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{{Multiple endocrine neoplasia}}
{{Multiple endocrine neoplasia}}
{{CMG}}
'''For patient information, click [[{{PAGENAME}} (patient information)|here]].'''


==[[Multiple endocrine neoplasia overview|Overview]]==
{{CMG}}; {{AE}} {{Ammu}} {{Soroush}}


==[[Multiple endocrine neoplasia historical perspective|Historical Perspective]]==
{{SK}} MEN syndromes; Familial endocrine adenomatosis; Multiple endocrine adenomatosis; Multiple endocrine neoplasia syndrome; MEN; MEN-Multiple endocrine neoplasia syndrome; Multiple endocrine neoplasms
==[[Multiple endocrine neoplasia classification|Classification]]==
[[Multiple endocrine neoplasia classification#MEN type 1|MEN Type 1]] | [[Multiple endocrine neoplasia classification#MEN type 2/type 2a|MEN Type 2a]] | [[Multiple endocrine neoplasia classification#MEN type 3/type 2b|MEN Type 2b]]


==[[Multiple endocrine neoplasia pathophysiology|Pathophysiology]]==
:'''For multiple endocrine neoplasia type 1, click [[Multiple endocrine neoplasia type 1|here]].'''
:'''For multiple endocrine neoplasia type 2a, click [[Multiple endocrine neoplasia type 2|here]].'''
:'''For multiple endocrine neoplasia type 2b, click [[Multiple endocrine neoplasia type 2|here]].'''
:'''For multiple endocrine neoplasia type 4, click [[multiple endocrine neoplasia type 4|here]].'''


==[[Multiple endocrine neoplasia causes|Causes]]==
==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]].
==Classification==
* The following flowchart depicts the classification of multiple endocrine neoplasia.<ref name="pmid25810047">{{cite journal| author=Wells SA, Asa SL, Dralle H, Elisei R, Evans DB, Gagel RF et al.| title=Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma. | journal=Thyroid | year= 2015 | volume= 25 | issue= 6 | pages= 567-610 | pmid=25810047 | doi=10.1089/thy.2014.0335 | pmc=PMC4490627 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25810047  }} </ref>
{{Familytree/start}}
{{Familytree|boxstyle=background: #E0FFFF;| | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | |A01= Multiple endocrine neoplasia}}
{{Familytree|boxstyle=background: #E0FFFF;| | | | | | | | | |,|-|-|-|-|^|-|-|-|-|v|-|-|-|-|-|-|-|-|-|-|-|.| | | | | | |}}
{{Familytree|boxstyle=background: #E0FFFF;| | | | | | | | | B01 | | | | | | | | B02 | | | | | | | | | | B03 | | | | |B01= [[Multiple endocrine neoplasia type 1]]|B02= [[Multiple endocrine neoplasia type 2]]|B03= [[Multiple endocrine neoplasia type 4]]}}
{{Familytree|boxstyle=background: #E0FFFF;| | | | | | | | | | | | | | | | | |,|-|^|-|-|-|-|-|-|-|-|-|.| | | | |}}
{{Familytree|boxstyle=background: #E0FFFF;| | | | | | | | | | | | | | | | | C01 | | | | | | | | | | C02 | | | |C01= [[Multiple endocrine neoplasia type 2A]]|C02= [[Multiple endocrine neoplasia type 2B]]/Multiple endocrine neoplasia type 3}}
{{Familytree|boxstyle=background: #E0FFFF;| | | | | | | | | | | |,|-|-|-|v|-|^|-|-|v|-|-|-|.| | | | |!| | | | | | | | | | | | | |}}
{{Familytree|boxstyle=background: #E0FFFF;| | | | | | | | | | | D01 | | D02 | | | D03 | | D04 | | | D05 | | | | | | | | | | |D01= [[Multiple endocrine neoplasia type 2A]] classical|D02= [[Multiple endocrine neoplasia type 2A]] with cutaneous lichen amyloidosis|D03= [[Multiple endocrine neoplasia type 2A]] with [[Hirschsprung disease]]|D04= [[Familial medullary thyroid carcinoma]] without [[pheochromocytoma]] or [[parathyroid]] hyperplasia|D05= [[Medullary thyroid cancer]], [[pheochromocytoma]], marfanoid habitus, and [[mucosal neuroma]]s or intestinal [[ganglioneuroma]]s}}
{{Familytree/end}}


==[[Multiple endocrine neoplasia differential diagnosis|Differentiating Multiple Endocrine Neoplasia from other Diseases]]==
==Comparison==
* The following diagram compares the various types of multiple endocrine neoplasia.<ref> Multiple endocrine neoplasia type 2. Wikipedia(30.09,2015)https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia accessed on September, 30, 2015</ref>
[[File:Multiple endocrine neoplasia.png|left|500px|Presentations of Multiple endocrine neoplasia.]]
<br><br><br><br><br><br><br><br><br><br><br><br><br><br><br><br><br><br><br><br><br><br>


==[[Multiple endocrine neoplasia epidemiology and demographics|Epidemiology and Demographics]]==
* The following table compares the various types of multiple endocrine neoplasia.<ref> Multiple endocrine neoplasia type 2. Wikipedia(30.09,2015)https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia accessed on September, 30, 2015</ref>
{| class="wikitable"
|-
! rowspan="2" | Feature
! rowspan="2" | [[Multiple endocrine neoplasia type 1|MEN 1]]
! colspan="3" | [[Multiple endocrine neoplasia type 2|MEN 2]]
|-
! [[Multiple endocrine neoplasia type 2|MEN 2A]]
! Multiple endocrine neoplasia type 2b|MEN 2B
! [[Medullary thyroid cancer|FMTC]]
|-
| Eponym
| [[Wermer syndrome]]
| [[Sipple syndrome]]
| (multiple)
| (none)
|-
| [[OMIM]]
| {{OMIM4|131100}}
| {{OMIM4|171400}}
| {{OMIM4|162300}}
| {{OMIM4|155240}}
|-
| [[Pancreatic]] tumors
| [[gastrinoma]] (50%<ref name=agabegi2nd4-7/>), <br>[[insulinoma]] (20%<ref name=agabegi2nd4-7/>), <br>[[vipoma]], <br>[[glucagonoma]], <br>[[PPoma]]
| -
| -
| -
|-
| [[Pituitary adenoma]]
| 66%<ref name=agabegi2nd4-7>Table 4-7 in:{{cite book |author=Elizabeth D Agabegi; Agabegi, Steven S. |title=Step-Up to Medicine (Step-Up Series) |publisher=Lippincott Williams & Wilkins |location=Hagerstwon, MD |year=2008 |pages= |isbn=0-7817-7153-6 |oclc= |doi= |accessdate=}}</ref>
| -
| -
| -
|-
| [[Angiofibroma]]
| 64%*<ref name=Asgharian>{{cite journal|last=Asgharian|first=B |author2=Turner, ML |author3=Gibril, F |author4=Entsuah, LK |author5=Serrano, J |author6=Jensen, RT|title=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.|journal=The Journal of Clinical Endocrinology and Metabolism|date=November 2004|volume=89|issue=11|pages=5328–36|pmid=15531478|doi=10.1210/jc.2004-0218}}</ref>
| -
| -
| -
|-
| [[Lipoma]]
| 17%*<ref name=Asgharian />
| -
| -
| -
|-
| [[Parathyroid]] hyperplasia
| 90%<ref name=agabegi2nd4-7/>
| 50%<ref name=agabegi2nd4-7/>
| -
| -
|-
| [[Medullary thyroid carcinoma]]
| -
| 100%<ref name=agabegi2nd4-7/>
| 85%<ref name=agabegi2nd4-7/>
| 100%
|-
| [[Pheochromocytoma]]
| -
| >33%<ref name=agabegi2nd4-7/>
| 50%
| -
|-
| [[Marfan syndrome|Marfanoid]] body habitus
| -
| -
| 80%
| -
|-
| Mucosal [[neuroma]]
| -
| -
| 100%<ref name=agabegi2nd4-7/>
| -
|-
| [[Gene]](s)
| [[MEN1]] ({{OMIM4|131100}})
| [[RET proto-oncogene|RET]] ({{OMIM4|164761}})
| [[RET proto-oncogene|RET]] ({{OMIM4|164761}})
| [[RET proto-oncogene|RET]] ({{OMIM4|164761}}),<br/>[[NTRK1]] ({{OMIM4|191315}})
|-
| Approx. [[prevalence]]
| 1 in 35,000 <br> (1 in 20,000 to<br> 1 in 40,000)<ref>[http://www.dovepress.com/getfile.php?fileID=5129] 123I labeled metaiodobenzylguanidine for diagnosis of neuroendocrine tumors. Jiang L, Schipper ML, Li P, Cheng Z, Reports in Medical Imaging. 2009: 2 79-89</ref>
| 1 in 40,000<ref>{{cite journal |author=Dora JM, Siqueira DR, Meyer EL, Puñales MK, Maia AL |title=Pancreatitis as the first manifestation of multiple endocrine neoplasia type 2A |journal=Arq Bras Endocrinol Metabol |volume=52 |issue=8 |pages=1332–6 |date=November 2008 |pmid=19169490 |doi= 10.1590/S0004-27302008000800021|url=}}</ref>
| 1 in 1,000,000<br/>(1 in 600,000<ref>{{cite book |last=Marx |first=Stephen J |editor-last=Melmed |editor-first=Shlomo |title=Williams Textbook of Endocrinology, 12th ed. |year=2011 |pages=1728–1767 |chapter=Chapter 41: Multiple endocrine neoplasia }}</ref> to<br/>1 in 4,000,000<ref>{{cite journal |author=Moline J, Eng C. |title= Multiple endocrine neoplasia type 2: An overview|journal=Genetics in Medicine |volume=13 |issue=9 |pages=755–764 |year=2011 |pmid= 21552134 |url=http://www.nature.com/gim/journal/v13/n9/full/gim2011127a.html |doi=10.1097/GIM.0b013e318216cc6d}}</ref>)<ref>{{cite book |author=Martino Ruggieri |title=Neurocutaneous Disorders : The Phakomatoses |publisher=Springer |location=Berlin |year=2005 |pages= |isbn=3-211-21396-1 |oclc= |doi= |accessdate=}} - Chapter: ''Multiple Endocrine Neoplasia Type 2B '' by Electron Kebebew, Jessica E. Gosnell and Emily Reiff. Pages 695-701. [http://www.springerlink.com/content/qu78313220701167/]  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.</ref>
|
|-
| Initial description (year)
| 1954<ref>{{cite journal |author=Wermer P |title=Genetic aspects of adenomatosis of endocrine glands |journal=Am. J. Med. |volume=16 |issue=3 |pages=363–71 |year=1954 |pmid=13138607 |doi= 10.1016/0002-9343(54)90353-8|url=http://linkinghub.elsevier.com/retrieve/pii/0002-9343(54)90353-8}}</ref>
| 1961<ref>{{cite journal |author=Sipple JH |title=The association of pheochromocytoma with carcinoma of the thyroid gland |journal=Am. J. Med. |volume=31 |issue= |pages=163–6 |year=1961 |pmid= |doi= 10.1016/0002-9343(61)90234-0|url=}}</ref>
| 1965
|
|}
<nowiki>*</nowiki>- of patients with MEN1 and gastrinoma


==[[Multiple endocrine neoplasia risk factors|Risk Factors]]==
{{#ev:youtube|9e5YudJLRYc}}


==[[Multiple endocrine neoplasia screening|Screening]]==
==References==
 
{{Reflist|2}}
==[[Multiple endocrine neoplasia natural history|Natural History, Complications and Prognosis]]==
 
==Diagnosis==
[[Multiple endocrine neoplasia staging|Staging]] | [[Multiple endocrine neoplasia history and symptoms|History and Symptoms]] | [[Multiple endocrine neoplasia physical examination|Physical Examination]] | [[Multiple endocrine neoplasia laboratory tests|Laboratory Findings]] | [[Multiple endocrine neoplasia chest x ray|Chest X Ray]] | [[Multiple endocrine neoplasia CT|CT]] | [[Multiple endocrine neoplasia MRI|MRI]] | [[Multiple endocrine neoplasia echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Multiple endocrine neoplasia other imaging findings|Other Imaging Findings]] | [[Multiple endocrine neoplasia other diagnostic studies|Other Diagnostic Studies]]
 
==Treatment==
[[Multiple endocrine neoplasia medical therapy|Medical Therapy]] | [[Multiple endocrine neoplasia surgery|Surgery]] | [[Multiple endocrine neoplasia primary prevention|Primary Prevention]] | [[Multiple endocrine neoplasia secondary prevention|Secondary Prevention]] | [[Multiple endocrine neoplasia cost-effectiveness of therapy|Cost-Effectivenes of Therapy]] | [[Multiple endocrine neoplasia future or investigational therapies|Future or Investigational Therapies]]
 
==Case Studies==
[[Multiple endocrine neoplasia case study one|Case #1]]
 
==External Links==
* [http://endocrine.niddk.nih.gov/ Endocrine and Metabolic Diseases Information Service]


 
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Latest revision as of 16:43, 8 October 2019

Multiple endocrine neoplasia Microchapters

Patient Information

Overview

Classification

Multiple endocrine neoplasia type 1
Multiple endocrine neoplasia type 2
Multiple endocrine neoplasia type 4

Causes

Differential Diagnosis

For patient information, click here.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [4] Soroush Seifirad, M.D.[5]

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.
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 Template:OMIM4 Template:OMIM4 Template:OMIM4 Template:OMIM4
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 (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)[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

{{#ev:youtube|9e5YudJLRYc}}

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. doi:10.1089/thy.2014.0335. PMC 4490627. PMID 25810047.
  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. doi:10.1210/jc.2004-0218. PMID 15531478.
  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. doi:10.1590/S0004-27302008000800021. PMID 19169490.
  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. doi:10.1097/GIM.0b013e318216cc6d. PMID 21552134.
  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. doi:10.1016/0002-9343(54)90353-8. PMID 13138607.
  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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