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Introduction Twenty-five years after the seminal work linking oncogenic RET and multiple endocrine neoplasia type 2 (MEN2), the field has advanced well beyond what Donis-Keller and coworkers ( 1993 ) imagined. We now have a deeper

Introduction Multiple endocrine neoplasia type 2 (MEN2) refers collectively to 2 main distinct types of autosomal dominantly inherited neuroendocrine tumour (NET) syndromes ( Barakat et al. 2004 ); MEN type 2A and MEN type 2B (also known as

P Grussendorf M Raue F Ritter MM Hoppner W 1998 A new hot spot for mutations in the ret protooncogene causing familial medullary thyroid carcinoma and multiple endocrine neoplasia type 2A . Journal of Clinical Endocrinology and Metabolism 83

seminal description ( Sipple 1961 ). It is now commonly referred to as multiple endocrine neoplasia type 2 or MEN2, especially when complemented by primary hyperparathyroidism. This achievement was brought about by a combination of astute clinical

(about 75% of cases) or familial (about 25% of cases) form. Hereditary MTC occurs in the context of different autosomal dominant syndromes, including multiple endocrine neoplasia type 2 (MEN2). MTC can be the only manifestation in familial MTC (FMTC), can

entity or in the context of several hereditary syndromes, such as von Hippel–Lindau syndrome (VHL), multiple endocrine neoplasia type 2 (MEN2), neurofibromatosis type 1 (NF1), and familial paragangliomas. The genes associated with these inherited

Introduction Multiple endocrine neoplasia type 2 (MEN 2) is an autosomal dominant multiglandular cancer syndrome, affecting tissues of neural crest origin. It is caused by germ line mutations of the RET proto-oncogene, encoding a transmembrane

The association of medullary thyroid carcinoma (MTC) and pheochromocytoma (PHEO) that we now recognize as multiple endocrine neoplasia type 2 (MEN2) ( Fig. 1 ) was first reported by John Sipple in 1961 ( Sipple 1961 ), but over 30 years passed

Overview The multiple endocrine neoplasia syndromes are a varied group of disorders that include multiple endocrine neoplasia types 1 and 2 (MEN 1, MEN 2), von Hippel-Lindau (VHL) disease , Carney complex (CC), hereditary pheochromocytoma

, multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2A (MEN2A), and the hyperparathyroidism-jaw tumor syndrome (HPT-JT) are familial syndromes of hyperparathyroidism that are associated with a spectrum of endocrine tumors and other