Multiple endocrine neoplasia type I
Dr Yuranga Weerakkody and Dr Frank Gaillard et al.
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.
Other associations
- lipomas
- angiofibromas
-
adrenal cortical lesions
- adrenal adenomas
- adrenocortical hyperplasia
- cortisol-secreting adenomas
- adrenal carcinomas (rare)
- carcinoid tumours
Clinical presenation
Primary hyperparathyroidism is one of the commonest presentations.
Pathology
Genetics
Abnormality is related to a tumour suppresor gene located in chromosome 11q13
Etymology
It was first characterised by P Wermer et.al in 1954 3
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