Central Nervous System Cancers Panel Members Can Be Found on Page 1151
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1114 NCCN David Tran, MD, PhD; Nam Tran, MD, PhD; Frank D. Vrionis, MD, MPH, PhD; Patrick Y. Wen, MD; Central Nervous Nicole McMillian, MS; and Maria Ho, PhD System Cancers Overview In 2013, an estimated 23,130 people in the United Clinical Practice Guidelines in Oncology States will be diagnosed with primary malignant brain Louis Burt Nabors, MD; Mario Ammirati, MD, MBA; and other central nervous system (CNS) neoplasms.1 Philip J. Bierman, MD; Henry Brem, MD; Nicholas Butowski, MD; These tumors will be responsible for approximately Marc C. Chamberlain, MD; Lisa M. DeAngelis, MD; 14,080 deaths. The incidence of primary brain tumors Robert A. Fenstermaker, MD; Allan Friedman, MD; Mark R. Gilbert, MD; Deneen Hesser, MSHSA, RN, OCN; has been increasing over the past 30 years, especially in Matthias Holdhoff, MD, PhD; Larry Junck, MD; elderly persons.2 Metastatic disease to the CNS occurs Ronald Lawson, MD; Jay S. Loeffler, MD; Moshe H. Maor, MD; much more frequently, with an estimated incidence ap- Paul L. Moots, MD; Tara Morrison, MD; proximately 10 times that of primary brain tumors. An Maciej M. Mrugala, MD, PhD, MPH; Herbert B. Newton, MD; Jana Portnow, MD; Jeffrey J. Raizer, MD; Lawrence Recht, MD; estimated 20% to 40% of patients with systemic cancer Dennis C. Shrieve, MD, PhD; Allen K. Sills Jr, MD; will develop brain metastases.3 Abstract Please Note Primary and metastatic tumors of the central nervous system are The NCCN Clinical Practice Guidelines in Oncology a heterogeneous group of neoplasms with varied outcomes and (NCCN Guidelines®) are a statement of consensus of the management strategies. Recently, improved survival observed in 2 randomized clinical trials established combined chemotherapy authors regarding their views of currently accepted ap- and radiation as the new standard for treating patients with proaches to treatment. Any clinician seeking to apply or ® pure or mixed anaplastic oligodendroglioma harboring the consult the NCCN Guidelines is expected to use inde- 1p/19q codeletion. For metastatic disease, increasing evidence pendent medical judgment in the context of individual supports the efficacy of stereotactic radiosurgery in treating clinical circumstances to determine any patient’s care or patients with multiple metastatic lesions but low overall tumor treatment. The National Comprehensive Cancer Net- volume. These guidelines provide recommendations on the diag- work® (NCCN®) makes no representation or warranties nosis and management of this group of diseases based on clinical of any kind regarding their content, use, or application evidence and panel consensus. This version includes expert advice and disclaims any responsibility for their applications or on the management of low-grade infiltrative astrocytomas, oligodendrogliomas, anaplastic gliomas, glioblastomas, medul- use in any way. The full NCCN Guidelines for Central loblastomas, supratentorial primitive neuroectodermal tumors, Nervous System Cancers are not printed in this issue of and brain metastases. The full online version, available at NCCN. JNCCN but can be accessed online at NCCN.org. org, contains recommendations on additional subtypes. (JNCCN © National Comprehensive Cancer Network, Inc. 2013;11:1114–1151) 2013, All rights reserved. The NCCN Guidelines and the NCCN Categories of Evidence and Consensus illustrations herein may not be reproduced in any form Category 1: Based upon high-level evidence, there is uni- without the express written permission of NCCN. form NCCN consensus that the intervention is appropriate. Disclosures for the Central Nervous System Category 2A: Based upon lower-level evidence, there Cancers Panel is uniform NCCN consensus that the intervention is appropriate. At the beginning of each NCCN Guidelines panel meeting, panel Category 2B: Based upon lower-level evidence, there is members review all potential conflicts of interest. NCCN, in keep- NCCN consensus that the intervention is appropriate. ing with its commitment to public transparency, publishes these disclosures for panel members, staff, and NCCN itself. Category 3: Based upon any level of evidence, there is major NCCN disagreement that the intervention is appropriate. Individual disclosures for the NCCN Central Nervous System Cancers Panel members can be found on page 1151. (The most All recommendations are category 2A unless otherwise noted. recent version of these guidelines and accompanying disclosures are available on the NCCN Web site at NCCN.org.) Clinical trials: NCCN believes that the best management for any cancer patient is in a clinical trial. Participation in clinical These guidelines are also available on the Internet. For the trials is especially encouraged. latest update, visit NCCN.org. © JNCCN—Journal of the National Comprehensive Cancer Network | Volume 11 Number 9 | September 2013 1115 NCCN Guidelines® Central Nervous Journal of the National Comprehensive Cancer Network System Cancers Principles of Management nicated to each patient. In addition, CNS tumors are Primary and metastatic brain tumors are a heteroge- associated with a range of symptoms and complications, neous group of neoplasms with varied outcomes and such as edema, seizures, endocrinopathy, fatigue, psychi- management strategies. Primary brain tumors range atric disorders, and venous thromboembolism, that can from pilocytic astrocytomas, which are very uncommon, seriously impact quality of life. The involvement of an noninvasive, and surgically curable, to glioblastoma interdisciplinary team, including neurosurgeons, radia- multiforme, the most common intraparenchymal brain tion therapists, oncologists, neurologists, and neuroradi- tumor in adults, which is highly invasive and virtu- ologists, is a key factor in the appropriate management ally incurable. Likewise, patients with metastatic brain of these patients. For any subtype of malignant brain disease may have rapidly progressive systemic disease lesions, the NCCN CNS Panel encourages a thorough or no systemic cancer at all. These patients may have multidisciplinary review of each patient case once the one or dozens of brain metastases, and they may have pathology results are available. a malignancy that is highly responsive or, alternatively, highly resistant to radiation or chemotherapy. Because Treatment Principles of this marked heterogeneity, the prognostic features Several important principles guide surgical and radi- and treatment options for brain tumors must be carefully ation therapy (RT) for adults with brain tumors. Re- reviewed on an individual basis and sensitively commu- gardless of tumor histology, neurosurgeons generally Text cont. on page 1134. NCCN Central Nervous System Cancers iTara Morrison, MDΨ Fox Chase Cancer Center Panel Members *c,d,gMaciej Mrugala, MD, PhD, MPHΨ *f,gLouis Burt Nabors, MD/ChairΨ University of Washington/Seattle Cancer Care Alliance University of Alabama at Birmingham Herbert B. Newton, MDΨ Comprehensive Cancer Center The Ohio State University Comprehensive Cancer Center – Mario Ammirati, MD, MBA¶ James Cancer Hospital and Solove Research Institute The Ohio State University Comprehensive Cancer Center – *d,gJana Portnow, MD†Ψ James Cancer Hospital and Solove Research Institute City of Hope Comprehensive Cancer Center cPhilip J. Bierman, MD†‡ a,fJeffrey J. Raizer, MDΨ UNMC Eppley Cancer Center at Robert H. Lurie Comprehensive Cancer Center of The Nebraska Medical Center Northwestern University aHenry Brem, MD¶ Lawrence Recht, MD The Sidney Kimmel Comprehensive Cancer Center at Ψ Johns Hopkins Stanford Cancer Institute h Nicholas Butowski, MDΨ Dennis C. Shrieve, MD, PhD§ UCSF Helen Diller Family Comprehensive Cancer Center Huntsman Cancer Institute at the University of Utah a,b,h,i *a,b,e,gMarc C. Chamberlain, MDΨ Allen K. Sills Jr, MD¶ University of Washington/Seattle Cancer Care Alliance Vanderbilt-Ingram Cancer Center a,cLisa M. DeAngelis, MDΨ David Tran, MD, PhD† Memorial Sloan-Kettering Cancer Center Siteman Cancer Center at Barnes-Jewish Hospital and a,bRobert A. Fenstermaker, MD¶ Washington University School of Medicine Roswell Park Cancer Institute Nam Tran, MD, PhD¶ Allan Friedman, MD¶ Moffitt Cancer Center Duke Cancer Institute *b,eFrank D. Vrionis, MD, MPH, PhD¶ Mark R. Gilbert, MDΨ Moffitt Cancer Center The University of Texas MD Anderson Cancer Center gPatrick Y. Wen, MDΨ a,i Deneen Hesser, MSHSA, RN, OCN¥ Dana-Farber/Brigham and Women’s Cancer Center American Brain Tumor Association gMatthias Holdhoff, MD, PhD† NCCN Staff: Maria Ho, PhD, and Nicole McMillian, MS The Sidney Kimmel Comprehensive Cancer Center at KEY: Johns Hopkins a,f,g,hLarry Junck, MDΨ *Writing Committee Member University of Michigan Comprehensive Cancer Center Subcommittees: aMeningiomas; bMetastatic Spine Tumors; Ronald Lawson, MD cPCNSL Review; dAdult Medulloblastoma; ePrimary Spinal Cord St. Jude Children’s Research Hospital/ Tumors; fPrinciples of Imaging; gPrinciples of Systemic Therapy; The University of Tennessee Health Science Center hPrinciples of Radiation Therapy; iPrinciples of Brain Tumor a,h Jay S. Loeffler, MD§ Management (Note: Underlining denotes subcommittee lead) Massachusetts General Hospital Cancer Center *b,hMoshe H. Maor, MD§ Specialties: †Medical Oncology; ‡Hematology/Hematology The University of Texas MD Anderson Cancer Center Oncology; §Radiotherapy/Radiation Oncology; ΨNeurology/ aPaul L. Moots, MDΨ Neuro-Oncology; ¶Surgery/Surgical Oncology; ¥Patient Vanderbilt-Ingram Cancer Center Advocacy © JNCCN—Journal of the National Comprehensive Cancer Network | Volume