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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