Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant genetic disorder characterized by the association of parathyroid, pituitary, and gastroenteropancreatic neuroendocrine tumors. Medullary thyroid carcinoma, on the other hand, is the major manifestation of MEN2. The coexistence of MEN1 and medullary thyroid carcinoma is exceptional and controversial. This review aims to analyze the available data and clarify the possible existence of a pathophysiological link.
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