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Version 2 2019 08 08 19 2019 National Comprehensive Cancer Network NCCN All rights reserved NCCN Guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN NCCN Clinical Practice Guidelines in Oncology NCCN Guidelines Genetic Familial High Risk Assessment Colorectal Version 2 2019 August 8 2019 Continue NCCN org NCCN Guidelines Version 2 2019 Genetic Familial High Risk Assessment Colorectal Version 2 2019 08 08 19 2019 National Comprehensive Cancer Network NCCN All rights reserved NCCN Guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN NCCN Guidelines Index Table of Contents Discussion NCCN Guidelines Panel Disclosures Continue Dawn Provenzale MD MS Chair Duke Cancer Institute Samir Gupta MD Vice chair UC San Diego Moores Cancer Center Dennis J Ahnen MD University of Colorado Cancer Center Lee May Chen MD UCSF Helen Diller Family Comprehensive Cancer Center Daniel C Chung MD Massachusetts General Hospital Cancer Center Gregory Cooper MD Case Comprehensive Cancer Center University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute Dayna S Early MD Siteman Cancer Center at Barnes Jewish Hospital and Washington University School of Medicine Francis M Giardiello MD MBA The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins William Grady MD Fred Hutchinson Cancer Research Center Seattle Cancer Care Alliance Michael J Hall MD MS Fox Chase Cancer Center Amy L Halverson MD Robert H Lurie Comprehensive Cancer Center of Northwestern University Stanley R Hamilton MD The University of Texas MD Anderson Cancer Center Heather Hampel MS CGC The Ohio State University Comprehensive Cancer Center James Cancer Hospital and Solove Research Institute Sigurdis Haraldsdottir MD PhD Stanford Cancer Institute Priyanka Kanth MD MS Huntsman Cancer Institute at the University of Utah Jason B Klapman MD Moffitt Cancer Center Audrey J Lazenby MD Fred 110 1059 1066 Footnote c was added Irrespective of degree of relatedness Also for LS 1 and POLYP 1 HRS 2 Qualifier 5 serrated polyps was revised by adding proximal to sigmoid colon After qualifier 10 adenomas Rare genetic causes of multiple adenomatous polyps was added with corresponding footnote i HRS 3 The Criteria for the Evaluation of Lynch Syndrome were reorganized by personal history family history and increased model predicted risk for Lynch syndrome Personal history 3rd sub bullet was revised by removing diagnosed 60 y Increased model predicted risk for Lynch syndrome sub bullet was revised An individual with a LS related cancer or unaffected individual with a 5 risk HRS A The first three pages of the Principles of Cancer Risk Assessment and Counseling are a new section in the guidelines UPDATES Continued HRS A 4 of 6 For family history of cancer and expanded pedigree 2nd bullet was changed from Minimal data set on each affected relative to Recommended data on each affected relative and two additional sub bullet were added Birth resulting from sperm or egg donor and History of allogeneic related or unrelated donor bone marrow transplant Lynch Syndrome LS 1 The algorithm and footnotes on this page were extensively revised LS 2 Lynch Syndrome Management Other Extracolonic Cancers Gastric and small bowel cancer recommendation was revised Also individuals of Asian descent or from countries with high background incidence of gastric cancer may have increased risk for stomach cancer and may benefit from surveillance Urothelial cancer recommendation was revised There is no clear evidence to support surveillance for urothelial cancers in LS Surveillance may be considered in selected individuals such as with a family history of urothelial cancer or individuals with MSH2 pathogenic variants especially males as these groups appear to be at higher risk may want to consider screening surveillance Footnotes Footnote o was added Patients who may benefit from a shorter 1 versus longer 2 year interval include those with risk factors such as history of CRC male sex MLH1 MSH2 pathogenic variant age 40 years and history of adenoma See Discussion Also for LS 5 Footnote q was revised from Patients with LS can consider ongoing clinical trials for pancreatic cancer screening to If screening is performed it should be considered in high volume centers with multidisciplinary teams preferably within research protocols The International Cancer of the Pancreas Screening CAPS consortium recommends that patients with LS with one first degree relative with pancreatic cancer should be considered for screening Updates in Version 2 2019 of the NCCN Guidelines for Genetic Familial High Risk Assessment Colorectal from Version 1 2019 include LS B 1 of 2 Cancer Risks in Lynch Syndrome by Gene Compared to the General Population table the breast cancer risk for MLH1 was changed from 12 25 to 12 17 Printed by Maria Chen on 9 15 2019 11 36 51 PM For personal use only 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