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'''Multiple endocrine neoplasia''', abbreviated ''MEN'', refers to collection of different genetic abnormalities that lead to endocrine neoplasms. | [[Image:C-cell hyperplasia in MEN 2 -- low mag.jpg|thumb|right|350px|[[C-cell hyperplasia]], as may be seen in the context of MEN 2A or MEN 2B. [[H&E stain]].]] | ||
'''Multiple endocrine neoplasia''', abbreviated '''MEN''', refers to collection of different genetic abnormalities that lead to endocrine neoplasms. | |||
==Simple== | ==Simple== | ||
===MEN 1=== | ===MEN 1=== | ||
* [[Parathyroid adenoma]].<ref name=omim131100>{{OMIM|131100}}</ref> | * [[Parathyroid adenoma]].<ref name=omim131100>{{OMIM|131100}}</ref> ‡ | ||
* [[Pancreatic neuroendocrine tumour]]s. | * [[Pancreatic neuroendocrine tumour]]s. | ||
* [[Pituitary adenoma]]. | * [[Pituitary adenoma]]. | ||
Memory device: '''3 Ps'''. | Memory device: '''3 Ps'''. | ||
Note:<br> | |||
‡ May be mislabeled as ''[[parathyroid hyperplasia]]''.<ref name=pmid15490065>{{cite journal |authors=Doherty GM, Lairmore TC, DeBenedetti MK |title=Multiple endocrine neoplasia type 1 parathyroid adenoma development over time |journal=World J Surg |volume=28 |issue=11 |pages=1139–42 |date=November 2004 |pmid=15490065 |doi=10.1007/s00268-004-7560-8 |url=}}</ref> | |||
===MEN 2A (II)=== | ===MEN 2A (II)=== | ||
* [[Parathyroid adenoma]]. | * [[Parathyroid adenoma]]. | ||
* [[Medullary thyroid carcinoma]] ( | * [[Medullary thyroid carcinoma]] (usually preceded by [[C-cell hyperplasia]]<ref name=Ref_PBoD8_1162>{{Ref PBoD8|1162}}}}</ref>). | ||
* [[Pheochromocytoma]]. | * [[Pheochromocytoma]]. | ||
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===MEN 2B (III)=== | ===MEN 2B (III)=== | ||
* Neuromas/[[ganglioneuroma]]s. | * Neuromas/[[ganglioneuroma]]s. | ||
* [[Medullary thyroid carcinoma]] ( | * [[Medullary thyroid carcinoma]] (usually preceded by C cell hyperplasia<ref name=Ref_PBoD8_1162>{{Ref PBoD8|1162}}}}</ref>). | ||
* [[Pheochromocytoma]]. | * [[Pheochromocytoma]]. | ||
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Characteristics:<ref name=Ref_PBoD8_1162>{{Ref PBoD8|1162}}}}</ref> | Characteristics:<ref name=Ref_PBoD8_1162>{{Ref PBoD8|1162}}}}</ref> | ||
*Endocrine tumours | *Endocrine tumours usually lead to most of the morbidity. | ||
** | **Usually arise in the [[duodenum]]. | ||
**May present as [[Zollinger-Ellison syndrome]] - triad of pancreatic gastrinoma, gastric hypersecretion, small bowel peptic ulcers.<ref name=pmid13259432>{{cite journal |author=Zollinger RM, Ellison EH |title=Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas |journal=Ann. Surg. |volume=142 |issue=4 |pages=709–23; discussion, 724–8 |year=1955 |pmid=13259432|doi=10.1097/00000658-195510000-00015 |PMC = 1465210 |URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1465210/?page=1 }}</ref> | **May present as [[Zollinger-Ellison syndrome]] - triad of pancreatic gastrinoma, gastric hypersecretion, small bowel peptic ulcers.<ref name=pmid13259432>{{cite journal |author=Zollinger RM, Ellison EH |title=Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas |journal=Ann. Surg. |volume=142 |issue=4 |pages=709–23; discussion, 724–8 |year=1955 |pmid=13259432|doi=10.1097/00000658-195510000-00015 |PMC = 1465210 |URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1465210/?page=1 }}</ref> | ||
*[[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia]] (DIPNECH) has been described in the context of ''multiple endocrine neoplasia 1''.<ref name=pmid17099078>{{Cite journal | last1 = Davies | first1 = SJ. | last2 = Gosney | first2 = JR. | last3 = Hansell | first3 = DM. | last4 = Wells | first4 = AU. | last5 = du Bois | first5 = RM. | last6 = Burke | first6 = MM. | last7 = Sheppard | first7 = MN. | last8 = Nicholson | first8 = AG. | title = Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. | journal = Thorax | volume = 62 | issue = 3 | pages = 248-52 | month = Mar | year = 2007 | doi = 10.1136/thx.2006.063065 | PMID = 17099078 }}</ref> | |||
===MEN 2A=== | ===MEN 2A=== | ||
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===MEN 2B=== | ===MEN 2B=== | ||
Gene: | |||
*RET.<ref name=omim162300>{{OMIM|162300}}</ref> | |||
Characteristics: | Characteristics: | ||
*[[Marfan syndrome|Marfanoid]] habitus.<ref name=Ref_PBoD8_1160>{{Ref PBoD8|1160 | *[[Marfan syndrome|Marfanoid]] habitus.<ref name=Ref_PBoD8_1160>{{Ref PBoD8|1160}}</ref> | ||
*[[Neuroma]]s. | *[[Neuroma]]s. | ||
*Multiple ganglioneuromas ([[ganglioneuromatosis]]) of the alimentary tract.<ref name=omim162300>{{OMIM|162300}}</ref><ref name=pmid17306015>{{cite journal |author=Haraguchi M, Kinoshita H, Koori M, ''et al.'' |title=Multiple rectal carcinoids with diffuse ganglioneuromatosis |journal=World J Surg Oncol |volume=5 |issue= |pages=19 |year=2007 |pmid=17306015 |pmc=1805501 |doi=10.1186/1477-7819-5-19 |url=}}</ref> | *Multiple ganglioneuromas ([[ganglioneuromatosis]]) of the alimentary tract.<ref name=omim162300>{{OMIM|162300}}</ref><ref name=pmid17306015>{{cite journal |author=Haraguchi M, Kinoshita H, Koori M, ''et al.'' |title=Multiple rectal carcinoids with diffuse ganglioneuromatosis |journal=World J Surg Oncol |volume=5 |issue= |pages=19 |year=2007 |pmid=17306015 |pmc=1805501 |doi=10.1186/1477-7819-5-19 |url=}}</ref> |
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