| Autor: |
Daly MB; 1Fox Chase Cancer Center., Pal T; 2Vanderbilt-Ingram Cancer Center., Berry MP; 3St. Jude Children's Research Hospital/The University of Tennessee Health Science Center., Buys SS; 4Huntsman Cancer Institute at the University of Utah., Dickson P; 5Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine., Domchek SM; 6Abramson Cancer Center at the University of Pennsylvania., Elkhanany A; 7O'Neal Comprehensive Cancer Center at UAB., Friedman S; 8FORCE: Facing Our Risk of Cancer Empowered., Goggins M; 9The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins., Hutton ML, Karlan BY; 11UCLA Jonsson Comprehensive Cancer Center., Khan S; 12Robert H. Lurie Comprehensive Cancer Center of Northwestern University., Klein C; 13University of Colorado Cancer Center., Kohlmann W, Kurian AW; 14Stanford Cancer Institute., Laronga C; 15Moffitt Cancer Center., Litton JK; 16The University of Texas MD Anderson Cancer Center., Mak JS, Menendez CS; 18Duke Cancer Institute., Merajver SD; 19University of Michigan Rogel Cancer Center., Norquist BS; 20Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance., Offit K; 21Memorial Sloan Kettering Cancer Center., Pederson HJ; 22Case Comprehensive Cancer Center/University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute., Reiser G, Senter-Jamieson L, Shannon KM; 25Massachusetts General Hospital Cancer Center., Shatsky R; 26UC San Diego Moores Cancer Center., Visvanathan K; 9The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins., Weitzel JN; 27City of Hope National Medical Center., Wick MJ; 28Mayo Clinic Cancer Center., Wisinski KB; 29University of Wisconsin Carbone Cancer Center., Yurgelun MB; 30Dana-Farber/Brigham and Women's Cancer Center; and., Darlow SD; 31National Comprehensive Cancer Network., Dwyer MA; 31National Comprehensive Cancer Network. |
| Abstrakt: |
The NCCN Guidelines for Genetic/Familial High-Risk Assessment: Breast, Ovarian, and Pancreatic focus primarily on assessment of pathogenic or likely pathogenic variants associated with increased risk of breast, ovarian, and pancreatic cancer and recommended approaches to genetic testing/counseling and management strategies in individuals with these pathogenic or likely pathogenic variants. This manuscript focuses on cancer risk and risk management for BRCA-related breast/ovarian cancer syndrome and Li-Fraumeni syndrome. Carriers of a BRCA1/2 pathogenic or likely pathogenic variant have an excessive risk for both breast and ovarian cancer that warrants consideration of more intensive screening and preventive strategies. There is also evidence that risks of prostate cancer and pancreatic cancer are elevated in these carriers. Li-Fraumeni syndrome is a highly penetrant cancer syndrome associated with a high lifetime risk for cancer, including soft tissue sarcomas, osteosarcomas, premenopausal breast cancer, colon cancer, gastric cancer, adrenocortical carcinoma, and brain tumors. |
