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Multiple endocrine neoplasia
The term multiple endocrine neoplasia (MEN) encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. In some cases, the tumors are malignant; in others, they are benign.
The presence of any one tumor type does not automatically have a patient labelled as MEN, but a search of the other at-risk areas is usually undertaken, especially when there are suggestive clinical signs. Confusingly, a single report of "MEN4" (OMIM 610755) has appeared.
The "MEN" syndromes discussed in this article are not the only medical conditions in which multiple endocrine tumors occur. Others include Carney complex and McCune-Albright syndrome. In general medical parlance, however, and in this article, "MEN" refers to the syndromes with "MEN" in their name, plus the entity of familial medullary thyroid cancer (FMTC).
MEN syndromes are inherited as autosomal dominant disorders.
(Blanks indicate data items yet-to-be-filled-in.)
MEN 2B was transiently known as MEN 3 in the 1970s, but that term is no longer used. An early report was Schimke et al in 1968.
|Adnexal And Skin appendage (8390-8429)||sweat gland (Hidrocystoma, Syringoma)|
|Cystic, Mucinous And Serous (8440-8499)||(Mucoepidermoid carcinoma - Cystadenoma/Cystadenocarcinoma/Pseudomyxoma peritonei - Signet ring cell carcinoma/Krukenberg tumor|
|Ductal, Lobular And Medullary (8500-8549)||Ductal carcinoma - Paget's disease of the breast/Extramammary Paget's disease|
|Acinar cell (8550-8559)||Acinic cell carcinoma|
|Complex epithelial (8560-8589)||Warthin's tumor - Thymoma|
|Gonadal (8590-8679)||Sex cord-stromal tumour - Thecoma - Granulosa cell tumour - Arrhenoblastoma/Sertoli-Leydig cell tumour|
|Paragangliomas And Glomus tumors (8680-8719)||Paraganglioma - Pheochromocytoma - Glomus tumor|
|Nevi and melanomas (8720-8799)||Melanocytic nevus - Nodular melanoma - Dysplastic nevus - Lentigo maligna melanoma - Superficial spreading melanoma - Blue nevus|