Multiple endocrine neoplasia type 1 (MEN 1)

Autosomal dominant tumor syndrome arising from inactivating mutations of tumor suppressive gene (MEN 1) situated on chromosome 11q13.

MEN type 1 occurs in 1 in 30,000 people, is characterized by pituitary, parathyroid, pancreatic and adrenal tumors.

More than 250 different causative mutations with no genotype-phenotype correlation.

In multiple endocrine neoplasia type I, benign tumors of the parathyroid of the first manifestation of the disease, followed by potentially malignant tumors of other parts of the body, such as the pancreas.

Menin is the gene responsible for MEN 1 and it is a tumor suppressor gene.

Thyroid adenomas and lipomas can occur but thyroid cancer is not involved.

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