Multiple Endocrine Neoplasia and Other Familial Endocrine Tumor Syndromes

The multiple endocrine neoplasia (MEN) syndromes are rare and fascinating autosomal dominant inherited conditions, each characterized by a cluster of endocrine neoplasms. The first recognizable description ofMEN1, byErdheim (1) in 1903, was of a patientwith acromegaly and four enlarged parathyroid glands. In 1954, Wermer (2) described the syndrome of familial pituitary, parathyroid, and pancreatic islet cell neoplasia and coined the term ‘‘adenomatosis’’ of the endocrine glands. Wermer’s syndrome is synonymous with MEN1.