Multiple endocrine neoplasia type I (MEN1), also known as Wermer syndrome, is an autosomal dominant genetic disease that results in proliferative lesions in multiple endocrine organs, particularly the pituitary gland, islet cells of the pancreas and parathyroid glands.
Primary hyperparathyroidism is the commonest presentation, followed by pancreatic islet cell tumor with associated hypersecretion syndromes; gastrinomas are most common and associated with Zollinger-Ellison syndrome 7.
The abnormality is related to a tumor suppressor gene located in chromosome 11q13. MEN type I is an autosomal dominant syndrome characterized by 1-7:
- prolactinoma (most common)
- 30% of patients
- islet cell tumors of the pancreas
- proliferative parathyroid gland lesions 7
Handy mnemonics for recalling MEN type I:
- PPP or PiParPanc
In addition to the aforementioned characteristic lesions involving the pituitary, parathyroid and pancreas, numerous other lesions are encountered with greater frequency in patients with MEN1. These include:
- adrenal cortical lesions
- carcinoid tumors
- hepatic focal nodular hyperplasia 5
Treatment and prognosis
Treatment is directed to each individual manifestation. These are therefore discussed separately.
Pancreatic malignancy is the leading cause of mortality in MEN type I.
History and etymology
MEN1 was first characterized by P Wermer et al. in 1954 3.
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