Multiple endocrine neoplasia type I (MEN1) is also known as Wermer syndrome.
Primary hyperparathyroidism is one of the commonest presentations.
The abnormality is related to a tumour suppressor gene located in chromosome 11q13. MEN type I is an autosomal dominant syndrome characterised by:
- pituitary adenomas: 30% of patients, most frequently prolactinomas
- islet cell tumours of the pancreas: 50% of patients, and a significant cause of mortality, most often gastrinomas followed by glucagonoma
- parathyroid hyperplasia: with resulting hyperparathyroidism seen in 80-95% of patients
Handy mnemonics for recalling MEN type I:
- PPP or PiParPanc
- adrenal cortical lesions
- carcinoid tumours
- hepatic focal nodular hyperplasia 5
- Zollinger-Ellison syndrome 6
Treatment and prognosis
Pancreatic malignancy is the leading cause of mortality in MEN type I.
History and etymology
It was first characterised by P Wermer et al. in 1954 3.
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- 3. Wermer P. Genetic aspects of adenomatosis of endocrine glands. Am. J. Med. 1954;16 (3): 363-71. Am. J. Med. (link) - Pubmed citation
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- 5. Vortmeyer AO, Lubensky IA, Skarulis M et-al. Multiple endocrine neoplasia type 1: atypical presentation, clinical course, and genetic analysis of multiple tumors. Mod. Pathol. 1999;12 (9): 919-24. Pubmed citation
- 6. Gibril F, Schumann M, Pace A et-al. Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. Medicine (Baltimore). 2004;83 (1): 43-83. doi:10.1097/01.md.0000112297.72510.32 - Pubmed citation