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Multiple endocrine neoplasia



Multiple endocrine neoplasia
Classification & external resources
ICD-10 D44.8
MeSH D009377

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.

Additional recommended knowledge

The older names "multiple endocrine adenomas", or "multiple endocrine adenomatosis" -- "MEA" -- should not be used.

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.

Feature MEN 1 MEN 2
MEN 2A MEN 2B FMTC
Eponym Wermer syndrome Sipple syndrome Williams-Pollock syndrome
Gorlin-Vickers syndrome
Wagenmann-Froboese syndrome
(none)
OMIM # 131100 171400 162300 155240
Pancreatic tumors insulinoma, gastrinoma - - -
Pituitary adenoma Yes - - -
Parathyroid hyperplasia Yes Yes - -
Medullary thyroid carcinoma - Yes 100% 100%
Pheochromocytoma - Yes 50% -
Marfanoid body habitus - - 80% -
multiple mucosal neuromata - - >95% -
spontaneous mutation rate 50%
Gene(s) MEN1 RET (164761) RET (164761) RET (164761),
NTRK1 (191315)
Approx. prevalence 1 in 1,000,000
Initial description (year) 1954[1] 1961[2] 1965

(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.[3]

In November 2007, cardiologist John G. Sotos announced his hypothesis that Abraham Lincoln, the 16th President of the United States (1861–1865), had MEN 2B.[4][5]

References

  1. ^ Wermer P. Genetic aspect of adenomatosis of endocrine glands. Am J Med 1954;16:363-371. PMID 13138607.
  2. ^ Sipple JH. The association of pheochromocytoma with carcinoma of the thyroid gland. Am J Med 1961;31:163-166.
  3. ^ Schimke RN, Hartmann WH, Prout TE, Rimoin DL. Syndrome of bilateral pheochromocytoma, medullary thyroid carcinoma and multiple neuromas. A possible regulatory defect in the differentiation of chromaffin tissue. N Engl J Med 1968;279:1-7. PMID 4968712
  4. ^ http://www.physical-lincoln.com/ The Physical Lincoln
  5. ^ Brown, David. "Is Lincoln Earliest Recorded Case of Rare Disease?", washingtonpost.com, 2007-11-26. Retrieved on 2007-11-26. 

pancreas (Insulinoma, Glucagonoma, Gastrinoma, VIPoma, Somatostatinoma)

Cholangiocarcinoma - Hepatocellular adenoma/Hepatocellular carcinoma - Adenoid cystic carcinoma - Familial adenomatous polyposis - Prolactinoma - Oncocytoma - Hurthle cell - Clear cell adenoma/adenocarcinoma - Renal cell carcinoma - Multiple endocrine neoplasia - Endometrioid tumor
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
  This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Multiple_endocrine_neoplasia". A list of authors is available in Wikipedia.
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