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Jeffrey B. Boord, Division of Cardiovascular Medicine, Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee, USA,
Lewis S. Blevins, Division of Diabetes, Endocrinology and Metabolism, Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee, USA
E. Steve Roach, Wake Forest University, North Carolina,Van S. Miller, University of Texas Southwestern Medical Center, Dallas
Multiple endocrine neoplasia Type 2 (MEN-2) is a distinct autosomal dominant inherited tumor syndrome with three recognized clinical subtypes and a common genetic origin from mutation of the RET proto-oncogene. The syndrome was originally identified by Sipple (1961), who reported an association of pheochromocytoma with medullary thyroid carcinoma (MTC). MEN-2A was originally known as Sipple's syndrome – the triad of MTC, pheochromocytoma, and hyperparathyroidism. Wagenmann (1922) and Froboese (1923) initially described the clinical syndrome of mucosal neuromas, and Williams and Pollack (1966) further delineated the MEN-2B phenotype with the description of a syndrome of mucosal neuromas, intestinal ganglioneuromatosis, pheochromocytoma, and MTC.
A great deal has been learned regarding the origins, pathogenesis, and clinical manifestations of the disease since its original descriptions. The isolation of the RET proto-oncogene has revolutionized screening for the disease in affected kindreds as well as the approach to management of the clinical manifestations. The clinical spectrum of MEN-2 is currently defined in the Operational Classification of MEN-2 Disease Phenotype by the International RET Mutation Consortium, summarized in Table 11.1 (Eng et al., 1996). MEN-2A is by far the most common variant, accounting for over 90% of cases of MEN-2. MEN-2B comprises about 5% of cases, and Familial MTC (FMTC) accounts for the remainder (Eng, 1999). Virtually all patients with MEN-2 develop C-cell hyperplasia and/or MTC, and MTC is responsible for a majority of the mortality associated with MEN-2. MEN-2A is also associated with pheochromocytoma in 50% and hyperparathyroidism in 20–30% of patients.
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