TY - CHAP M1 - Book, Section TI - Multiple Endocrine Neoplasia Type 2 (MEN 2) A1 - Doherty, Gerard M. PY - 2010 T2 - Quick Answers Surgery AB - • Family of syndromes including: -MEN 2A (Sipple syndrome)-MEN 2B-Familial medullary thyroid carcinoma (FMTC)• MEN 2A is characterized by: -Medullary thyroid carcinoma (MTC)-Pheochromocytomas-Parathyroid hyperplasia• MEN 2B consist of MTC, pheochromocytoma, mucosal neuromas, gangliomatosis of the GI tract, and a distinctive marfinoid habitus-Also have high incidence of skeletal abnormalities, such as congenital dislocation of the hip, pes planus or cavus, kyphosis, and pectus excavatum• Transmitted as mendelian autosomal trait, but can occur de novo (especially MEN 2B)• Common mutation is in the Ret proto-oncogone (tyrosine kinase), which maps to the centromeric region of chromosome 10• Families with hereditary MTC need to be identified with an aggressive screening program because early diagnosis and thyroidectomy renders MTC curable in a large percentage of patients SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/11/07 UR - accesssurgery.mhmedical.com/content.aspx?aid=58095296 ER -