Multiple endocrine neoplasia (MEN) type I is also known as Wermer syndrome.
Primary hyperparathyroidism is one of the commonest presentations.
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 major cause of mortality, most often gastrinomas followed by glucogonomas
- 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
History and etymology
It was first characterised by P Wermer et al. in 1954 3.
Treatment and prognosis
Pancreatic malignancy is the leading cause of mortality in MEN type I.
- 1. Weissleder R, Wittenberg J, Harisinghani MG. Primer of diagnostic imaging. Mosby Inc. (2007) ISBN:0323040683. Read it at Google Books - Find it at Amazon
- 2. 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
- 3. Wermer P. Genetic aspects of adenomatosis of endocrine glands. Am. J. Med. 1954;16 (3): 363-71. Am. J. Med. (link) - Pubmed citation
- 4. 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
- 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
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Multiple endocrine neoplasia type 1 (MEN I)||✗|
|Multiple endocrine neoplasia type I||✗|
|Multiple endocrine neoplasia type 1||✗|
|multiple endocrine neoplasia syndrome type 1||✗|