NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Vulvar Cancer (Squamous Cell Carcinoma) Version 2.2019 — December 17, 2018 NCCN.org Continue Version 2.2019, 12/17/18 © 2018 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 NCCN Guidelines Version 2.2019 Table of Contents Vulvar Cancer (Squamous Cell Carcinoma) Discussion *Wui-Jin Koh, MD/Chair § Shari Damast, MD § Christa Nagel, MD Ω Fred Hutchinson Cancer Research Yale Cancer Center/ Case Comprehensive Cancer Center/ Center/Seattle Cancer Care Alliance Smilow Cancer Hospital University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute *Nadeem R. Abu-Rustum, MD Ω/Vice Chair Oliver Dorigo, MD, PhD Ω Memorial Sloan Kettering Cancer Center Stanford Cancer Institute Larissa Nekhlyudov, MD, MPH Dana-Farber/Brigham and Women’s Sarah Bean, MD ≠ Patricia J. Eifel, MD § Cancer Center Duke Cancer Institute The University of Texas MD Anderson Cancer Center Amanda Nickles Fader, MD Ω Kristin Bradley, MD § The Sidney Kimmel Comprehensive University of Wisconsin Christine M. Fisher, MD, MPH § Cancer Center at Johns Hopkins Carbone Cancer Center University of Colorado Cancer Center Steven W. Remmenga, MD Ω Susana M. Campos, MD, MPH, MS † Peter Frederick, MD Ω Fred & Pamela Buffett Cancer Center Dana-Farber/Brigham and Women’s Roswell Park Cancer Institute Cancer Center R. Kevin Reynolds, MD Ω David K. Gaffney, MD, PhD § University of Michigan Kathleen R. Cho, MD ≠ Huntsman Cancer Institute Rogel Cancer Center University of Michigan at the University of Utah Rogel Cancer Center Ernest Han, MD, PhD Ω Todd Tillmanns, MD Ω Hye Sook Chon, MD Ω City of Hope Comprehensive St. Jude Children’s Research Hospital/ Moffitt Cancer Center Cancer Center The University of Tennessee Health Science Center Christina Chu, MD Ω Warner K. Huh, MD Ω Fox Chase Cancer Center University of Alabama at Birmingham Stefanie Ueda, MD Ω Comprehensive Cancer Center UCSF Helen Diller Family Rachel Clark Ω Comprehensive Cancer Center Massachusetts General Hospital John R. Lurain, III, MD Ω Cancer Center Robert H. Lurie Comprehensive Cancer Center of Northwestern University Emily Wyse ¥ David Cohn, MD Ω Patient Advocate The Ohio State University Comprehensive Andrea Mariani, MD Ω Cancer Center - James Cancer Hospital Mayo Clinic Cancer Center Catheryn M. Yashar, MD § and Solove Research Institute UC San Diego Moores Cancer Center David Mutch, MD Ω NCCN Marta Ann Crispens, MD Ω Siteman Cancer Center at Barnes- Nicole McMillian, MS Vanderbilt-Ingram Cancer Center Jewish Hospital and Washington Jillian Scavone, PhD University School of Medicine Ω Gynecologic oncology † Medical oncology NCCN Guidelines Panel Disclosures Continue § Radiotherapy/Radiation oncology ≠ Pathology ¥ Patient advocacy * Discussion Section Writing committee Version 2.2019, 12/17/18 © 2018 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 NCCN Guidelines Version 2.2019 Table of Contents Vulvar Cancer (Squamous Cell Carcinoma) Discussion NCCN Vulvar Cancer Panel Members Clinical Trials: NCCN believes that Summary of the Guidelines Updates the best management for any patient with cancer is in a clinical trial. Participation in clinical trials is Workup (VULVA-1) especially encouraged. Early Stage: T1, Smaller T2 (VULVA-2) To find clinical trials online at NCCN Adjuvant Therapy Based On Primary Tumor Risk Factors (VULVA-3) Member Institutions, click here: Adjuvant Therapy Based On Nodal Status (VULVA-4) nccn.org/clinical_trials/clinicians.aspx. Locally Advanced: Larger T2, T3 (VULVA-5) NCCN Categories of Evidence and Additional Treatment (VULVA-6) Consensus: All recommendations are category 2A unless otherwise Metastatic Disease Beyond Pelvis: Any T, Any N, M1 (VULVA-7) indicated. Surveillance (VULVA-8) See NCCN Categories of Evidence Therapy for Recurrence Clinically Limited to the Vulva (VULVA-9) and Consensus. Therapy for Clinical Nodal or Distant Recurrence (VULVA-10) NCCN Categories of Preference: All recommendations are considered Principles of Pathology (VULVA-A) appropriate. Principles of Imaging (VULVA-B) See NCCN Categories of Preference Principles of Surgery (VULVA-C) Principles of Radiation Therapy (VULVA-D) Systemic Therapy (VULVA-E) Staging (ST-1) For vulvar melanoma see the NCCN Guidelines for Cutaneous Melanoma 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 2.2019, 12/17/18 © 2018 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 NCCN Guidelines Version 2.2019 Table of Contents Vulvar Cancer (Squamous Cell Carcinoma) Discussion Updates in Version 2.2019 of the NCCN Guidelines for Vulvar Cancer from Version 1.2019 include: VULVA-A Principles of Pathology • This is a new section that includes recommendations for pathologic assessment after vulvectomy. MS-1 • The Discussion section has been updated to reflect the changes in the algorithm. Updates in Version 1.2019 of the NCCN Guidelines for Vulvar Cancer from Version 1.2018 include: VULVA-1 • Workup: New bullet added, “For elderly patients with vulvar cancer, see the NCCN Guidelines for Older Adult Oncology” VULVA-3 • Footnote k revised: “Other primary risk factors include: close tumor margins, lymphovascular invasion, negative but close tumor margins (<8 mm), tumor size....” VULVA-4 • Adjuvant therapy to the nodes: For SLN(s) positive, recommendation revised, “EBRT ± concurrent chemotherapy (category 1 for radiation).” • Footnote l revised: “If ipsilateral groin is positive, the contralateral groin should be evaluated surgically and/or treated with EBRT. In select cases of a single, small-volume, unilateral, positive inguinal node with a well-lateralized primary tumor diameter ≤2 cm and depth of invasion ≤5 mm and with a clinically negative contralateral groin examination, a contralateral groin dissection or radiation may be omitted. (Bosquet JG, Magrina Jf, Magtibay PM, et al. Patterns of inguinal groin metastases in squamous cell carcinoma of the vulva. Gynecologic Oncol 2007;105:742-746.)” VULVA-5 • Footnote o revised: “Larger T2 tumors: >4 cm and/or with involvement of the urethra, vagina, or anus.” VULVA-6 • Additional Treatment: Revised, “Consider biopsy of tumor bed to confirm pathologically complete response (pCR).” VULVA-8 • Workup: Second bullet revised, “Consider biopsy to confirm local and/or distant metastasis recurrence.” • The following reference was removed as footnote: “Salani R, Khanna N, Frimer M, et al. An update on post-treatment surveillance and diagnosis of recurrence in women with gynecologic malignancies: Society of Gynecol Oncology (SGO) recommendations. Gynecol Oncol 2017;146:3-10.” It is included in the Discussion section. VULVA-9 • Therapy for Recurrence: Recommendation revised, “Radical excision and ± unilateral or bilateral inguinofemoral LN dissection (if lymphadenectomy not previously performed)” Version 2.2019, 12/17/18 © 2018 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. UPDATES NCCN Guidelines Index NCCN Guidelines Version 2.2019 Table of Contents Vulvar Cancer (Squamous Cell Carcinoma) Discussion VULVA-B Principles of Surgery • 1 of 4 Tumor Margin Status Third bullet revised: “In the setting of a close or positive surgical tumor margin (<8 mm from tumor), re-resection may be considered to obtain more adequate wider margins.” Footnote removed: “For margins that are free but close (>0 mm but <8 mm), evidence is lacking to support decreased recurrence and improved survival with re-resection of disease or adjuvant local radiation to the primary tumor site.” • 2 of 4 Surgical Staging New bullet added: “Lymphadenectomy or SLN evaluation can be omitted in patients with stage IA primary disease with clinically negative groins due to a <1% risk of lymphatic metastases.” Bullet removed: “Some patients are not candidates for lymphadenectomy including those with stage IA disease due to a <1% risk of lymphatic metastases.” • 3 of 4 Inguinofemoral Senitnel Lymph Node Biopsy Seventh bullet revised: “...Approximately 3–4 cc of dye is injected peritumorally using a four-point injection technique at 2, 5, 7, and 10 o’clock... VULVA-C Principles of Radiation Therapy • This section was extensively revised. VULVA-D Systemic Therapy • The NCCN Categories