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


==Overview==
==Overview==
'''Multiple endocrine [[neoplasia]]''' ('''MEN''') (or "multiple endocrine [[adenomas]]", or "multiple endocrine adenomatosis" -- "MEA") consists of three [[syndrome]]s featuring tumors of [[endocrine gland]]s, each with its own characteristic pattern. The presence of any one tumor type does not automatically have a patient labelled as MEN, but a search of the other at-risk areas is usually undertaken, especially when there are suggestive clinical signs.
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==
MEN syndromes are inherited as [[autosomal dominant]] disorders. Medullary carcinoma of the thyroid may occur as an autosomal dominant in the absence of other features.
* 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}}


==Comparison==
==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>


* 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"
{| class="wikitable"
|-
|-
Line 33: Line 39:
|-
|-
! [[Multiple endocrine neoplasia type 2|MEN 2A]]
! [[Multiple endocrine neoplasia type 2|MEN 2A]]
! [[Multiple endocrine neoplasia type 2|MEN 2B]]
! Multiple endocrine neoplasia type 2b|MEN 2B
! FMTC
! [[Medullary thyroid cancer|FMTC]]
|-
|-
| [[Eponym]]
| Eponym
| Wermer syndrome
| [[Wermer syndrome]]
| Sipple syndrome
| [[Sipple syndrome]]
| (see below)
| (multiple)
| (none)
| (none)
|-
|-
Line 49: Line 55:
|-
|-
| [[Pancreatic]] tumors
| [[Pancreatic]] tumors
| [[insulinoma]], [[gastrinoma]]
| [[gastrinoma]] (50%<ref name=agabegi2nd4-7/>), <br>[[insulinoma]] (20%<ref name=agabegi2nd4-7/>), <br>[[vipoma]], <br>[[glucagonoma]], <br>[[PPoma]]  
| -
| -
| -
| -
Line 55: Line 61:
|-
|-
| [[Pituitary adenoma]]
| [[Pituitary adenoma]]
| Yes
| 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 />
| -
| -
| -
| -
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|-
|-
| [[Parathyroid]] hyperplasia
| [[Parathyroid]] hyperplasia
| Yes
| 90%<ref name=agabegi2nd4-7/>
| Yes
| 50%<ref name=agabegi2nd4-7/>
| -
| -
| -
| -
|-
|-
| [[thyroid cancer#medullary thyroid cancer (MTC)|Medullary thyroid carcinoma]]
| [[Medullary thyroid carcinoma]]
| -
| -
| Yes
| 100%<ref name=agabegi2nd4-7/>
| 100%
| 85%<ref name=agabegi2nd4-7/>
| 100%
| 100%
|-
|-
| [[Pheochromocytoma]]  
| [[Pheochromocytoma]]
| -
| -
| Yes
| >33%<ref name=agabegi2nd4-7/>
| 50%
| 50%
| -
| -
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| -
| -
|-
|-
| Multiple Mucosal Neuromata
| Mucosal [[neuroma]]
| -
| -
| -
| -
| >95%
| 100%<ref name=agabegi2nd4-7/>
| -
| -
|-
| Spontaneous mutation rate
|
|
| 50%
|
|-
|-
| [[Gene]](s)
| [[Gene]](s)
| [[MEN1|MEN1]] ({{OMIM4|131100}})
| [[MEN1]] ({{OMIM4|131100}})
| [[RET proto-oncogene|RET]] ({{OMIM4|164761}})
| [[RET proto-oncogene|RET]] ({{OMIM4|164761}})
| [[RET proto-oncogene|RET]] ({{OMIM4|164761}})
| [[RET proto-oncogene|RET]] ({{OMIM4|164761}})
| [[RET proto-oncogene|RET]] ({{OMIM4|164761}}),<br/>NTRK1 ({{OMIM4|191315}})
| [[RET proto-oncogene|RET]] ({{OMIM4|164761}}),<br/>[[NTRK1]] ({{OMIM4|191315}})
|-
|-
| Approx. [[prevalence]]
| 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
| 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)
| 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= |url=http://linkinghub.elsevier.com/retrieve/pii/0002-9343(54)90353-8}}</ref>
| 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= |url=}}</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
| 1965
|  
|
|}
|}
<nowiki>*</nowiki>- of patients with MEN1 and gastrinoma


(Blanks indicate that data are not yet available.)
{{#ev:youtube|9e5YudJLRYc}}
 
MEN 2B was known as MEN 3 for a short time in the 1970s, but that term is no longer used.
Although a variety of eponyms have been proposed for MEN2B (e.g. Williams-Pollock syndrome, Gorlin-Vickers syndrome, and Wagenmann-Froboese syndrome), none ever gained suffiicient traction to merit continued use and, indeed, are all but abandoned in the medical literature.  Another early report was Schimke ''et al'' in 1968.<ref>{{cite journal |author=Schimke RN, Hartmann WH, Prout TE, Rimoin DL |title=Syndrome of bilateral pheochromocytoma, medullary thyroid carcinoma and multiple neuromas. A possible regulatory defect in the differentiation of chromaffin tissue |journal=N. Engl. J. Med. |volume=279 |issue=1 |pages=1–7 |year=1968 |pmid=4968712 |doi= |url=}}</ref>
 
[[OMIM]] also includes a fourth form of multiple endocrine neoplasia ("MEN4"), associated with CDKN1B.<ref>{{OMIM|610755|MULTIPLE ENDOCRINE NEOPLASIA, TYPE IV; MEN4}}</ref> The presentation is believed to overlap that of MEN1 and MEN2.<ref name="pmid17030811">{{cite journal |author=Pellegata NS, Quintanilla-Martinez L, Siggelkow H, ''et al'' |title=Germ-line mutations in p27Kip1 cause a multiple endocrine neoplasia syndrome in rats and humans |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=103 |issue=42 |pages=15558–63 |year=2006 |month=Oct |pmid=17030811 |pmc=1622862 |doi=10.1073/pnas.0603877103 |url=http://www.pnas.org/cgi/pmidlookup?view=long&pmid=17030811}}</ref>
 
==MEN type 1==
{{main|Multiple endocrine neoplasia type 1}}
Type 1 is also known as Wermer's syndrome after Dr Paul Wermer, who described it in 1954:<ref>Wermer P. ''Genetic aspect of adenomatosis of endocrine glands.'' Am J Med 1954;16:363-371. PMID 13138607.</ref>
#[[Parathyroid]] hyperplasia/tumour causing [[hyperparathyroidism]].
#Pancreatic [[islet cell]] tumours causing [[hypoglycaemia]] ([[insulinoma]]) and [[Zollinger-Ellison syndrome]] ([[gastrinoma]]).
#[[Pituitary]] adenoma which may cause pituitary hormone excess.
The causative mutation is in the MEN1 gene at 11q13 which encodes a nuclear protein called menin that is believed to act as a tumor suppressor. Most cases of multiple endocrine neoplasia type 1 are inherited in an [[autosomal dominant]] pattern.
 
==MEN type 2==
===MEN type 2/type 2a===
{{main|Multiple endocrine neoplasia type 2}}
 
MEN syndrome types 2 and 3 have their basis in molecular genetics. Individuals can be tested for this genetic disorder reliably even when asymptomatic. The mutation is in the [[RET proto-oncogene]]. Most cases of multiple endocrine neoplasia types 2 and 3 are inherited in an [[autosomal dominant]] pattern.
 
Type 2 is also known as Sipple syndrome (after the American Dr John H. Sipple, who described it in 1961)<ref>Sipple JH. ''The association of pheochromocytoma with carcinoma of the thyroid gland.'' Am J Med 1961;31:163-166.</ref> and used to be called type 2A:
 
#[[Medullary carcinoma]] of the [[thyroid]] which is associated with increased [[calcitonin]] secretion. A test for elevated calcitonin should be done after [[pentagastrin]] injection and/or calcium infusion, to ensure that all affected patients are detected.
#[[Pheochromocytoma]]
#[[Parathyroid]] hyperplasia/tumour causing [[hyperparathyroidism]].
 
===MEN type 3/type 2b===
This syndrome has no eponym; it was described by Schimke ''et al'' in 1968.<ref>Schimke RN, Hartmann WH, Prout TE, Rimoin DL. ''Syndrome of bilateral pheochromocytoma, medullary thyroid carcinoma and multiple neuromas. A possible regulatory defect in the differentiation of chromaffin tissue.'' [[N Engl J Med]] 1968;279:1-7. PMID 4968712</ref> Originally thought to be a third MEN, then considered a variant of II (especially after linkage to ''RET'' was confirmed), it is now considered its own syndrome.
#[[Pheochromocytoma]]
#Medullary carcinoma of [[thyroid]] which is associated with increased [[calcitonin]] secretion. A test for elevated calcitonin should be done after pentagastrin injection and/or calcium infusion, to ensure that all affected patients are detected.
#Mucosal neuromas which are usually situated in the [[gastrointestinal tract]].
#[[Marfan syndrome|Marfanoid]] habitus


==References==
==References==
{{reflist|2}}
{{Reflist|2}}


==External links==
{{WikiDoc Help Menu}}
* [http://endocrine.niddk.nih.gov/ Endocrine and Metabolic Diseases Information Service]
{{WikiDoc Sources}}
* [http://www.amend.org.uk/ The Association for Multiple Endocrine Neoplasia Disorders (AMEND)]
* [http://www.arup.utah.edu/database/MEN2/MEN2_welcome.php/ Multiple Endocrine Neoplasia type 2 (MEN2 RET database)]


 
[[Category:Disease]]
{{Endocrine gland neoplasia}}
[[Category:Oncology]]
{{Endocrine pathology}}
[[Category:Up-To-Date]]
{{Tumor morphology}}
[[Category:Oncology]]
{{SIB}}
[[Category:Medicine]]
[[Category:Endocrinology]]
[[Category:Endocrinology]]
[[Category:Hereditary cancers]]
[[Category:Surgery]]
[[Category:Oncology]]
 
 
 
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[[it:Neoplasie multiendocrine]]
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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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