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Titolo:
Multiple endocrine neoplasias
Autore:
Phay, JE; Moley, JF; Lairmore, TC;
Indirizzi:
Washington Univ, Sch Med, Dept Surg, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 , Dept Surg, St Louis, MO 63110 USA
Titolo Testata:
SEMINARS IN SURGICAL ONCOLOGY
fascicolo: 4, volume: 18, anno: 2000,
pagine: 324 - 332
SICI:
8756-0437(200006)18:4<324:MEN>2.0.ZU;2-B
Fonte:
ISI
Lingua:
ENG
Soggetto:
MEDULLARY-THYROID CARCINOMA; ZOLLINGER-ELLISON SYNDROME; TYROSINE KINASE DOMAIN; MICE LACKING GDNF; RET PROTOONCOGENE; LONG-TERM; HIRSCHSPRUNGS-DISEASE; TRANSFORMING GENE; MEN 2A; MUTATIONS;
Keywords:
multiple endocrine neoplasia type 1; multiple endocrine neoplasia type 2A; multiple endocrine neoplasia type 2B; medullary thyroid carcinoma; differential diagnosis; neoplasm proteins; thyroid neoplasms; hyperparathyroidism; pancreatic neoplasms; duodenal neoplasms; neuroendocrine neoplasms; pituitary neoplasms; proto-oncogenes; thyroid neoplasms; thyroidectomy; pheochromocytoma; proto-oncogene proteins; frameshift mutation; nonsense codon; missense mutation; RNA splicing; germ-line mutations; tumor suppressor genes; parathyroid glands/surgery/transplantation; gastrinoma; insulinoma; Hirschsprung's disease; calcitonin; genetic screening;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
47
Recensione:
Indirizzi per estratti:
Indirizzo: Moley, JF Washington Univ, Sch Med, Dept Surg, 660 S Euclid Ave,Box 8109, St Louis, MO 63110 USA Washington Univ 660 S Euclid Ave,Box 8109 St Louis MO USA 63110
Citazione:
J.E. Phay et al., "Multiple endocrine neoplasias", SEM SURG ON, 18(4), 2000, pp. 324-332

Abstract

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 theprincipal 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. Semin. Surg. Oncol. 18:324-332, 2000, (C) 2000 Wiley-Liss, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/09/20 alle ore 08:30:36