NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Ovarian Cancer Including Fallopian Tube Cancer and Primary Peritoneal Cancer Version 4.2017 — November 9, 2017 NCCN.org NCCN Guidelines for Patients® available at www.nccn.org/patients Continue Version 4.2017, 11/09/17 © National Comprehensive Cancer Network, Inc. 2017, All rights reserved. The NCCN Guidelines®and this illustration may not be reproduced in any form without the express written permission of NCCN®. NCCN Guidelines Version 4.2017 Panel Members NCCN Guidelines Index Ovarian Cancer TOC Ovarian Cancer Discussion *Deborah K. Armstrong, MD/Chair Ω † Laura J. Havrilesky, MD Ω Matthew A. Powell, MD Ω The Sidney Kimmel Comprehensive Duke Cancer Institute Siteman Cancer Center at Barnes- Cancer Center at Johns Hopkins Ω Jewish Hospital and Washington Carolyn Johnston, MD University School of Medicine *Steven C. Plaxe, MD/Vice Chair Ω University of Michigan UC San Diego Moores Cancer Center Comprehensive Cancer Center Elena Ratner, MD Ω Ronald D. Alvarez, MD Ω Monica B. Jones, MD Ω Yale Cancer Center/ Vanderbilt-Ingram Cancer Center Duke Cancer Institute Smilow Cancer Hospital Jamie N. Bakkum-Gamez, MD Ω Charles A. Leath III, MD Ω Steven W. Remmenga, MD Ω Mayo Clinic Cancer Center University of Alabama at Birmingham Fred & Pamela Buffett Cancer Center Comprehensive Cancer Center Lisa Barroilhet, MD Ω Peter G. Rose, MD Ω University of Wisconsin Shashikant Lele, MD Ω Case Comprehensive Cancer Center/ Carbone Cancer Center Roswell Park Cancer Institute University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute Kian Behbakht, MD Ω Lainie Martin, MD † University of Colorado Cancer Center Fox Chase Cancer Center Paul Sabbatini, MD † Þ Memorial Sloan Kettering Cancer Center Lee-may Chen, MD Ω Ursula A. Matulonis, MD Ω † UCSF Helen Diller Family Dana-Farber/Brigham and Joseph T. Santoso, MD Ω Comprehensive Cancer Center Women’s Cancer Center St. Jude Children’s Research Hospital/ University of Tennessee Health Science Center Marta Ann Crispens, MD Ω David M. O’Malley, MD Ω Vanderbilt-Ingram Cancer Center The Ohio State University Shohreh Shahabi, MD Ω Comprehensive Cancer Center - Robert H. Lurie Comprehensive Cancer Center Mihaela Cristea, MD † James Cancer Hospital and of Northwestern University City of Hope Comprehensive Cancer Center Solove Research Institute Theresa L. Werner, MD † ‡ Maria DeRosa, RN ¥ Richard T. Penson, MD, MRCP Ω † Huntsman Cancer Institute Oliver Dorigo, MD, PhD Ω Massachusetts General Hospital at the University of Utah Stanford Cancer Institute Cancer Center NCCN David M. Gershenson, MD Ω Sanja Percac-Lima, MD Þ The University of Texas Massachusetts General Hospital Jennifer Burns MD Anderson Cancer Center Cancer Center Miranda Hughes, PhD Heidi J. Gray, MD Ω Mario Pineda, MD, PhD Ω Ω Gynecology oncology University of Washington Medical Center/ Robert H. Lurie Comprehensive Cancer ‡ Hematology/Hematology oncology Seattle Cancer Care Alliance Center of Northwestern University † Medical oncology Þ Internal medicine ≠ Ardeshir Hakam, MD Continue ≠ Pathology Moffitt Cancer Center ¥ Patient advocacy * Discussion writing committee member NCCN Guidelines Panel Disclosures Version 4.2017, 11/09/17 © National Comprehensive Cancer Network, Inc. 2017, All rights reserved. The NCCN Guidelines®and this illustration may not be reproduced in any form without the express written permission of NCCN®. NCCN Guidelines Index NCCN Guidelines Version 4.2017 Table of Contents Ovarian Cancer TOC Ovarian Cancer Discussion NCCN Ovarian Cancer Panel Members Clinical Trials: NCCN believes that Summary of the Guidelines Updates the best management for any patient Epithelial Ovarian Cancer/Fallopian Tube Cancer/Primary Peritoneal Cancer: with cancer is in a clinical trial. Clinical Presentation, Workup, Clinical Stage, Primary Treatment (OV-1) Participation in clinical trials is especially encouraged. Diagnosis by Previous Surgery: Findings and Primary Treatment (OV-2) Pathologic Staging, Primary Chemotherapy/Primary Adjuvant Therapy (OV-3) To find clinical trials online at NCCN Post-Primary Treatment: Secondary Adjuvant Therapy (OV-4) Member Institutions, click here: nccn.org/clinical_trials/physician.html. Monitoring/Follow-Up, Recurrent Disease (OV-5) Disease Status, Therapy for Persistent Disease or Recurrence (OV-6) NCCN Categories of Evidence and Consensus: All recommendations Less Common Ovarian Histopathologies: are category 2A unless otherwise Diagnosis (LCOH-1) indicated. Carcinosarcoma (Malignant Mixed Müllerian Tumors) (LCOH-2) See NCCN Categories of Evidence Clear Cell Carcinoma (LCOH-3) and Consensus. Mucinous Carcinoma (LCOH-4) Low-Grade (Grade 1) Serous/Endometrioid Epithelial Carcinoma (LCOH-5) Borderline Epithelial Tumors (Low Malignant Potential) (LCOH-6) NCCN Guidelines for Patients® are Malignant Sex Cord-Stromal Tumors (LCOH-9) available at www.nccn.org/patients. Malignant Germ Cell Tumors (LCOH-10) Surveillance for Malignant Germ Cell and Sex Cord-Stromal Tumors (LCOH-12) Staging (ST-1) Principles of Surgery (OV-A) Principles of Systemic Therapy (OV-B) • Primary Systemic Therapy Regimens (OV-B, 3 of 8) • Acceptable Recurrence Therapies (OV-B, 5 of 8) Management of Drug Reactions (OV-C) WHO Histologic Classification (OV-D) 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. ©2017. Version 4.2017, 11/09/17 © National Comprehensive Cancer Network, Inc. 2017, All rights reserved. The NCCN Guidelines®and this illustration may not be reproduced in any form without the express written permission of NCCN®. NCCN Guidelines Index NCCN Guidelines Version 4.2017 Updates Ovarian Cancer TOC Ovarian Cancer Discussion Updates in Version 4.2017 of the NCCN Guidelines for Ovarian Cancer from Version 3.2017 include: MS-1 • The Discussion section has been updated to reflect the changes in the algorithm. Updates in Version 3.2017 of the NCCN Guidelines for Ovarian Cancer from Version 2.2017 include: OV-6 • After platinum-based recurrence therapy for platinum-sensitive disease: Olaparib was added as a maintenance therapy option: "Consider niraparib or olaparib maintenance therapy if partial or complete response." Footnote "aa" added to olaparib: "For those with platinum-sensitive disease who have completed two or more lines of platinum- based therapy." Updates in Version 2.2017 of the NCCN Guidelines for Ovarian Cancer from Version 1.2017 include: OV-B (5 of 8)/(6 of 8) • Footnote"i" revised: "In general, the panel would recommend combination regimens, platinum-based regimens for platinum-sensitive recurrent disease based on randomized trial data, especially in first relapses." • Footnote "k" revised: In patients who have not previously received bevacizumab. There are limited data on the efficacy of bevacizumab in the recurrence therapy setting for patients previously treated with bevacizumab. • Footnote "m" added: "If response after chemotherapy, bevacizumab can be continued as maintenance therapy until disease progression or unacceptable toxicity." Continued on next page Version 4.2017, 11/09/17 © National Comprehensive Cancer Network, Inc. 2017, All rights reserved. The NCCN Guidelines®and this illustration may not be reproduced in any form without the express written permission of NCCN®. UPDATES NCCN Guidelines Index NCCN Guidelines Version 4.2017 Updates Ovarian Cancer TOC Ovarian Cancer Discussion Updates in Version 1.2017 of the NCCN Guidelines for Ovarian Cancer from Version 1.2016 include: General Less Common Ovarian Histopathologies • Imaging recommendations have been clarified to include the target anatomy, and LCOH-1 the recommended imaging modalities. • Reference added to footnote "b": "Committee on the State of the OV-1 Science in Ovarian Cancer R, Board on Health Care S, Institute of M, • Workup, added: "Evaluate total serum protein and nutritional status." et al. Ovarian Cancers: Evolving Paradigms in Research and Care. • Clinical stage column added. Primary treatment options significantly revised and Washington (DC): National Academies Press (US) Copyright 2016 by reorganized by clinical stage for those with a suspicious mass and/or symptoms. the National Academy of Sciences. All rights reserved; 2016." • Footnote added: "May be an option for select patients with stage IC based on LCOH-2 histology." • First adjuvant option revised: "Treat Chemotherapy as per epithelial OV-3 ovarian cancer (preferred) (See Primary Chemotherapy/Primary • Stage II-IV, clarified that IP and IV chemotherapy options are category 2A Adjuvant Therapy on OV-3)." recommendations for LCOH. LCOH-3 • Stage I-IV, following primary/adjuvant therapy, a link has been added to the NCCN • Adjuvant therapy for stage II-IV revised: "Treat Chemotherapy as Guidelines for Survivorship. per epithelial ovarian cancer