Difference between revisions of "Multiple endocrine neoplasia"
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==Details== | ==Details== | ||
===MEN 1=== | |||
Characteristics:<ref name=Ref_PBoD8_1162>{{Ref PBoD8|1162}}}}</ref> | |||
*Endocrine tumours usu. lead to most of the morbidity. | |||
**Usu. arise in the duodenum. | |||
***Zollinger-Ellison syndrome is common. | |||
===MEN 2A=== | ===MEN 2A=== | ||
Characteristics:<ref name=Ref_PBoD8_1162>{{Ref PBoD8|1162}}}}</ref> | |||
*Medullary [[thyroid]] carcinoma - in almost 100%. | |||
*Ganglioneuromatosis of the alimentary tract.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/162300 http://www.ncbi.nlm.nih.gov/omim/162300]. Accessed on: 19 November 2010.</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> | *Ganglioneuromatosis of the alimentary tract.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/162300 http://www.ncbi.nlm.nih.gov/omim/162300]. Accessed on: 19 November 2010.</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> | ||
**Extra large nerves in GI tract. | **Extra large nerves in GI tract. | ||
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Images: | Images: | ||
*[http://wjso.com/content/5/1/19/figure/F3 Ganglioneuromatosis - crappy quality (wjso.com)]. | *[http://wjso.com/content/5/1/19/figure/F3 Ganglioneuromatosis - crappy quality (wjso.com)]. | ||
Trivia: | |||
*In MEN 2A and 2B the ''RET'' gene is abnormally activated. In [[Hirschsprung disease]], it is inactivated.<ref name=Ref_PBoD8_1162>{{Ref PBoD8|1162}}}}</ref> | |||
===MEN 2B=== | ===MEN 2B=== | ||
* | Characteristics: | ||
*[[Marfan syndrome|Marfanoid]] habitus.<ref name=Ref_PBoD8_1160>{{Ref PBoD8|1160}}}}</ref> | |||
==References== | ==References== | ||
Revision as of 21:14, 19 March 2011
Multiple endocrine neoplasia, abbreviated MEN, refers to collection of different genetic abnormalities that lead to endocrine neoplasms.
Simple
MEN 1
- Parathyroid adenoma.[1]
- Pancreatic neuroendocrine tumours.
- Pituitary adenoma.
Memory device: 3 Ps.
MEN 2A/2B (II/III)
- Parathyroid adenoma.
- Medullary thyroid carcinoma (usu. preceded by C cell hyperplasia[2]).
- Pheochromocytoma.
Notes:
- BOTH have parathyroid adenoma.
- EACH has one below and one above the diaphragm.
Details
MEN 1
Characteristics:[2]
- Endocrine tumours usu. lead to most of the morbidity.
- Usu. arise in the duodenum.
- Zollinger-Ellison syndrome is common.
- Usu. arise in the duodenum.
MEN 2A
Characteristics:[2]
- Medullary thyroid carcinoma - in almost 100%.
- Ganglioneuromatosis of the alimentary tract.[3][4]
- Extra large nerves in GI tract.
Images:
Trivia:
- In MEN 2A and 2B the RET gene is abnormally activated. In Hirschsprung disease, it is inactivated.[2]
MEN 2B
Characteristics:
References
- ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970475-2. Accessed on: 2 September 2010.
- ↑ 2.0 2.1 2.2 2.3 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1162. ISBN 978-1416031215. }}
- ↑ URL: http://www.ncbi.nlm.nih.gov/omim/162300. Accessed on: 19 November 2010.
- ↑ Haraguchi M, Kinoshita H, Koori M, et al. (2007). "Multiple rectal carcinoids with diffuse ganglioneuromatosis". World J Surg Oncol 5: 19. doi:10.1186/1477-7819-5-19. PMC 1805501. PMID 17306015. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1805501/.
- ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1160. ISBN 978-1416031215. }}