Multiple endocrine neoplasias

The multiple endocrine neoplasia syndromes form a distinct group of genetic tumor syndromes. They include multiple endocrine neoplasia types 1 and 2, von Hippel Lindau syndrome, neurofibromatosis, and Carney complex. Research over the past decade has identified a molecular basis for each of these sy ndromes. This knowledge has revolutionized not only the clinical management but also has illuminated the field of human cancer research by the identif ication of new and important genes critical for regulation of cell growth, differentiation, and death. This review focuses on the structure, physiolog ic function, and molecular abnormalities of the genes involved in these syn dromes.