Multiple endocrine neoplasia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [4]

Synonyms and Keywords: MEN syndromes; Familial endocrine adenomatosis; Multiple endocrine adenomatosis; Multiple endocrine neoplasia syndrome; MEN; MEN-Multiple endocrine neoplasia syndrome; Multiple endocrine neoplasms

For multiple endocrine neoplasia type 1, click here.
For multiple endocrine neoplasia type 2a, click here.
For multiple endocrine neoplasia type 2b, click here.
For multiple endocrine neoplasia type 4, click here.

Overview

Multiple endocrine neoplasia (MEN) encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. Multiple endocrine neoplasia may be classified according to tumor characteristics into 3 subtypes: multiple endocrine neoplasia type 1, multiple endocrine neoplasia type 2 and multiple endocrine neoplasia type 4.

Classification

  • The following flowchart depicts the classification of multiple endocrine neoplasia.[1]
 
 
 
 
 
 
 
 
 
 
 
 
 
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 3
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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 thyroid carcinoma without pheochromocytoma or parathyroid hyperplasia
 
 
Medullary thyroid cancer, pheochromocytoma, marfanoid habitus, and mucosal neuromas or intestinal ganglioneuromas
 
 
 
 
 
 
 
 
 
 

Comparison

  • The following diagram compares the various types of multiple endocrine neoplasia.[2]
Presentations of Multiple endocrine neoplasia.























  • The following table compares the various types of multiple endocrine neoplasia.[3]
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%[4]),
insulinoma (20%[4]),
vipoma,
glucagonoma,
PPoma
- - -
Pituitary adenoma 66%[4] - - -
Angiofibroma 64%*[5] - - -
Lipoma 17%*[5] - - -
Parathyroid hyperplasia 90%[4] 50%[4] - -
Medullary thyroid carcinoma - 100%[4] 85%[4] 100%
Pheochromocytoma - >33%[4] 50% -
Marfanoid body habitus - - 80% -
Mucosal neuroma - - 100%[4] -
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)[6]
1 in 40,000[7] 1 in 1,000,000
(1 in 600,000[8] to
1 in 4,000,000[9])[10]
Initial description (year) 1954[11] 1961[12] 1965

*- of patients with MEN1 and gastrinoma

References

  1. Wells SA, Asa SL, Dralle H, Elisei R, Evans DB, Gagel RF; et al. (2015). "Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma.". Thyroid. 25 (6): 567–610. PMC 4490627Freely accessible. PMID 25810047. doi:10.1089/thy.2014.0335. 
  2. Multiple endocrine neoplasia type 2. Wikipedia(30.09,2015)https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia accessed on September, 30, 2015
  3. Multiple endocrine neoplasia type 2. Wikipedia(30.09,2015)https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia accessed on September, 30, 2015
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 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. 
  5. 5.0 5.1 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. PMID 15531478. doi:10.1210/jc.2004-0218. 
  6. [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
  7. 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. PMID 19169490. doi:10.1590/S0004-27302008000800021. 
  8. Marx, Stephen J (2011). "Chapter 41: Multiple endocrine neoplasia". In Melmed, Shlomo. Williams Textbook of Endocrinology, 12th ed. pp. 1728–1767. 
  9. Moline J, Eng C. (2011). "Multiple endocrine neoplasia type 2: An overview". Genetics in Medicine. 13 (9): 755–764. PMID 21552134. doi:10.1097/GIM.0b013e318216cc6d. 
  10. 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.
  11. Wermer P (1954). "Genetic aspects of adenomatosis of endocrine glands". Am. J. Med. 16 (3): 363–71. PMID 13138607. doi:10.1016/0002-9343(54)90353-8. 
  12. 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. 

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