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320 NCCN Jatin P. Shah, MD, PhD; Sharon Spencer, MD; Andrea Trotti, III, MD; Randal S. Weber, MD; Gregory Wolf, MD; Mucosal Melanoma and Frank Worden, MD of the Head and Neck Overview Mucosal melanoma (MM) is a rare, very aggres- Clinical Practice Guidelines in Oncology sive noncutaneous melanoma that affects the upper David G. Pfister, MD; Kie-Kian Ang, MD, PhD; David M. Brizel, MD; aerodigestive tract, genitourinary tract, and anal/rec- Barbara Burtness, MD; Anthony J. Cmelak, MD; tal region.1 This portion of the NCCN Clinical Prac- A. Dimitrios Colevas, MD; Frank Dunphy, MD; David W. Eisele, MD; Jill Gilbert, MD; Maura L. Gillison, MD, PhD; tice Guidelines in Oncology (NCCN Guidelines) Robert I. Haddad, MD; Bruce H. Haughey, MBChB, MS; for Head and Neck (H&N) Cancers only describes Wesley L. Hicks, Jr., MD; Ying J. Hitchcock, MD; MMs of the H&N, which constitute fewer than 10% Merrill S. Kies, MD; William M. Lydiatt, MD; Ellie Maghami, MD; of melanomas of the H&N.1,2 Note that a separate Renato Martins, MD, MPH; Thomas McCaffrey, MD, PhD; Bharat B. Mittal, MD; Harlan A. Pinto, MD; NCCN Guideline is available for cutaneous melano- John A. Ridge, MD, PhD; Sandeep Samant, MD; ma (see NCCN Guidelines for Melanoma, available Giuseppe Sanguineti, MD; David E. Schuller, MD; in this issue and online at www.NCCN.org). NCCN Clinical Practice Guidelines in Please Note Oncology for Mucosal Melanoma of the The NCCN Clinical Practice Guidelines in Oncology ® Head and Neck (NCCN Guidelines ) are a statement of consensus of the authors regarding their views of currently accepted ap- proaches to treatment. Any clinician seeking to apply or Key Words consult the NCCN Guidelines® is expected to use indepen- NCCN Clinical Practice Guidelines, NCCN Guidelines, muco- dent medical judgment in the context of individual clinical sal melanoma, head and neck cancer, biopsy, neck dissec- circumstances to determine any patient’s care or treatment. tion, adjuvant therapy, radiation therapy, chemotherapy, The National Comprehensive Cancer Network® (NCCN®) pathology(JNCCN 2012;10:320–338) makes no representation or warranties of any kind regarding NCCN Categories of Evidence and Consensus their content, use, or application and disclaims any respon- Category 1: Based upon high-level evidence, there is uniform sibility for their applications or use in any way. NCCN consensus that the intervention is appropriate. © National Comprehensive Cancer Network, Inc. Category 2A: Based upon lower-level evidence, there 2012, All rights reserved. The NCCN Guidelines and the is uniform NCCN consensus that the intervention is illustrations herein may not be reproduced in any form appropriate. without the express written permission of NCCN. Category 2B: Based upon lower-level evidence, there is Disclosures for the NCCN Mucosal Melanoma NCCN consensus that the intervention is appropriate. of the Head and Neck Panel Category 3: Based upon any level of evidence, there is major NCCN disagreement that the intervention is At the beginning of each NCCN Guidelines panel meeting, panel appropriate. members disclosed any financial support they have received from industry. Through 2008, this information was published in an All recommendations are category 2A unless otherwise aggregate statement in JNCCN and online. Furthering NCCN’s noted. commitment to public transparency, this disclosure process has Clinical trials: NCCN believes that the best management for now been expanded by listing all potential conflicts of interest any cancer patient is in a clinical trial. Participation in clinical respective to each individual expert panel member. trials is especially encouraged. Individual disclosures for the NCCN Mucosal Melanoma of the Head and Neck Panel members can be found on page 338. (The most recent version of these guidelines and accompany- ing disclosures, including levels of compensation, are avail- able on the NCCN Web site at www.NCCN.org.) These guidelines are also available on the Internet. For the latest update, visit www.NCCN.org. © JNCCN–Journal of the National Comprehensive Cancer Network | Volume 10 Number 3 | March 2012 321 NCCN Guidelines® Mucosal Melanoma of the Journal of the National Comprehensive Cancer Network Head and Neck The full NCCN Guidelines for H&N Cancers Management Approaches address tumors arising in the upper aerodigestive The staging system for MM begins with stage III dis- tract (i.e., lip, oral cavity, pharynx, larynx, paranasal ease, which is the most limited form of disease for MM sinuses; see Figure 1). Occult primary cancer, salivary (see Workup and Staging, page 333).4 Surgery (with gland cancer, and MM are also addressed.3 Many of or without radiation therapy [RT]) is the primary the approaches for managing H&N cancer are also treatment for stage III MM, whereas surgery followed applicable to MM (e.g., multidisciplinary team, sur- by RT or systemic therapy is the primary treatment for gical principles). To view the full NCCN Guidelines stage IV MM, depending on systemic involvement. for H&N Cancers, visit the NCCN Web site at www. NCCN.org. By definition, the NCCN Guidelines cannot in- Multidisciplinary Team Involvement corporate all possible clinical variations and are not The initial evaluation and development of a plan intended to replace good clinical judgment or individ- for treating patients with MM require a multidisci- ualization of treatments. Exceptions to the rule were plinary team of health care providers with expertise discussed among the members of the NCCN H&N in caring for these patients. Similarly, managing Cancers Panel while developing these guidelines. and preventing sequelae of radical surgery, RT, and Text continues on p. 332 NCCN Mucosal Melanoma of the Head and City of Hope Comprehensive Cancer Center Renato Martins, MD, MPH† Neck Panel Members Fred Hutchinson Cancer Research Center/ *David G. Pfister, MD/Chair†Þ Seattle Cancer Care Alliance Memorial Sloan-Kettering Cancer Center Thomas McCaffrey, MD, PhDζ *Kie-Kian Ang, MD, PhD§ H. Lee Moffitt Cancer Center & Research Institute The University of Texas MD Anderson Cancer Center Bharat B. Mittal, MD§ *David M. Brizel, MD§ Robert H. Lurie Comprehensive Cancer Center of Duke Cancer Institute Northwestern University Barbara Burtness, MD† Harlan A. Pinto, MD†Þ Fox Chase Cancer Center Stanford Comprehensive Cancer Center John A. Ridge, MD, PhD¶ Anthony J. Cmelak, MD§ Fox Chase Cancer Center Vanderbilt-Ingram Cancer Center Sandeep Samant, MD¶ A. Dimitrios Colevas, MD† St. Jude Children’s Research Hospital/ Stanford Comprehensive Cancer Center University of Tennessee Cancer Institute Frank Dunphy, MD† Giuseppe Sanguineti, MD§ Duke Cancer Institute The Sidney Kimmel Comprehensive Cancer Center at David W. Eisele, MD¶ Johns Hopkins UCSF Helen Diller Family Comprehensive Cancer Center David E. Schuller, MD¶ Jill Gilbert, MD† The Ohio State University Comprehensive Cancer Center – Vanderbilt-Ingram Cancer Center James Cancer Hospital and Solove Research Institute *Maura L. Gillison, MD, PhD¶ *‡Jatin P. Shah, MD, PhD¶ The Ohio State University Comprehensive Cancer Center – Memorial Sloan-Kettering Cancer Center James Cancer Hospital and Solove Research Institute Sharon Spencer, MD§ Robert I. Haddad, MD† University of Alabama at Birmingham Dana-Farber/Brigham and Women’s Cancer Center Comprehensive Cancer Center ‡ Massachusetts General Hospital Cancer Center * Andrea Trotti, III, MD§ H. Lee Moffitt Cancer Center & Research Institute Bruce H. Haughey, MBChB, MS¶ Randal S. Weber, MD¶ Siteman Cancer Center at Barnes-Jewish Hospital and The University of Texas MD Anderson Cancer Center Washington University School of medicine Gregory Wolf, MD¶ζ Wesley L. Hicks, Jr., MD¶ University of Michigan Comprehensive Cancer Center Roswell Park Cancer Institute Frank Worden, MD¶† Ying J. Hitchcock, MD§ University of Michigan Comprehensive Cancer Center Huntsman Cancer Institute at the University of Utah NCCN Staff: Miranda Hughes, PhD, and Nicole McMillian, MS Merrill S. Kies, MD† KEY: The University of Texas MD Anderson Cancer Center *‡William M. Lydiatt, MD¶ζ *Writing Committee Member ‡ UNMC Eppley Cancer Center at Mucosal Melanoma Subcommittee Member. The Nebraska Medical Center Specialties: †Medical Oncology; ÞInternal Medicine; §Radiation Ellie Maghami, MD¶ζ Oncology; ¶Surgery/Surgical Oncology; ζOtolaryngology © JNCCN–Journal of the National Comprehensive Cancer Network | Volume 10 Number 3 | March 2012 322 Mucosal Melanoma of the Head and Neck Version 1.2012 PRESENTATION WORKUP PRIMARY ADJUVANT TREATMENT TREATMENT Strongly consider Stage III Wide surgical resection of primarya postoperative RT to primary siteb Postoperative RT T4a, N0 Wide surgical resectiona to primary siteb Recurrent or Persistent disease Follow-up (see the NCCN Clinical Practice Postoperative RT (see page 325) Wide surgical resection Sinus or nasal cavity T3-T4a, N1 to primary site and Guidelines in Oncology (NCCN + neck dissection of positive necka mucosal melanoma neckb Guidelines) for [cutaneous] Melanoma, elsewhere in this issue and online at www.NCCN.org) Clinical trial (preferred) • H&P including complete head and neck or exam; mirror and fiberoptic examination Stage IVB Primary RTb as clinically indicated or Biopsy c Verification of pathology using appropriate Systemic therapy confirms • staining (HMB-45, S-100, Melan-A) diagnosis of CT and/or MRI to determine anatomic malignant • mucosal extent of disease, particularly for sinus melanoma disease • Chest imaging as indicated • Consider PET-CT scan to rule