multiple endokrine Neoplasie Typ 1

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.

Clinical presentation

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 .


The abnormality is related to a tumor suppressor gene located on chromosome 11q13. MEN type I is an autosomal dominant syndrome characterized by :

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 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 .

See also

Siehe auch:
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