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Mary B. Daly, Robert Pilarski, Michael Berry, Saundra S. Buys, Meagan Farmer, Susan Friedman, Judy E. Garber, Noah D. Kauff, Seema Khan, Catherine Klein, Wendy Kohlmann, Allison Kurian, Jennifer K. Litton, Lisa Madlensky, Sofia D. Merajver, Kenneth Offit, Tuya Pal, Gwen Reiser, Kristen Mahoney Shannon, Elizabeth Swisher, Shaveta Vinayak, Nicoleta C. Voian, Jeffrey N. Weitzel, Myra J. Wick, Georgia L. Wiesner, Mary Dwyer, and Susan Darlow

The NCCN Clinical Practice Guidelines in Oncology for Genetic/Familial High-Risk Assessment: Breast and Ovarian provide recommendations for genetic testing and counseling for hereditary cancer syndromes and risk management recommendations for patients who are diagnosed with a syndrome. Guidelines focus on syndromes associated with an increased risk of breast and/or ovarian cancer. The NCCN Genetic/Familial High-Risk Assessment: Breast and Ovarian panel meets at least annually to review comments from reviewers within their institutions, examine relevant new data from publications and abstracts, and reevaluate and update their recommendations. The NCCN Guidelines Insights summarize the panel's discussion and most recent recommendations regarding risk management for carriers of moderately penetrant genetic mutations associated with breast and/or ovarian cancer.

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Mary B. Daly, Robert Pilarski, Jennifer E. Axilbund, Michael Berry, Saundra S. Buys, Beth Crawford, Meagan Farmer, Susan Friedman, Judy E. Garber, Seema Khan, Catherine Klein, Wendy Kohlmann, Allison Kurian, Jennifer K. Litton, Lisa Madlensky, P. Kelly Marcom, Sofia D. Merajver, Kenneth Offit, Tuya Pal, Huma Rana, Gwen Reiser, Mark E. Robson, Kristen Mahoney Shannon, Elizabeth Swisher, Nicoleta C. Voian, Jeffrey N. Weitzel, Alison Whelan, Myra J. Wick, Georgia L. Wiesner, Mary Dwyer, Rashmi Kumar, and Susan Darlow

The NCCN Guidelines for Genetic/Familial High-Risk Assessment: Breast and Ovarian provide recommendations for genetic testing and counseling and risk assessment and management for hereditary cancer syndromes. Guidelines focus on syndromes associated with an increased risk of breast and/or ovarian cancer and are intended to assist with clinical and shared decision-making. These NCCN Guidelines Insights summarize major discussion points of the 2015 NCCN Genetic/Familial High-Risk Assessment: Breast and Ovarian panel meeting. Major discussion topics this year included multigene testing, risk management recommendations for less common genetic mutations, and salpingectomy for ovarian cancer risk reduction. The panel also discussed revisions to genetic testing criteria that take into account ovarian cancer histology and personal history of pancreatic cancer.

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NCCN Guidelines Insights: Hepatobiliary Cancers, Version 2.2019

Featured Updates to the NCCN Guidelines

Al B. Benson III, Michael I. D’Angelica, Daniel E. Abbott, Thomas A. Abrams, Steven R. Alberts, Daniel A. Anaya, Robert Anders, Chandrakanth Are, Daniel Brown, Daniel T. Chang, Jordan Cloyd, Anne M. Covey, William Hawkins, Renuka Iyer, Rojymon Jacob, Andreas Karachristos, R. Kate Kelley, Robin Kim, Manisha Palta, James O. Park, Vaibhav Sahai, Tracey Schefter, Jason K. Sicklick, Gagandeep Singh, Davendra Sohal, Stacey Stein, G. Gary Tian, Jean-Nicolas Vauthey, Alan P. Venook, Lydia J. Hammond, and Susan D. Darlow

The NCCN Guidelines for Hepatobiliary Cancers provide treatment recommendations for cancers of the liver, gallbladder, and bile ducts. The NCCN Hepatobiliary Cancers Panel meets at least annually to review comments from reviewers within their institutions, examine relevant new data from publications and abstracts, and reevaluate and update their recommendations. These NCCN Guidelines Insights summarize the panel’s discussion and updated recommendations regarding systemic therapy for first-line and subsequent-line treatment of patients with hepatocellular carcinoma.

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Michelle B. Riba, Kristine A. Donovan, Barbara Andersen, IIana Braun, William S. Breitbart, Benjamin W. Brewer, Luke O. Buchmann, Matthew M. Clark, Molly Collins, Cheyenne Corbett, Stewart Fleishman, Sofia Garcia, Donna B. Greenberg, Rev. George F. Handzo, Laura Hoofring, Chao-Hui Huang, Robin Lally, Sara Martin, Lisa McGuffey, William Mitchell, Laura J. Morrison, Megan Pailler, Oxana Palesh, Francine Parnes, Janice P. Pazar, Laurel Ralston, Jaroslava Salman, Moreen M. Shannon-Dudley, Alan D. Valentine, Nicole R. McMillian, and Susan D. Darlow

Distress is defined in the NCCN Guidelines for Distress Management as a multifactorial, unpleasant experience of a psychologic (ie, cognitive, behavioral, emotional), social, spiritual, and/or physical nature that may interfere with the ability to cope effectively with cancer, its physical symptoms, and its treatment. Early evaluation and screening for distress leads to early and timely management of psychologic distress, which in turn improves medical management. The panel for the Distress Management Guidelines recently added a new principles section including guidance on implementation of standards of psychosocial care for patients with cancer.

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Ronald S. Go, Eric Jacobsen, Robert Baiocchi, Ilia Buhtoiarov, Erin B. Butler, Patrick K. Campbell, Don W. Coulter, Eli Diamond, Aron Flagg, Aaron M. Goodman, Gaurav Goyal, Dita Gratzinger, Paul C. Hendrie, Meghan Higman, Michael D. Hogarty, Filip Janku, Reem Karmali, David Morgan, Anne C. Raldow, Alexandra Stefanovic, Srinivas K. Tantravahi, Kelly Walkovich, Ling Zhang, Mary Anne Bergman, and Susan D. Darlow

Histiocytic neoplasms are rare hematologic disorders accounting for less than 1% of cancers of the soft tissue and lymph nodes. Clinical presentation and prognosis of these disorders can be highly variable, leading to challenges for diagnosis and optimal management of these patients. Treatment often consists of systemic therapy, and recent studies support use of targeted therapies for patients with these disorders. Observation (“watch and wait”) may be sufficient for select patients with mild disease. These NCCN Guidelines for Histiocytic Neoplasms include recommendations for diagnosis and treatment of adults with the most common histiocytic disorders: Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease.

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Mary B. Daly, Tuya Pal, Michael P. Berry, Saundra S. Buys, Patricia Dickson, Susan M. Domchek, Ahmed Elkhanany, Susan Friedman, Michael Goggins, Mollie L. Hutton, CGC, Beth Y. Karlan, Seema Khan, Catherine Klein, Wendy Kohlmann, CGC, Allison W. Kurian, Christine Laronga, Jennifer K. Litton, Julie S. Mak, LCGC, Carolyn S. Menendez, Sofia D. Merajver, Barbara S. Norquist, Kenneth Offit, Holly J. Pederson, Gwen Reiser, CGC, Leigha Senter-Jamieson, CGC, Kristen Mahoney Shannon, Rebecca Shatsky, Kala Visvanathan, Jeffrey N. Weitzel, Myra J. Wick, Kari B. Wisinski, Matthew B. Yurgelun, Susan D. Darlow, and Mary A. Dwyer

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.

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Mary B. Daly, Robert Pilarski, Matthew B. Yurgelun, Michael P. Berry, Saundra S. Buys, Patricia Dickson, Susan M. Domchek, Ahmed Elkhanany, Susan Friedman, Judy E. Garber, Michael Goggins, Mollie L. Hutton, Seema Khan, Catherine Klein, Wendy Kohlmann, Allison W. Kurian, Christine Laronga, Jennifer K. Litton, Julie S. Mak, Carolyn S. Menendez, Sofia D. Merajver, Barbara S. Norquist, Kenneth Offit, Tuya Pal, Holly J. Pederson, Gwen Reiser, Kristen Mahoney Shannon, Kala Visvanathan, Jeffrey N. Weitzel, Myra J. Wick, Kari B. Wisinski, Mary A. Dwyer, and Susan D. Darlow

The NCCN Guidelines for Genetic/Familial High-Risk Assessment: Breast, Ovarian, and Pancreatic provide recommendations for genetic testing and counseling for hereditary cancer syndromes, and risk management recommendations for patients who are diagnosed with syndromes associated with an increased risk of these cancers. The NCCN panel meets at least annually to review comments, examine relevant new data, and reevaluate and update recommendations. These NCCN Guidelines Insights summarize the panel’s discussion and most recent recommendations regarding criteria for high-penetrance genes associated with breast and ovarian cancer beyond BRCA1/2, pancreas screening and genes associated with pancreatic cancer, genetic testing for the purpose of systemic therapy decision-making, and testing for people with Ashkenazi Jewish ancestry.

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Margaret A. Tempero, Mokenge P. Malafa, Mahmoud Al-Hawary, Horacio Asbun, Andrew Bain, Stephen W. Behrman, Al B. Benson III, Ellen Binder, Dana B. Cardin, Charles Cha, E. Gabriela Chiorean, Vincent Chung, Brian Czito, Mary Dillhoff, Efrat Dotan, Cristina R. Ferrone, Jeffrey Hardacre, William G. Hawkins, Joseph Herman, Andrew H. Ko, Srinadh Komanduri, Albert Koong, Noelle LoConte, Andrew M. Lowy, Cassadie Moravek, Eric K. Nakakura, Eileen M. O'Reilly, Jorge Obando, Sushanth Reddy, Courtney Scaife, Sarah Thayer, Colin D. Weekes, Robert A. Wolff, Brian M. Wolpin, Jennifer Burns, and Susan Darlow

Ductal adenocarcinoma and its variants account for most pancreatic malignancies. High-quality multiphase imaging can help to preoperatively distinguish between patients eligible for resection with curative intent and those with unresectable disease. Systemic therapy is used in the neoadjuvant or adjuvant pancreatic cancer setting, as well as in the management of locally advanced unresectable and metastatic disease. Clinical trials are critical for making progress in treatment of pancreatic cancer. The NCCN Guidelines for Pancreatic Adenocarcinoma focus on diagnosis and treatment with systemic therapy, radiation therapy, and surgical resection.

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Al B. Benson III, Michael I. D'Angelica, Daniel E. Abbott, Thomas A. Abrams, Steven R. Alberts, Daniel A. Anaya, Chandrakanth Are, Daniel B. Brown, Daniel T. Chang, Anne M. Covey, William Hawkins, Renuka Iyer, Rojymon Jacob, Andrea Karachristos, R. Kate Kelley, Robin Kim, Manisha Palta, James O. Park, Vaibhav Sahai, Tracey Schefter, Carl Schmidt, Jason K. Sicklick, Gagandeep Singh, Davendra Sohal, Stacey Stein, G. Gary Tian, Jean-Nicolas Vauthey, Alan P. Venook, Andrew X. Zhu, Karin G. Hoffmann, and Susan Darlow

The NCCN Guidelines for Hepatobiliary Cancers provide treatment recommendations for cancers of the liver, gallbladder, and bile ducts. The NCCN Hepatobiliary Cancers Panel meets at least annually to review comments from reviewers within their institutions, examine relevant new data from publications and abstracts, and reevaluate and update their recommendations. These NCCN Guidelines Insights summarize the panel's discussion and most recent recommendations regarding locoregional therapy for treatment of patients with hepatocellular carcinoma.

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Jennifer M. Weiss, Samir Gupta, Carol A. Burke, Lisen Axell, Lee-May Chen, Daniel C. Chung, Katherine M. Clayback, Susan Dallas, Seth Felder, Olumide Gbolahan, Francis M. Giardiello, William Grady, Michael J. Hall, Heather Hampel, Rachel Hodan, Gregory Idos, Priyanka Kanth, Bryson Katona, Laura Lamps, Xavier Llor, Patrick M. Lynch, Arnold J. Markowitz, Sara Pirzadeh-Miller, Niloy Jewel Samadder, David Shibata, Benjamin J. Swanson, Brittany M. Szymaniak, Georgia L. Wiesner, Andrew Wolf, Matthew B. Yurgelun, Mae Zakhour, Susan D. Darlow, Mary A. Dwyer, and Mallory Campbell

Identifying individuals with hereditary syndromes allows for timely cancer surveillance, opportunities for risk reduction, and syndrome-specific management. Establishing criteria for hereditary cancer risk assessment allows for the identification of individuals who are carriers of pathogenic genetic variants. The NCCN Guidelines for Genetic/Familial High-Risk Assessment: Colorectal provides recommendations for the assessment and management of patients at risk for or diagnosed with high-risk colorectal cancer syndromes. The NCCN Genetic/Familial High-Risk Assessment: Colorectal panel meets annually to evaluate and update their recommendations based on their clinical expertise and new scientific data. These NCCN Guidelines Insights focus on familial adenomatous polyposis (FAP)/attenuated familial adenomatous polyposis (AFAP) syndrome and considerations for management of duodenal neoplasia.