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Hereditary ovarian cancer.

AbstractAt least 10% of ovarian tumors are hereditary and associated with highly penetrant, autosomal, dominant genetic predisposition. Three clinical manifestations of hereditary ovarian cancer have been identified: site-specific ovarian cancer, hereditary breast and/or ovarian cancer (HBOC) and hereditary non-polyposis colorectal cancer (HNPCC) syndromes. BRCA germline mutations account for more than 90% of all hereditary epithelial ovarian tumors whereas most of the remaining 10% are caused by MLH1 and MSH2 mutations, which are susceptibility genes of HNPCC. Genetic testing is available for each of the three hereditary syndromes above mentioned. The recommendations for OC surveillance in high-risk women having a strong family history or BRCA mutation carriers include transvaginal pelvic ultrasound with color Doppler and serum CA125 every 6 months. Bilateral salpingo-oophorectomy appears to be effective to reduce the risk of ovarian cancer in BRCA mutation carriers. Hysterosalpingo-oophorectomy should be considered in HNPCC women who undergo surgery for colorectal carcinoma.
AuthorsAntonio Russo, Valentina Calò, Loredana Bruno, Sergio Rizzo, Viviana Bazan, Gaetana Di Fede (Affiliation: Department of Surgery and Oncology, Regional Reference Center for the Biomolecular Characterization and Genetic Screening of Hereditary Tumors, Università di Palermo, Palermo, Italy. lab-oncobiologia at usa.net)
JournalCritical reviews in oncology/hematology (Crit Rev Oncol Hematol) Vol. 69 Issue 1 Pg. 28-44 (Jan 2009) ISSN: 1879-0461 Ireland
PMID18656380 (Publication Type: Journal Article, Review)
Topics
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • Neoplastic Syndromes, Hereditary (diagnosis, genetics, prevention & control, therapy)
  • Ovarian Neoplasms (diagnosis, genetics, prevention & control)
  • Prognosis