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
Memory device: 3 Ps.
Note:
‡ May be mislabeled as parathyroid hyperplasia.[2]
MEN 2A (II)
- Parathyroid adenoma.
- Medullary thyroid carcinoma (usually preceded by C-cell hyperplasia[3]).
- 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 (usually preceded by C cell hyperplasia[3]).
- Pheochromocytoma.
Details
MEN 1
Gene:
- MEN1.[1]
Characteristics:[3]
- 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.[4]
- Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) has been described in the context of multiple endocrine neoplasia 1.[5]
MEN 2A
Gene:
- RET.[6]
Characteristics:[3]
- 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.[3]
MEN 2B
Gene:
- RET.[7]
Characteristics:
- Marfanoid habitus.[8]
- Neuromas.
- Multiple ganglioneuromas (ganglioneuromatosis) of the alimentary tract.[7][9]
- Ganglion cells + spindle cells.[10]
Treatment:
- Patients are advised to have prophylactic thyroidectomy.
References
- ↑ 1.0 1.1 Online 'Mendelian Inheritance in Man' (OMIM) 131100
- ↑ Doherty GM, Lairmore TC, DeBenedetti MK (November 2004). "Multiple endocrine neoplasia type 1 parathyroid adenoma development over time". World J Surg 28 (11): 1139–42. doi:10.1007/s00268-004-7560-8. PMID 15490065.
- ↑ 3.0 3.1 3.2 3.3 3.4 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/.
- ↑ Davies, SJ.; Gosney, JR.; Hansell, DM.; Wells, AU.; du Bois, RM.; Burke, MM.; Sheppard, MN.; Nicholson, AG. (Mar 2007). "Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease.". Thorax 62 (3): 248-52. doi:10.1136/thx.2006.063065. PMID 17099078.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 171400
- ↑ 7.0 7.1 Online 'Mendelian Inheritance in Man' (OMIM) 162300
- ↑ 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.
- ↑ 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.