Multiple endocrine neoplasia
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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 (II)
- Parathyroid adenoma.
- Medullary thyroid carcinoma (usu. preceded by C cell hyperplasia[2]).
- Pheochromocytoma.
Notes - memory device:
- Both MEN 1 and MEN 2A have parathyroid adenomas.
- Each has additional neoplasms below and above the diaphragm.
MEN 2B (III)
- Neuromas/ganglioneuromas.
- Medullary thyroid carcinoma (usu. preceded by C cell hyperplasia[2]).
- Pheochromocytoma.
Details
MEN 1
Characteristics:[2]
- Endocrine tumours usu. lead to most of the morbidity.
- Usu. arise in the duodenum.
- May present as Zollinger-Ellison syndrome - triad of pancreatic gastrinoma, gastric hypersecretion, small bowel peptic ulcers.[3]
MEN 2A
Characteristics:[2]
- Medullary thyroid carcinoma - in almost 100%.
Treatment:
- Patients are advised to have prophylactic thyroidectomy.
Images:
Trivia:
- In MEN 2A and 2B the RET gene is abnormally activated. In Hirschsprung disease, it is inactivated.[2]
MEN 2B
Characteristics:
- Marfanoid habitus.[4]
- Neuromas.
- Multiple ganglioneuromas (ganglioneuromatosis) of the alimentary tract.[5][6]
- Ganglion cells + spindle cells.[7]
Treatment:
- Patients are advised to have prophylactic thyroidectomy.
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 2.4 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. }}
- ↑ Zollinger RM, Ellison EH (1955). "Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas". Ann. Surg. 142 (4): 709–23; discussion, 724–8. doi:10.1097/00000658-195510000-00015. PMC 1465210. PMID 13259432. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1465210/.
- ↑ 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. }}
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 162300
- ↑ 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/.
- ↑ Iacobuzio-Donahue, Christine A.; Montgomery, Elizabeth A. (2005). Gastrointestinal and Liver Pathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 215. ISBN 978-0443066573.