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Multiple endocrine neoplasia
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Multiple endocrine neoplasia
Multiple endocrine neoplasia (abbreviated MEN) is a condition which encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. In some cases, the tumors are malignant, in others, benign. Benign or malignant tumors of nonendocrine tissues occur as components of some of these tumor syndromes.
MEN syndromes are inherited as autosomal dominant disorders.
Although not officially categorized as multiple endocrine neoplasia syndromes, Von Hippel–Lindau disease and Carney complex are two other autosomal dominant endocrine tumor syndromes with features that overlap the clinical features of the MEN syndromes. Although not transmitted in the germline, McCune–Albright syndrome is a genetic disorder characterized by endocrine neoplastic features involving endocrine glands that overlap with those involved in MEN1 or MEN2.
Percentages in the table below refer to the percentage of people with the MEN type who develop the neoplasia type.
*- of patients with MEN1 and gastrinoma
FMTC = familial medullary thyroid cancer
MEN 2B is sometimes known as MEN 3 and the designation varies by institution (cf. www.ClinicalReview.com). Although a variety of additional eponyms have been proposed for MEN2B (e.g. Williams-Pollock syndrome, Gorlin-Vickers syndrome, and Wagenmann–Froboese syndrome), none ever gained sufficient traction to merit continued use and, indeed, are all but abandoned in the medical literature. Another early report was Schimke et al. in 1968.
OMIM also includes a fourth form of multiple endocrine neoplasia ("MEN4"), associated with CDKN1B. The presentation is believed to overlap that of MEN1 and MEN2.
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Multiple endocrine neoplasia
Multiple endocrine neoplasia (abbreviated MEN) is a condition which encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. In some cases, the tumors are malignant, in others, benign. Benign or malignant tumors of nonendocrine tissues occur as components of some of these tumor syndromes.
MEN syndromes are inherited as autosomal dominant disorders.
Although not officially categorized as multiple endocrine neoplasia syndromes, Von Hippel–Lindau disease and Carney complex are two other autosomal dominant endocrine tumor syndromes with features that overlap the clinical features of the MEN syndromes. Although not transmitted in the germline, McCune–Albright syndrome is a genetic disorder characterized by endocrine neoplastic features involving endocrine glands that overlap with those involved in MEN1 or MEN2.
Percentages in the table below refer to the percentage of people with the MEN type who develop the neoplasia type.
*- of patients with MEN1 and gastrinoma
FMTC = familial medullary thyroid cancer
MEN 2B is sometimes known as MEN 3 and the designation varies by institution (cf. www.ClinicalReview.com). Although a variety of additional eponyms have been proposed for MEN2B (e.g. Williams-Pollock syndrome, Gorlin-Vickers syndrome, and Wagenmann–Froboese syndrome), none ever gained sufficient traction to merit continued use and, indeed, are all but abandoned in the medical literature. Another early report was Schimke et al. in 1968.
OMIM also includes a fourth form of multiple endocrine neoplasia ("MEN4"), associated with CDKN1B. The presentation is believed to overlap that of MEN1 and MEN2.
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