Multiple endocrine neoplasia type IIa

Last revised by Mohammad Taghi Niknejad on 18 Jul 2023

Citation, DOI, disclosures and article data

Citation:

Gaillard F, Niknejad M, Mohajeri S, et al. Multiple endocrine neoplasia type IIa. Reference article, Radiopaedia.org (Accessed on 24 Apr 2024) https://doi.org/10.53347/rID-1621

DOI:

https://doi.org/10.53347/rID-1621

Permalink:

https://radiopaedia.org/articles/1621

rID:

1621

Article created:

2 May 2008, Frank Gaillard ◉ ◈

Disclosures:

At the time the article was created Frank Gaillard had no recorded disclosures.

View Frank Gaillard's current disclosures

Last revised:

18 Jul 2023, Mohammad Taghi Niknejad ◉

Disclosures:

At the time the article was last revised Mohammad Taghi Niknejad had no financial relationships to ineligible companies to disclose.

View Mohammad Taghi Niknejad's current disclosures

Revisions:

13 times, by 10 contributors - see full revision history and disclosures

Systems:

Head & Neck

Sections:

Syndromes

Tags:

refs, endocrine

Synonyms:

MEN IIa
Multiple endocrine neoplasia type IIa
MEN 2a
Sipple syndrome

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

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

Mnemonic:

PMP

Pathology

Genetics

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