Multiple endocrine neoplasia (MEN) type I is also known as Wermer syndrome. It is an autosomal dominant syndrome characterised by:
- parathyroid hyperplasia - with resulting hyperparathyroidism seen in 80 - 95% of patients
- islet cell tumours of the pancreas - 50% of patients, and a major cause of mortality, most often gastrinomas followed by glucogonomas
- pituitary adenomas - 30% patients, most frequently prolactinomas.
- adrenal cortical lesions
- carcinoid tumours
Primary hyperparathyroidism is one of the commonest presentations.
Abnormality is related to a tumour suppresor gene located in chromosome 11q13
It was first characterised by P Wermer et.al in 1954 3
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- 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
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||✗|