovation in 1981 by Hacker et al. involved separate incisions for groin lymphadenectomy, decoupling it from vulvar resection; this reduced wound complications to 14% and shortened hospital stays to 19 days compared to en bloc methods.[16] Further refinements in the 2000s solidified conservative techniques as standard for stages I-II vulvar cancer. Studies demonstrated that partial vulvectomy or wide local excision with 1 cm margins achieved equivalent survival to radical procedures—such as 97% and 90% 5-year survival for stages I and II, respectively—while preserving clitoral sensation, sexual function, and cosmesis.[22] [21] The introduction of sentinel lymph node biopsy by van der Zee in 2007 for unifocal tumors under 4 cm, supported by GROINSS-V (2008) and GOG-173 (2012) trials, minimized unnecessary full lymphadenectomy, lowering lymphedema incidence to 2% and groin recurrence to 2-5%, with disease-free survival rates of 72.5-92.5% at 3 years.[16] [21] These evidence-based changes prioritized quality-of-life outcomes without compromising cure rates, driven by pathological insights into tumor behavior and lymphatic drainage rather than blanket radicalism.[20]Indications and Patient Selection
Primary Medical Indications
Vulvectomy is primarily indicated for the surgical management of invasive vulvar cancer, most commonly squamous cell carcinoma, which constitutes approximately 90-95% of vulvar malignancies. In early-stage disease (FIGO stages I-II), partial or radical vulvectomy is employed to achieve clear surgical margins of at least 8-15 mm while preserving as much healthy tissue as possible, often combined with sentinel lymph node biopsy or inguinofemoral lymphadenectomy for staging and treatment.[23][24] For more advanced central tumors exceeding 2 cm in diameter or with significant stromal invasion, radical vulvectomy remains the standard to ensure oncologic clearance, though modified approaches aim to minimize morbidity.[2][25] High-grade vulvar intraepithelial neoplasia (uVIN or VIN 2/3), a precancerous lesion associated with human papillomavirus, represents another key indication, particularly when lesions are extensive, multifocal, or recurrent despite conservative therapies like laser ablation or topical imiquimod. In such cases, wide local excision or skinning vulvectomy—removing the epithelial layer without underlying structures—may be necessary to eradicate multifocal disease and reduce progression risk to invasive carcinoma, which occurs in 6-30% of untreated high-grade VIN cases.[26][27] Less commonly, vulvectomy is indicated for rare vulvar malignancies such as melanoma, basal cell carcinoma, or extramammary Paget's disease when localized resection fails to achieve margins or in cases of verrucous carcinoma unresponsive to alternatives. For non-neoplastic conditions, indications are exceptional and typically limited to refractory severe vulvar dystrophy or chronic inflammatory states like lichen sclerosus with malignant transformation risk, though evidence favors less radical interventions first.[25][28]Diagnostic Criteria and Staging
Diagnosis of vulvar cancer requires histopathological confirmation, typically through biopsy of suspicious vulvar lesions identified during physical examination.[29] Clinical evaluation begins with a detailed history and inspection of the vulva for abnormalities such as persistent itching, thickened skin, ulcers, or palpable masses, often using colposcopy for magnification.[8] Biopsy, either incisional for larger lesions or excisional for smaller ones, is essential to distinguish invasive carcinoma (e.g., squamous cell carcinoma in over 90% of cases) from vulvar intraepithelial neoplasia or benign conditions, with pathology assessing tumor type, grade, and stromal invasion depth.[30] Additional tests like cervical screening may be performed but do not diagnose vulvar cancer; imaging such as MRI or CT scans evaluates local extension and lymph node involvement only after biopsy confirmation.[29] Staging employs the 2021 revised FIGO system, which integrates tumor size, invasion depth, lymph node status, and distant metastasis to guide prognosis and treatment, including vulvectomy extent.[31] Unlike prior versions, it consolidates node-positive disease into Stage III with substages based on unilateral/bilateral involvement and extracapsular extension, while Stage IV denotes unresectable or metastatic disease.[32] The NCCN guidelines endorse this system, recommending sentinel lymph node biopsy for early-stage cases to refine staging accuracy over traditional inguinofemoral lymphadenectomy.[33]| Stage | Description |
|---|---|
| IA | Tumor ≤2 cm, stromal invasion ≤1 mm, negative nodes.[31] |
| IB | Tumor >2 cm or invasion >1 mm, confined to vulva/perineum, negative nodes.[31] |
| II | Tumor of any size with adjacent spread (e.g., urethra, vagina, anus) but negative nodes.[31] |
| III | Positive ipsilateral (IIIA1: single node ≤5 mm; IIIA2: single node >5 mm or multiple ≤5 mm) or contralateral/bilateral nodes (IIIB: any node >5 mm or extracapsular extension; IIIC: ≥2 nodes with extracapsular extension).[31] |
| IVA | Tumor invading upper urethra, bladder, rectum mucosa, or fixed/ulcerated pelvic nodes.[31] |
| IVB | Distant metastasis (e.g., lungs, bones).[31] |
Types and Surgical Variations
Simple and Partial Vulvectomy
Simple vulvectomy involves the excision of the entire vulvar skin and underlying subcutaneous tissue down to, but not including, the deep fascia, without removal of deeper structures or lymph nodes.[36] This procedure is typically reserved for extensive or multifocal superficial lesions, such as vulvar intraepithelial neoplasia (VIN) or Paget's disease, where local excision is insufficient to achieve adequate margins.[37] [38] Partial vulvectomy, in contrast, removes only the affected portion of the vulva, along with a margin of healthy tissue, preserving as much normal anatomy as possible.[8] It may be performed as a simple partial procedure, limited to skin and subcutaneous layers for noninvasive or early invasive lesions, or as a partial radical vulvectomy extending to deeper tissues for small tumors.[39] [2] This approach is indicated for localized vulvar cancers or precancerous conditions confined to one area, such as the labia or perineum, allowing for oncologic control with reduced morbidity compared to more extensive resections.[40] [1] Surgical techniques for both emphasize wide margins—typically 1-2 cm of unaffected tissue—to minimize recurrence risk, with intraoperative frozen section analysis to confirm adequacy.[37] Closure is achieved primarily when possible, or with skin grafts or flaps for larger defects, particularly in simple vulvectomy where the entire vulva is denuded.[3] Lymph node evaluation is not routinely included unless staging indicates inguinofemoral involvement, distinguishing these from radical variants.[2] Oncologic outcomes for simple and partial vulvectomies in early-stage disease yield high local control rates, with 5-year survival approaching 100% for noninvasive lesions treated via simple vulvectomy.[41] Recurrence rates for VIN can reach 20-30% due to field cancerization, necessitating vigilant follow-up, though these procedures preserve sexual function and body image better than radical options.[42] Complications include wound breakdown (10-20% incidence) and infection, but overall morbidity is lower than in radical surgery.[8]Radical Vulvectomy
Radical vulvectomy is a surgical procedure involving the en bloc resection of the entire vulva, encompassing the labia majora, labia minora, clitoris, vestibule, and Bartholin glands, along with underlying subcutaneous tissue and deeper structures down to the deep fascia of the thigh, pubic periosteum, and inferior fascia of the urogenital diaphragm.[43] This approach ensures wide margins for oncologic control in invasive vulvar cancers, particularly squamous cell carcinomas that are multifocal, larger than 2 cm, or involve critical structures requiring complete excision to prevent local recurrence.[25][34] Indicated primarily for stage II or higher vulvar cancers where tumors extend beyond superficial layers or exhibit stromal invasion greater than 1 mm, radical vulvectomy contrasts with simple or partial vulvectomy by incorporating deeper tissue removal to address invasive disease rather than confining excision to skin and mucosa.[25][2] Unlike simple vulvectomy, which targets precancerous lesions like vulvar intraepithelial neoplasia by removing only the epidermis and dermis, radical procedures achieve R0 resection margins of at least 1-2 cm, essential for prognosis in invasive cases.[1][44] Surgical techniques have evolved from the classic Taussig-Basset en bloc radical vulvectomy, introduced in 1912, which included butterfly incisions across the perineum and groins, to modified approaches using separate incisions for the vulva and inguinal-femoral lymph nodes to reduce wound complications and lymphedema.[45][46] Contemporary practice favors modified radical vulvectomy, preserving distal urethral and anal sphincter function when feasible, often combined with sentinel lymph node biopsy or inguinofemoral lymphadenectomy for staging and treatment, as per NCCN guidelines for tumors with depth of invasion exceeding 1 mm.[34][47] The 3-in-1 incision technique, involving anterior, central, and posterior cuts, facilitates precise resection while minimizing disruption to adjacent structures.[48] This procedure carries significant morbidity, including chronic wound healing issues, sexual dysfunction, and urinary complications, prompting a shift toward less radical options like radical wide local excision for early-stage disease where equivalent survival rates are achieved with reduced psychosexual impact.[49][50] Nonetheless, for advanced lesions, radical vulvectomy remains a cornerstone, with 5-year survival rates approaching 70-80% when margins are negative and nodes are uninvolved.[25][51]Modified and Extended Procedures
Modified radical vulvectomy involves the excision of most, but not all, of the vulva—including the labia majora, labia minora, clitoris, and Bartholin's glands—along with underlying subcutaneous fat and deep tissues, while sparing select structures to preserve sexual and urinary function.[52] This procedure typically includes removal of nearby lymph nodes via inguinal-femoral lymphadenectomy and is indicated for stage I-II invasive vulvar squamous cell carcinoma where tumor margins can be achieved without en bloc resection of the entire vulva.[3] Compared to classical radical vulvectomy, the modified approach employs separate incisions (e.g., triple-incision technique) to reduce wound complications and lymphedema, with retrospective studies reporting equivalent 5-year survival rates of approximately 80-90% for early-stage disease but lower psychosexual morbidity.[21] [53] Extended vulvectomy extends beyond standard radical resection for locally advanced (stage III-IV) or recurrent vulvar carcinoma, incorporating wide margins that may involve partial resection of the distal urethra, anterior vaginal wall, perianal skin, or anus, often requiring multidisciplinary input for bowel or urinary reconstruction.[54] [55] Performed in 10-20% of advanced cases, this procedure achieves local control in up to 70% of patients with gross residual disease but carries higher risks of wound dehiscence (up to 50%) and necessitates immediate or delayed flap reconstruction using autologous tissue, such as gluteal or thigh perforator flaps, to restore vulvar contour and prevent contractures.[56] [57] Oncologic efficacy is evidenced by 5-year disease-free survival rates of 40-60% in selected cohorts, though selection bias toward operable advanced tumors limits generalizability.[55] Both modified and extended variants prioritize histologically negative margins (≥1 cm) confirmed intraoperatively via frozen section, with adjuvant radiation or chemoradiation reserved for positive nodes or close margins per NCCN guidelines.[58]Surgical Techniques
Preoperative Evaluation and Preparation
Preoperative evaluation for vulvectomy begins with a detailed medical history and physical examination to identify comorbidities such as cardiovascular disease, diabetes, or pulmonary conditions that may increase perioperative risks, alongside assessment of performance status and frailty using tools like the modified 5-item frailty index, which identifies approximately 25% of patients undergoing radical vulvectomy as frail and correlates with higher complication rates.[59][60] Laboratory tests, including complete blood count, serum chemistry, coagulation profile, and squamous cell carcinoma antigen levels, are performed to evaluate baseline organ function and nutritional status.[61] Cardiac evaluation via electrocardiogram and chest X-ray may be indicated for patients over age 50 or with risk factors, while pulmonary function tests are considered for those with respiratory compromise.[62] Staging assessment confirms the diagnosis through prior vulvar biopsy and employs imaging modalities such as pelvic ultrasound for inguinal lymph node evaluation (sensitivity 72-100%, specificity 72-97%), MRI for local tumor extension (accuracy 85%), and PET/CT for detecting metastases in advanced cases (mean SUV 8.4 for malignant lesions).[61] Vulvoscopy enhances diagnostic accuracy for histological confirmation, with sensitivity up to 98% but lower specificity.[61] A multidisciplinary team, including gynecologic oncologists, pathologists, and radiologists, reviews findings to determine surgical extent, balancing oncologic clearance with preservation of sexual and urinary function.[61] Preparation involves informed consent, where patients are counseled on procedure-specific risks including wound dehiscence, lymphedema, and psychosexual impacts, as well as alternatives like neoadjuvant therapy for locally advanced disease.[59] Optimization strategies include smoking cessation to mitigate wound healing delays, nutritional support for malnourished patients, and glycemic control in diabetics.[59] Preoperative thromboprophylaxis with low-molecular-weight heparin is standard for major vulvar procedures due to venous thromboembolism risk, alongside prophylactic antibiotics administered within 60 minutes of incision to reduce surgical site infections.[37] Patients undergo pre-admission assessment 1-2 weeks prior, including team consultations and education on postoperative exercises; fasting protocols require cessation of solids 6 hours and clear fluids 2 hours preoperatively.[62]Intraoperative Methods
![Diagram of a 3-in-1 incision vulvectomy][float-right] The patient undergoes general anesthesia and is positioned in the lithotomy or split-leg configuration, with hips abducted 30 degrees and knees flexed 90 degrees to facilitate access to the vulva and perineum.[63][64] A Foley catheter is inserted to decompress the bladder and protect the urethra during dissection.[65] Surgical margins are marked 1 to 2 cm beyond the visible tumor extent, reduced to 1 cm adjacent to the urethra or anus to preserve continence and sphincter function.[4][64] Incision commences with a scalpel along the marked perimeter, encircling the vulvar structures from the mons pubis to the perineum, often in a butterfly or triangular pattern for radical procedures.[4] Dissection proceeds through skin and subcutaneous fat to the urogenital fascia or perineal membrane using ultrasonic scalpels, harmonic devices, or electrocautery for precise tissue separation and hemostasis, excising labia majora, minora, clitoris, and vestibular tissue as indicated while sparing deeper structures like the pubic bone periosteum.[64][4] In modified techniques, such as single-incision approaches, the vulvectomy integrates with groin access to minimize incisions, with lymphatic tissue divided to the deep fascia.[64] Hemostasis is achieved via ligature of vessels and electrocoagulation, followed by layered closure: superficial fascia approximated with absorbable sutures like 3-0 Vicryl, and skin edges closed primarily if tension-free or via advancement flaps for defects.[64][4] No drains are routinely placed in the vulvar bed for select minimally invasive variants, though suction drainage may be used based on extent of resection.[64] Intraoperative frozen section analysis confirms margin negativity, guiding extent of resection.[66]Lymph Node Management
In vulvar cancer surgery, lymph node management primarily involves assessment of the inguinal-femoral nodes, as vulvar drainage follows the superficial inguinal nodes to the deep femoral and external iliac chains, with nodal metastasis serving as the dominant prognostic factor influencing survival.[67][68] For early-stage disease (typically FIGO stages I-II with clinically negative groins and unifocal tumors ≤4 cm and >1 mm stromal invasion), sentinel lymph node biopsy (SLNB) is recommended as the initial procedure to stage the axilla while reducing morbidity compared to full inguinofemoral lymphadenectomy (IFL).[34][69] SLNB employs a combined technique of peritumoral injection with technetium-99m radiocolloid (for lymphoscintigraphy and gamma probe detection) and isosulfan blue or patent blue dye (for visual identification), achieving a detection rate of approximately 87-93% and a false-negative rate of 5-7% in validated protocols.[70][71] When SLNB identifies negative sentinel nodes, observation without further dissection is standard, correlating with groin recurrence rates of 1-2% and 5-year survival exceeding 90% in node-negative cases, comparable to historical IFL outcomes but with substantially lower rates of lymphedema (3-5% vs. 20-30%).[72][73] Positive sentinel nodes (micrometastases <2 mm or macrometastases) prompt either completion IFL or, per GROINSS-V II trial results, inguinofemoral radiation therapy (50.4 Gy) as a safe alternative, yielding isolated groin recurrence rates of 2.3% and overall survival of 74% at 3 years without routine chemotherapy.[74] For clinically suspicious nodes (palpable or imaging-positive), upfront IFL remains indicated, often combined with neoadjuvant chemotherapy for bulky disease (>4 cm or fixed nodes) to downstage prior to resection, though evidence for routine bilateral IFL in unilateral drainage cases is limited to midline tumors.[75][76] Pathologic ultrastaging of sentinel nodes via serial sectioning and immunohistochemistry (e.g., cytokeratin AE1/AE3) enhances micrometastasis detection, with isolated tumor cells (<0.2 mm) not altering management per current guidelines.[77] Adoption of SLNB has increased since 2012, reducing IFL utilization from over 80% to under 50% in select centers, supported by meta-analyses confirming noninferiority in recurrence-free survival (hazard ratio 1.03) while improving quality-of-life metrics like leg edema and cellulitis incidence.[78][79] Bilateral SLNB is advised for tumors within 1-2 cm of midline due to potential crossover drainage, whereas lateral tumors (>2 cm from midline) permit unilateral assessment if ipsilateral nodes are negative.[75] Contraindications to SLNB include prior groin surgery, multifocal disease, or clinically positive nodes, where full IFL or imaging-guided biopsy precedes definitive therapy.[80]Complications and Risks
Immediate Postoperative Complications
Immediate postoperative complications after vulvectomy, typically occurring within the first 30 days, encompass wound infections, dehiscence, and lymphatic issues, with overall rates ranging from 11% in early-stage disease to 54% when combined with inguinofemoral lymphadenectomy (IFL).[81][7] These arise due to the procedure's involvement of perineal tissues and potential concurrent groin dissection, leading to disrupted vascular and lymphatic drainage. Advanced disease stage increases risk, with 38% complication rate versus 11% in early stages, often necessitating prolonged healing or re-intervention.[81] Wound infections represent a dominant short-term issue, affecting 13.7-39% of patients, particularly in groin incisions from IFL, where rates reach 36.1% compared to 15.1% with sentinel lymph node biopsy alone.[7][82] These infections contribute to delayed presentation relative to standard surgical wounds and may require antibiotics or drainage.[83] Wound dehiscence or breakdown occurs in 6-17% of cases, more frequently with radical procedures or en bloc resection, prompting re-operation in up to 25% of advanced cases.[81][82][84] Risk factors include older age and higher postoperative drain output, exacerbating tissue separation in the vulvar or groin areas.[84] Lymphoceles form in approximately 40% of patients post-IFL, resulting from lymphatic leakage into the groin, often managed conservatively but contributing to overall morbidity in 76% of cases with groin involvement.[82] Seromas and hematomas, though less quantified, align with these lymphatic disruptions and wound issues.[85] Other immediate concerns include urinary retention or tract infections, though less prevalent in vulvectomy-specific data, and early lymphedema in 4-17% depending on stage.[81] Diabetes elevates overall short-term risk (OR 4.10).[84] No significant associations with age over 80, BMI, or smoking were found in population cohorts.[7]Long-Term Morbidity
Long-term morbidity after vulvectomy primarily arises from extensive tissue resection and associated lymph node dissection, with chronic lymphedema representing one of the most prevalent and debilitating complications, affecting 20-38% of patients undergoing inguinofemoral lymphadenectomy compared to 3-4% with sentinel lymph node biopsy alone.[7][86] Incidence escalates with the number of nodes removed (17% for ≤4 nodes, 43% for ≥9 nodes) and adjuvant radiotherapy, persisting for years and linked to early complications like lymphocele.[87][7] Risk factors include lymph node metastases and procedure extent, though patient factors like age or comorbidities show inconsistent associations.[86] Sexual dysfunction constitutes another major long-term sequela, with up to 89% of patients experiencing complications post-radical vulvectomy, including dyspareunia (36%), vaginal narrowing (33-36%), reduced lubrication, and diminished arousal (at the 8th percentile in early assessments).[88][89] Only 20% of survivors remain sexually active years later, versus 69% in age-matched norms, correlating with vulvar scarring (25%), tight skin (27%), and body image impairment (4th percentile).[89][87] These effects persist beyond 12 months, exacerbating emotional distress without clear mitigation from conservative approaches alone.[90] Persistent vulvar symptoms further compound morbidity, including chronic pain (41%), swelling (14%), and difficulties with sitting (18%), reported at rates significantly exceeding general population norms (e.g., vulvar pain 20% vs. 6%).[89] Physical functioning declines durably (e.g., -15 points on EORTC scale at 12 months), alongside elevated fatigue and social limitations, independent of cancer stage in some cohorts.[90] While reconstruction may alleviate some issues, it associates with higher readmission risks without eliminating these outcomes.[91] Overall, these morbidities underscore the trade-offs in radical surgery, with conservative techniques like sentinel biopsy reducing lymphedema but not fully abrogating sexual or symptomatic burdens.[87]Oncologic Outcomes
Survival Statistics by Stage
Five-year relative survival rates for vulvar cancer, the primary indication for vulvectomy, demonstrate a strong inverse correlation with FIGO stage at diagnosis, reflecting the efficacy of surgical resection in early disease and the challenges of nodal or distant involvement. Data from population-based analyses indicate rates ranging from over 90% in stage I to approximately 25% in stage IV, with radical or modified vulvectomy often combined with lymphadenectomy forming the cornerstone of treatment for stages I-III.[92] These figures account for multimodal approaches in advanced cases but underscore surgery's role in achieving local control, though outcomes decline with age, comorbidity, and positive margins.[92] [93]| FIGO Stage | Description | 5-Year Relative Survival Rate |
|---|---|---|
| I | Tumor confined to vulva or perineum, ≤2 cm (IA) or >2 cm (IB), no nodal metastasis | 91.9%[92] |
| II | Tumor of any size with adjacent spread (e.g., lower urethra, vagina, anus) but negative nodes | 78.2%[92] |
| III | Positive inguinofemoral nodes or adjacent spread with nodal involvement | 59.6%[92] |
| IV | Invasion of upper urethra/anal mucosa, bladder/rectal mucosa, fixed/ulcerative pelvic nodes, or distant metastasis | 25.1%[92] |
Recurrence and Prognostic Factors
Recurrence following vulvectomy for vulvar squamous cell carcinoma occurs in approximately 22-37% of cases, with rates varying by stage and treatment factors.[95][97] Most recurrences (40-80%) manifest within two years of primary surgery, often as isolated local vulvar lesions amenable to salvage excision.[98] Patterns of recurrence typically involve the vulva (53%), followed by inguinal nodes (19%), multisite disease (14%), distant metastases (8%), and pelvic sites (6%), with median time to relapse around 15 months.[34][99] Lymph node involvement remains the dominant prognostic factor, correlating with reduced 5-year survival from 80-90% in node-negative early-stage disease to 20-40% in node-positive cases, independent of vulvectomy extent.[100] Advanced FIGO stage (>I) independently predicts regional and distant recurrences, while surgical margins below 8 mm strongly forecast local vulvar relapse, outperforming other variables in multivariate models.[101][102] Tumor factors such as size >4 cm, poor differentiation, lymphovascular space invasion, and deep stromal invasion (>5 mm) further worsen outcomes, with older age (>70 years) linked to higher recurrence risk.[95][103] Adjuvant radiotherapy mitigates local recurrence in node-positive or margin-involved cases, reducing vulvar-only relapse from 26% to 23%, though it does not alter distant failure rates.[104]| Prognostic Factor | Impact on Recurrence/Survival | Source |
|---|---|---|
| Positive inguinal nodes | Increases risk; 5-year survival drops to 20-40% | [100] |
| Inadequate margins (<8 mm) | Strongest predictor of local recurrence | [102] |
| FIGO stage >I | Predicts regional/distant failure | [101] |
| LVSI or deep invasion | Adverse for progression-free survival | [103] [105] |
| BMI ≥25 kg/m² | Associated with worse outcomes | [106] |