| Literature DB >> 33406487 |
Mary B Daly1, Tuya Pal2, Michael P Berry3, Saundra S Buys4, Patricia Dickson5, Susan M Domchek6, Ahmed Elkhanany7, Susan Friedman8, Michael Goggins9, Mollie L Hutton, Beth Y Karlan10, Seema Khan11, Catherine Klein12, Wendy Kohlmann, Allison W Kurian13, Christine Laronga14, Jennifer K Litton15, Julie S Mak, Carolyn S Menendez16, Sofia D Merajver17, Barbara S Norquist18, Kenneth Offit19, Holly J Pederson20, Gwen Reiser, Leigha Senter-Jamieson, Kristen Mahoney Shannon21, Rebecca Shatsky22, Kala Visvanathan9, Jeffrey N Weitzel23, Myra J Wick24, Kari B Wisinski25, Matthew B Yurgelun26, Susan D Darlow27, Mary A Dwyer27.
Abstract
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.Entities:
Mesh:
Year: 2021 PMID: 33406487 DOI: 10.6004/jnccn.2021.0001
Source DB: PubMed Journal: J Natl Compr Canc Netw ISSN: 1540-1405 Impact factor: 11.908