Multiple endocrine neoplasia type IIa

Last revised by Craig Hacking on 17 Oct 2024

Multiple endocrine neoplasia (MEN) type IIa, also known as Sipple syndrome, accounts for most cases of MEN2 and is characterized by:

Mnemonic: 

  • PMP

The estimated prevalence of MEN2a is about 1 per 50,000 4.

MEN2a is subdivided into 4 groups 4:

  1. Classical MEN 2A associating MTC, pheochromocytoma and primary hyperparathyroidism

  2. MEN 2A associated with cutaneous lichen amyloidosis

  3. MEN 2A associated with Hirschsprung disease

  4. familial MTC (FMTC)

A small proportion of individuals have a RET D631 proto-oncogene mutation. RET mutation is different from the RET translocation in papillary thyroid carcinoma. The chromosome locus is 10q11.2. 3

Cases and figures

  • Case 1: pheochromocytoma
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