Genetic/Familial High-Risk Assessment: Breast, Ovarian, And Pancreatic, version 2.2021, NCCN Clinical Practice Guidelines in Oncology Guidelines


Authors: Daly, M. B.; Pal, T.; Berry, M. P.; Buys, S. S.; Dickson, P.; Domchek, S. M.; Elkhanany, A.; Friedman, S.; Goggins, M.; Hutton, M. L.; Karlan, B. Y.; Khan, S.; Klein, C.; Kohlmann, W.; Kurian, A. W.; Laronga, C.; Litton, J. K.; Mak, J. S.; Menendez, C. S.; Merajver, S. D.; Norquist, B. S.; Offit, K.; Pederson, H. J.; Reiser, G.; Senter-Jamieson, L.; Shannon, K. M.; Shatsky, R.; Visvanathan, K.; Weitzel, J. N.; Wick, M. J.; Wisinski, K. B.; Yurgelun, M. B.; Darlow, S. D.; Dwyer, M. A.
Title: Genetic/Familial High-Risk Assessment: Breast, Ovarian, And Pancreatic, version 2.2021, NCCN Clinical Practice Guidelines in Oncology
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. © 2021 Harborside Press. All rights reserved.
Journal Title: Journal of the National Comprehensive Cancer Network
Volume: 19
Issue: 1
ISSN: 1540-1405
Publisher: Harborside Press  
Date Published: 2021-01-01
Start Page: 77
End Page: 102
Language: English
DOI: 10.6004/jnccn.2021.0001
PROVIDER: scopus
PUBMED: 33406487
DOI/URL:
Notes: Review -- Export Date: 1 February 2021 -- Source: Scopus
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  1. Kenneth Offit
    765 Offit