Multiple endocrine neoplasia
277098
220466337
2008-06-20T00:18:11Z
69.219.158.85
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The term '''multiple endocrine [[neoplasia]]''' ('''MEN''') encompasses several distinct [[syndrome]]s featuring [[Endocrine gland neoplasm|tumors of endocrine gland]]s, 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.
The older names "multiple endocrine [[adenomas]]", or "multiple endocrine [[adenomatosis]]" (MEA) have been replaced by the current terminology.
The term multiple endocrine neoplasia is used when two or more endocrine tumor types, known to occur as a part of one of the defined MEN syndromes, occurs in a single patient and there is evidence for either a causative mutation or hereditary transmission. The presence of two or more tumor types in a single patient does not automatically designate that individual as having MEN because there is a small statistical chance that development of two "sporadic" tumors that occur in one of the MEN syndromes could occur by chance.
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 syndrome characterized by endocrine neoplastic features involving endocrine glands that overlap with those involved in MEN1 or MEN2.
Confusingly, a single report of "MEN4" (OMIM {{OMIM2|610755}}) has appeared.
MEN syndromes are inherited as [[autosomal dominant]] disorders.
{| class="wikitable"
|-
! rowspan="2" | Feature
! rowspan="2" | [[Multiple endocrine neoplasia type 1|MEN 1]]
! colspan="3" | [[Multiple endocrine neoplasia type 2|MEN 2]]
|-
! [[Multiple endocrine neoplasia type 2|MEN 2A]]
! [[Multiple endocrine neoplasia type 2|MEN 2B]]
! FMTC
|-
| [[Eponym]]
| Wermer syndrome
| Sipple syndrome
| (see below)
| (none)
|-
| [[OMIM]]
| {{OMIM2|131100}}
| {{OMIM2|171400}}
| {{OMIM2|162300}}
| {{OMIM2|155240}}
|-
| [[Pancreatic]] tumors
| [[insulinoma]], [[gastrinoma]]
| -
| -
| -
|-
| [[Pituitary adenoma]]
| Yes
| -
| -
| -
|-
| [[Parathyroid]] hyperplasia
| Yes
| Yes
| -
| -
|-
| [[thyroid cancer#medullary thyroid cancer (MTC)|Medullary thyroid carcinoma]]
| -
| Yes
| 100%
| 100%
|-
| [[Pheochromocytoma]]
| -
| Yes
| 50%
| -
|-
| [[Marfan syndrome|Marfanoid]] body habitus
| -
| -
| 80%
| -
|-
| [[Multiple Mucosal Neuromata]]
| -
| -
| >95%
| -
|-
| spontaneous mutation rate
|
|
| 50%
|
|-
| [[Gene]](s)
| [[MEN1|MEN1]] ({{OMIM2|131100}})
| [[RET proto-oncogene|RET]] ({{OMIM2|164761}})
| [[RET proto-oncogene|RET]] ({{OMIM2|164761}})
| [[RET proto-oncogene|RET]] ({{OMIM2|164761}}),<br/>NTRK1 ({{OMIM2|191315}})
|-
| Approx. [[prevalence]]
|
|
| 1 in 1,000,000
|
|-
| Initial description (year)
| 1954<ref>Wermer P. ''Genetic aspect of adenomatosis of endocrine glands.'' Am J Med 1954;16:363-371. PMID 13138607.</ref>
| 1961<ref>Sipple JH. ''The association of pheochromocytoma with carcinoma of the thyroid gland.'' Am J Med 1961;31:163-166.</ref>
| 1965
|
|}
(Blanks indicate that data are not yet available.)
MEN 2B was known as MEN 3 for a short time in the 1970s, but that term is no longer used.
Although a variety of eponyms have been proposed for MEN2B (e.g. Williams-Pollock syndrome, Gorlin-Vickers syndrome, and Wagenmann-Froboese syndrome), none ever gained suffiicient traction to merit continued use and, indeed, are all but abandoned in the medical literature. Another early report was Schimke ''et al'' in 1968.<ref>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</ref>
In November 2007, [[cardiologist]] John G. Sotos announced his hypothesis that [[Abraham Lincoln]], the 16th [[POTUS|President of the United States]] ([[1861]]–[[1865]]), had MEN 2B.<ref>http://www.physical-lincoln.com/ ''The Physical Lincoln''</ref><ref>{{cite news|title=Is Lincoln Earliest Recorded Case of Rare Disease?|publisher=washingtonpost.com|last=Brown|first=David|accessdate=2007-11-26| date=[[2007-11-26]]|url=http://www.washingtonpost.com/wp-dyn/content/story/2007/11/26/ST2007112600664.html?sid=ST2007112600664}}</ref>
==References==
{{reflist|2}}
==External links==
* [http://endocrine.niddk.nih.gov/ Endocrine and Metabolic Diseases Information Service]
* [http://www.amend.org.uk/ The Association for Multiple Endocrine Neoplasia Disorders (AMEND)]
{{Endocrine pathology}}
{{Tumor morphology}}
[[Category:Endocrinology]]
[[Category:Hereditary cancers]]
[[da:Multipel endokrin neoplasi]]
[[de:Multiple endokrine Neoplasie]]
[[fr:Néoplasie endocrinienne multiple]]
[[it:Neoplasie multiendocrine]]
[[pl:Mnoga gruczolakowatość wewnątrzwydzielnicza]]
[[sv:Multipla hormonella tumörer]]