NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Hepatobiliary Cancers Version 1.2018 — February 14, 2018 NCCN.org Continue Version 1.2018 02/14/18 © National Comprehensive Cancer Network, Inc. 2018, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. Printed by Anton Kabakov on 3/5/2018 6:53:14 AM. For personal use only. Not approved for distribution. Copyright © 2018 National Comprehensive Cancer Network, Inc., All Rights Reserved. NCCN Guidelines Index NCCN Guidelines Version 1.2018 Panel Members Table of Contents Hepatobiliary Cancers Discussion *Al B. Benson, III, MD/Chair † Rojymon Jacob, MD § Gagandeep Singh, MD ¶ Robert H. Lurie Comprehensive Cancer University of Alabama at Birmingham City of Hope Comprehensive Center of Northwestern University Comprehensive Cancer Center Cancer Center *Michael I. D’Angelica, MD/Vice-Chair ¶ Andrea Karachristos, MD ¶ Davendra Sohal, MD, MPH † Memorial Sloan Kettering Cancer Center Fox Chase Cancer Center Case Comprehensive Cancer Center/ University Hospitals Seidman Cancer Daniel Abbott, MD ¶ R. Kate Kelley, MD † ‡ Center and Cleveland Clinic Taussig University of Wisconsin UCSF Helen Diller Family Cancer Institute Carbone Cancer Center Comprehensive Cancer Center Stacey Stein, MD Thomas A. Abrams, MD † Robin Kim, MD ξ Yale Cancer Center/Smilow Cancer Hospital Dana-Farber/Brigham and Women’s Huntsman Cancer Institute Cancer Center at the University of Utah G. Gary Tian, MD, PhD † St. Jude Children’s Steven R. Alberts, MD, MPH † Manisha Palta, MD § Research Hospital/ Mayo Clinic Cancer Center Duke Cancer Institute The University of Tennessee Health Science Center Daniel A. Anaya, MD ¶ James O. Park, MD ¶ Moffitt Cancer Center Fred Hutchinson Cancer Research Center/ Jean-Nicolas Vauthey, MD ¶ Seattle Cancer Care Alliance The University of Texas Chandrakanth Are, MD ¶ MD Anderson Cancer Center Fred & Pamela Buffett Cancer Center Vaibhav Sahai, MD, MS † University of Michigan Alan P. Venook, MD † ‡ Þ *Daniel Brown, MD † § ComprehensiveCancer Center UCSF Helen Diller Family Vanderbilt-Ingram Cancer Center Comprehensive Cancer Center Tracey Schefter, MD § Daniel T. Chang, MD § University of Colorado Cancer Center Andrew X. Zhu, MD, PhD † Stanford Cancer Institute Massachusetts General Hospital Carl Schmidt, MD ¶ Cancer Center *Anne M. Covey, MD § The Ohio State University Comprehensive NCCN Memorial Sloan Kettering Cancer Center Cancer Center - James Cancer Hospital Susan Darlow, PhD and Solove Research Institute Karin G. Hoffmann, RN, CCM William Hawkins, MD ¶ Siteman Cancer Center at Barnes- Jason K. Sicklick, MD ¶ UC San Diego Moores Cancer Center † Medical oncology Jewish Hospital and Washington § Radiotherapy/Radiation oncology/Interventional radiology University School of Medicine ¶ Surgery/Surgical oncology Þ Internal medicine Renuka Iyer, MD Þ † Continue ‡ Hematology/Hematology oncology Roswell Park Cancer Institute *Discussion section writing committee ξ Bone marrow transplantation ф Diagnostic radiology NCCN Guidelines Panel Disclosures Version 1.2018 02/14/18 © National Comprehensive Cancer Network, Inc. 2018, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. Printed by Anton Kabakov on 3/5/2018 6:53:14 AM. For personal use only. Not approved for distribution. Copyright © 2018 National Comprehensive Cancer Network, Inc., All Rights Reserved. NCCN Guidelines Index NCCN Guidelines Version 1.2018 Table of Contents Table of Contents Hepatobiliary Cancers Discussion NCCN Hepatobiliary Cancers Panel Members Clinical Trials: NCCN believes Summary of the Guidelines Updates that the best management for any Hepatocellular Carcinoma (HCC) patient with cancer is in a clinical • HCC Screening (HCC-1) trial. Participation in clinical trials is • Diagnosis of HCC (HCC-2) especially encouraged. • HCC Confirmed (HCC-3) To find clinical trials online at NCCN • Potentially Resectable or Transplantable, Operable (HCC-4) Member Institutions, click here: • Unresectable (HCC-5) nccn.org/clinical trials/physician.html. • Inoperable, Local Disease, Metastatic Disease, Extensive Liver Tumor Burden (HCC-6) • Principles of Imaging (HCC-A) NCCN Categories of Evidence and All recommendations • Principles of Biopsy (HCC-B) Consensus: are category 2A unless otherwise • Child-Pugh Score (HCC-C) indicated. • Principles of Surgery (HCC-D) • Principles of Locoregional Therapy (HCC-E) See NCCN Categories of Evidence and Consensus. Gallbladder Cancer • Incidental Finding at Surgery (GALL-1) • Incidental Finding on Pathologic Review (GALL-2) • Mass on Imaging (GALL-3) • Jaundice (GALL-4) • Metastatic Disease (GALL-4) Extrahepatic Cholangiocarcinoma • Post-Resection (GALL-5) • Presentation, Workup, Primary Treatment (EXTRA-1) • Principles of Imaging (GALL-A) • Adjuvant Treatment, Surveillance (EXTRA-2) • Principles of Surgery and Pathology (GALL-B) • Principles of Imaging (EXTRA-A) • Principles of Radiation Therapy (GALL-C) • Principles of Surgery (EXTRA-B) Hepatobiliary Cancers Intrahepatic Cholangiocarcinoma Staging (ST-1) • Presentation, Workup, Primary Treatment (INTRA-1) • Adjuvant Treatment, Surveillance (INTRA-2) • Principles of Surgery (INTRA-A) The NCCN Guidelines® are a statement of evidence and consensus of the authors regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult the NCCN Guidelines is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patient’s care or treatment. The National Comprehensive Cancer Network® (NCCN®) makes no representations or warranties of any kind regarding their content, use or application and disclaims any responsibility for their application or use in any way. The NCCN Guidelines are copyrighted by National Comprehensive Cancer Network®. All rights reserved. The NCCN Guidelines and the illustrations herein may not be reproduced in any form without the express written permission of NCCN. ©2018. Version 1.2018 02/14/18 © National Comprehensive Cancer Network, Inc. 2018, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. Printed by Anton Kabakov on 3/5/2018 6:53:14 AM. For personal use only. Not approved for distribution. Copyright © 2018 National Comprehensive Cancer Network, Inc., All Rights Reserved. NCCN Guidelines Index NCCN Guidelines Version 1.2018 Updates Table of Contents Hepatobiliary Cancers Discussion Updates in Version 1.2018 of the NCCN Guidelines for Hepatobiliary Cancers from Version 4.2017 include: Hepatocellular Carcinoma HCC-1 • Patients a risk for HCC 5th sub-bullet was revised: "Stage 4 primary biliary cirrhosis cholangitis" Footnote "f" was added: "Beuers U, Gershwin M, Gish R, et al. Changing Nomenclature for PBC: From 'Cirrhosis' to ‘Cholangitis' Am J Gastroenterol 2015;110(11):1536–1538." • Footnote "j" about alpha fetoprotein (AFP) was revised: "AFP is considered optional for surveillance screening. (See Principles of Imaging, HCC-A)." • AFP Positive or US Nodule(s) ≥10 mm "Abdominal multiphasic CT or MRI" statement was removed. A footnote was removed: "Multiphase CT: Pre-contrast is suggested. Late hepatic arterial phase (AP), portal venous phase (PVP), and delayed phase (DP) are required." (Also for HCC-2) A footnote was removed: "Multiphase MRI: Pre-contrast. Late hepatic AP, PVP, and DP are required." (Also for HCC-2) HCC-2 • Additional Workup A footnote linking to the Principles of Biopsy section was added to this statement: "Individualized workup, which may include additional imaging or biopsy, as informed by multidisciplinary discussion" HCC-4 • Treatment 3rd bullet was revised: "External-beam radiation therapy (EBRT) (category 2B) Radiation therapy" ( Also for HCC-5 and HCC-6) Footnote "bb" was revised: "Case series and single-arm studies demonstrate suggest safety and possible efficacy of radiation therapy in selected cases. See Principles of Locoregional Therapy (HCC-E)." (Also for HCC-5 and HCC-6) • Surveillance Footnote "cc" was revised: "Multiphasic abdominal/pelvic MRI or multi-phase CT scans for liver assessment are recommended. Consider chest CT. See Principles of Imaging (HCC-A)." (Also for HCC-5) HCC-5 • Surveillance Regorafenib (Child-Pugh Class A only) (Category 1) ◊ Footnote"jj" was added: "Regorafenib for patients with hepatocellular carcinoma who progressed on sorafenib treatment (RESORCE): a randomized, double-blind, placebo-controlled, phase 3 trial. Bruix J, Qin S, Merle P, et al. Lancet 2017;389:56-66." (Also for HCC-6) Continued Version 1.2018 02/14/18 © National Comprehensive Cancer Network, Inc. 2018, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. UPDATES Printed by Anton Kabakov on 3/5/2018 6:53:14 AM. For personal use only. Not approved for distribution. Copyright © 2018 National Comprehensive Cancer Network, Inc., All Rights Reserved. NCCN Guidelines Index NCCN Guidelines Version 1.2018 Updates Table of Contents Hepatobiliary Cancers Discussion HCC-A (1 of 3) Principles of Imaging • Imaging diagnosis of HCC 1st bullet, 5th sentence was revised: "Major imaging features of HCC include arterial phase hyperenhancement, venous or delayed phase washout appearance and capsule appearance, and threshold growth.