Localization and identification of the multiple endocrine neoplasia type 1 disease gene.

The familial and genetic nature of multiple endocrine neoplasia type 1 (MEN 1) syndrome was first pointed out by Wermer in 1954, who suggested that an autosomal dominant gene with high penetrance controls the trait. The clinical picture is variable, depending mainly on which glands are involved and whether the tumors hypersecrete symptom-causing hormones. The most frequent endocrinopathies are hyperparathyroidism, pancreatic-duodenal, and pituitary tumors. Other tumors are also seen more frequently than in the general population, e.g., adrenocortical and thyroid tumors, carcinoids, lipomas, and pinealomas.