Multiple endocrine neoplasia: Difference between revisions

Jump to navigation Jump to search
No edit summary
Line 2: Line 2:
{{CMG}}; {{AE}} {{Ammu}}
{{CMG}}; {{AE}} {{Ammu}}


{{SK}} MEN syndromes
{{SK}} MEN syndromes; Familial endocrine adenomatosis; Familial chromaffinomatosis; Multiple endocrine adenomatosis; Multiple endocrine neoplasia syndrome; MEN; MEN-Multiple endocrine neoplasia syndrome; Multiple endocrine neoplasms
==Overview==
==Overview==
Multiple endocrine neoplasia (MEN) encompasses several distinct [[syndrome]]s featuring [[Endocrine gland neoplasm|tumors of endocrine gland]]s, 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]].
Multiple endocrine neoplasia (MEN) encompasses several distinct [[syndrome]]s featuring [[Endocrine gland neoplasm|tumors of endocrine gland]]s, 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]].

Revision as of 03:50, 1 October 2015

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; Familial chromaffinomatosis; Multiple endocrine adenomatosis; Multiple endocrine neoplasia syndrome; MEN; MEN-Multiple endocrine neoplasia syndrome; Multiple endocrine neoplasms

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

 
 
 
 
 
 
 
 
 
 
 
 
 
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 cancer without pheochromocytoma or parathyroid hyperplasia
 
 
Medullary thyroid cancer, pheochromocytoma and mucosal neuromas or intestinal ganglioneuromas and marfanoid habitus
 
 
 
 
 
 
 
 
 
 

Pathophysiology

Comparison

Presentations of Multiple endocrine neoplasia.

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

Treatment

Surgery

  • Surgery is the mainstay of treatment for tumors of multiple endocrine neoplasia.

References

  1. Template:DorlandsDict
  2. 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. 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.
  4. [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
  5. 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.
  6. Marx, Stephen J (2011). "Chapter 41: Multiple endocrine neoplasia". In Melmed, Shlomo. Williams Textbook of Endocrinology, 12th ed. pp. 1728–1767.
  7. 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.
  8. 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.
  9. 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.
  10. 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.


Template:WikiDoc Sources