Multiple Endocrine Neoplasia - Comparison

Comparison

Percentages in table below refer to how large fraction of people with the MEN type develop the neoplasia type.

Feature MEN 1 MEN 2
MEN 2A MEN 2B FMTC
Eponym Wermer syndrome Sipple syndrome (multiple) (none)
OMIM 131100 171400 162300 155240
Pancreatic tumors gastrinoma (50%),
insulinoma (20%),
vipoma,
glucagonoma,
PPoma
- - -
Pituitary adenoma 66% - - -
Angiofibroma 64%* - - -
Lipoma 17%* - - -
Parathyroid hyperplasia 90% 50% - -
Medullary thyroid carcinoma - 100% 85% 100%
Pheochromocytoma - >33% 50% -
Marfanoid body habitus - - 80% -
Mucosal neuroma - - 100% -
Gene(s) MEN1 (131100) RET (164761) RET (164761) RET (164761),
NTRK1 (191315)
Approx. prevalence 1 in 35,000
(1 in 20,000 to
1 in 40,000)
1 in 40,000 1 in 40,000
Initial description (year) 1954 1961 1965

*- 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 (c.f. 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.

Read more about this topic:  Multiple Endocrine Neoplasia

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