Multiple endocrine neoplasia type 1
Updates to Article Attributes
Body
was changed:
Multiple endocrine neoplasia (MEN) type I is also known as Wermer syndrome.
Clinical presentation
Primary hyperparathyroidism is one of the commonest presentations.
Pathology
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
Associations
- lipomas
- angiofibromas
-
adrenal cortical lesions
- adrenal adenomas
- adrenocortical hyperplasia
- cortisol-secreting adenomas
- adrenal carcinomas (rare)
- 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.
See also
-<a title="Pancreatic neuroendocrine tumours" href="/articles/endocrine-tumours-of-the-pancreas">islet cell tumours of the pancreas</a>: 50% of patients, and a significant cause of mortality, most often <a href="/articles/gastrinoma">gastrinomas </a>followed by <a href="/articles/glucogonoma">glucagonoma</a>- +<a href="/articles/endocrine-tumours-of-the-pancreas">islet cell tumours of the pancreas</a>: 50% of patients, and a significant cause of mortality, most often <a href="/articles/gastrinoma">gastrinomas </a>followed by <a title="Glucagonoma" href="/articles/glucagonoma">glucagonoma</a>
Unable to process the form. Check for errors and try again.