PMID- 10805954 OWN - NLM STAT- MEDLINE DCOM- 20000621 LR - 20211203 IS - 8756-0437 (Print) IS - 1098-2388 (Linking) VI - 18 IP - 4 DP - 2000 Jun TI - Multiple endocrine neoplasias. PG - 324-32 AB - Multiple endocrine neoplasia type 1 (MEN 1), and the multiple endocrine neoplasia type 2 syndromes (MEN 2A, MEN 2B, and familial non-MEN medullary thyroid carcinoma [FMTC]) encompass a wide range of endocrine problems, but arise from only two genes: the MEN 1 tumor suppressor gene and the RET proto-oncogene. MEN 1 is characterized by parathyroid hyperplasia, pancreaticoduodenal neuroendocrine tumors (PNTs), and pituitary adenomas. Surgery is the principal treatment modality for hyperparathyroidism and PNTs, but questions still remain concerning the timing and extent of surgery for PNTs. The MEN 2 syndromes are characterized by complete penetrance of medullary thyroid cancer. The MEN 2 syndromes differ in their variable expression of hyperparathyroidism, pheochromocytomas, and other clinical features. Genetic testing for mutations in the RET gene has revolutionized treatment by enabling thyroidectomies before significant disease occurs. CI - Copyright 2000 Wiley-Liss, Inc. FAU - Phay, J E AU - Phay JE AD - Department of Surgery, Washington University School of Medicine, St. Louis, Missouri, USA. FAU - Moley, J F AU - Moley JF FAU - Lairmore, T C AU - Lairmore TC LA - eng PT - Journal Article PT - Review PL - United States TA - Semin Surg Oncol JT - Seminars in surgical oncology JID - 8503713 SB - IM MH - Adenoma/*genetics/pathology/therapy MH - Carcinoma, Medullary/*genetics/pathology/therapy MH - Gene Expression Regulation, Neoplastic MH - Genes, Tumor Suppressor/genetics MH - Genetic Predisposition to Disease MH - Humans MH - Hyperparathyroidism/etiology MH - Multiple Endocrine Neoplasia/*genetics/pathology/therapy MH - Pheochromocytoma/etiology MH - Pituitary Neoplasms/*genetics/pathology/therapy MH - Proto-Oncogene Mas MH - Proto-Oncogenes/genetics MH - Thyroid Gland/surgery MH - Thyroid Neoplasms/*genetics/pathology/therapy RF - 47 EDAT- 2000/05/11 09:00 MHDA- 2000/06/24 11:00 CRDT- 2000/05/11 09:00 PHST- 2000/05/11 09:00 [pubmed] PHST- 2000/06/24 11:00 [medline] PHST- 2000/05/11 09:00 [entrez] AID - 10.1002/(SICI)1098-2388(200006)18:4<324::AID-SSU7>3.0.CO;2-5 [pii] AID - 10.1002/(sici)1098-2388(200006)18:4<324::aid-ssu7>3.0.co;2-5 [doi] PST - ppublish SO - Semin Surg Oncol. 2000 Jun;18(4):324-32. doi: 10.1002/(sici)1098-2388(200006)18:4<324::aid-ssu7>3.0.co;2-5.