Multiple endocrine neoplasia type I

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

There are other multiple endocrine neoplasia syndromes and these are discussed separately. 

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 on chromosome 11q13. MEN type I is an autosomal dominant syndrome characterized by 1-7:

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: 

Treatment is directed to each individual manifestation. These are therefore discussed separately.

Pancreatic malignancy is the leading cause of mortality in MEN type I. 

MEN1 was first characterized by P Wermer et al. in 1954 3.

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Article information

rID: 1619
Section: Syndromes
Synonyms or Alternate Spellings:
  • MEN I
  • Wermer syndrome
  • MEN 1
  • Multiple endocrine neoplasia type 1 (MEN I)
  • Multiple endocrine neoplasia type I
  • Multiple endocrine neoplasia type 1
  • Men1
  • multiple endocrine neoplasia syndrome type 1
  • MEN1

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