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Multiple endocrine neoplasia type IIa

Dr Yuranga Weerakkody and Dr Frank Gaillard et al.

Multiple endocrine neoplasia (MEN) type IIa also known as Sipple syndrome, accounts for most cases of MEN II and is characterised by:

pheochromocytomas : in 50% of patients, often bilateral, and can be extra-adrenal
medullary thyroid cancer : 100% of patients. aggressive, and may elaborate calcitonin.
parathyroid hyperplasia : only seen in 20% of patients, and often presents with hypercalcaemia and renal calculi.

Mnemonic : PMP

Pathology

Genetics

A small proportion of individuals have a ret 631 proto-oncogene mutation

References

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1. Scarsbrook AF, Thakker RV, Wass JA et-al. Multiple endocrine neoplasia: spectrum of radiologic appearances and discussion of a multitechnique imaging approach. Radiographics. 2006;26 (2): 433-51. Radiographics (full text) - doi:10.1148/rg.262055073 - Pubmed citation
2. Callender GG, Rich TA, Perrier ND. Multiple endocrine neoplasia syndromes. Surg. Clin. North Am. 2008;88 (4): 863-95, viii. doi:10.1016/j.suc.2008.05.001 - Pubmed citation