Sandbox MEN
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]
Overview
Classification
| Multiple endocrine neoplasia | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Multiple endocrine neoplasia type 1 | Multiple endocrine neoplasia type 2 | Multiple endocrine neoplasia type 4 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Multiple endocrine neoplasia type 2A | Multiple endocrine neoplasia type 2B | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Multiple endocrine neoplasia type 2A classical | Multiple endocrine neoplasia type 2A with cutaneous lichen amyloidosis | Multiple endocrine neoplasia type 2A with hirschsprung disease | Familial medullary cancer without pheochromocytoma or parathyroid hyperplasia | Medullary thyroid cancer, pheochromocytoma and mucosal neuromas or intestinal ganglioneuromas and marfanoid habitus | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Pathophysiology
- 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, benign. Benign or malignant tumors of nonendocrine tissues occur as components of some of these tumor syndromes.
- MEN syndromes are inherited as autosomal dominant disorders.[1]
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 | Template:OMIM4 | Template:OMIM4 | Template:OMIM4 | Template:OMIM4 |
| Pancreatic tumors | gastrinoma (50%[2]), insulinoma (20%[2]), vipoma, glucagonoma, PPoma |
- | - | - |
| Pituitary adenoma | 66%[2] | - | - | - |
| Angiofibroma | 64%*[3] | - | - | - |
| Lipoma | 17%*[3] | - | - | - |
| Parathyroid hyperplasia | 90%[2] | 50%[2] | - | - |
| Medullary thyroid carcinoma | - | 100%[2] | 85%[2] | 100% |
| Pheochromocytoma | - | >33%[2] | 50% | - |
| Marfanoid body habitus | - | - | 80% | - |
| Mucosal neuroma | - | - | 100%[2] | - |
| Gene(s) | MEN1 (Template:OMIM4) | RET (Template:OMIM4) | RET (Template:OMIM4) | RET (Template:OMIM4), NTRK1 (Template:OMIM4) |
| Approx. prevalence | 1 in 35,000 (1 in 20,000 to 1 in 40,000)[4] |
1 in 40,000[5] | 1 in 1,000,000 (1 in 600,000[6] to 1 in 4,000,000[7])[8] |
|
| Initial description (year) | 1954[9] | 1961[10] | 1965 | |
*- of patients with MEN1 and gastrinoma
Associated Conditions
Surgery
- Surgery is the mainstay of treatment of tumors of multiple endocrine neoplasia.
References
- ↑ Template:DorlandsDict
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 Table 4-7 in:Elizabeth D Agabegi; Agabegi, Steven S. (2008). Step-Up to Medicine (Step-Up Series). Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-7153-6.
- ↑ 3.0 3.1 Asgharian, B; Turner, ML; Gibril, F; Entsuah, LK; Serrano, J; Jensen, RT (November 2004). "Cutaneous tumors in patients with multiple endocrine neoplasm type 1 (MEN1) and gastrinomas: prospective study of frequency and development of criteria with high sensitivity and specificity for MEN1". The Journal of Clinical Endocrinology and Metabolism. 89 (11): 5328–36. doi:10.1210/jc.2004-0218. PMID 15531478.
- ↑ [1] 123I labeled metaiodobenzylguanidine for diagnosis of neuroendocrine tumors. Jiang L, Schipper ML, Li P, Cheng Z, Reports in Medical Imaging. 2009: 2 79-89
- ↑ Dora JM, Siqueira DR, Meyer EL, Puñales MK, Maia AL (November 2008). "Pancreatitis as the first manifestation of multiple endocrine neoplasia type 2A". Arq Bras Endocrinol Metabol. 52 (8): 1332–6. doi:10.1590/S0004-27302008000800021. PMID 19169490.
- ↑ Marx, Stephen J (2011). "Chapter 41: Multiple endocrine neoplasia". In Melmed, Shlomo. Williams Textbook of Endocrinology, 12th ed. pp. 1728–1767.
- ↑ Moline J, Eng C. (2011). "Multiple endocrine neoplasia type 2: An overview". Genetics in Medicine. 13 (9): 755–764. doi:10.1097/GIM.0b013e318216cc6d. PMID 21552134.
- ↑ Martino Ruggieri (2005). Neurocutaneous Disorders : The Phakomatoses. Berlin: Springer. ISBN 3-211-21396-1. - Chapter: Multiple Endocrine Neoplasia Type 2B by Electron Kebebew, Jessica E. Gosnell and Emily Reiff. Pages 695-701. [2] This reference quotes a prevalence of 1 in 40,000, but this figure is inconsistent with the same reference's calculated incidence of 4 per 100 million per year for MEN2B.
- ↑ Wermer P (1954). "Genetic aspects of adenomatosis of endocrine glands". Am. J. Med. 16 (3): 363–71. doi:10.1016/0002-9343(54)90353-8. PMID 13138607.
- ↑ Sipple JH (1961). "The association of pheochromocytoma with carcinoma of the thyroid gland". Am. J. Med. 31: 163–6. doi:10.1016/0002-9343(61)90234-0.