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Central Nervous System Cancers, Version 1.2015

Louis Burt Nabors, Jana Portnow, Mario Ammirati, Joachim Baehring, Henry Brem, Paul Brown, Nicholas Butowski, Marc C. Chamberlain, Robert A. Fenstermaker, Allan Friedman, Mark R. Gilbert, Jona Hattangadi-Gluth, Matthias Holdhoff, Larry Junck, Thomas Kaley, Ronald Lawson, Jay S. Loeffler, Mary P. Lovely, Paul L. Moots, Maciej M. Mrugala, Herbert B. Newton, Ian Parney, Jeffrey J. Raizer, Lawrence Recht, Nicole Shonka, Dennis C. Shrieve, Allen K. Sills Jr, Lode J. Swinnen, David Tran, Nam Tran, Frank D. Vrionis, Stephanie Weiss, Patrick Yung Wen, Nicole McMillian, and Anita M. Engh

The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Central Nervous System (CNS) Cancers provide interdisciplinary recommendations for managing adult CNS cancers. Primary and metastatic brain tumors are a heterogeneous group of neoplasms with varied outcomes and management strategies. These NCCN Guidelines Insights summarize the NCCN CNS Cancers Panel's discussion and highlight notable changes in the 2015 update. This article outlines the data and provides insight into panel decisions regarding adjuvant radiation and chemotherapy treatment options for high-risk newly diagnosed low-grade gliomas and glioblastomas. Additionally, it describes the panel's assessment of new data and the ongoing debate regarding the use of alternating electric field therapy for high-grade gliomas.

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Central Nervous System Cancers

Louis Burt Nabors, Mario Ammirati, Philip J. Bierman, Henry Brem, Nicholas Butowski, Marc C. Chamberlain, Lisa M. DeAngelis, Robert A. Fenstermaker, Allan Friedman, Mark R. Gilbert, Deneen Hesser, Matthias Holdhoff, Larry Junck, Ronald Lawson, Jay S. Loeffler, Moshe H. Maor, Paul L. Moots, Tara Morrison, Maciej M. Mrugala, Herbert B. Newton, Jana Portnow, Jeffrey J. Raizer, Lawrence Recht, Dennis C. Shrieve, Allen K. Sills Jr, David Tran, Nam Tran, Frank D. Vrionis, Patrick Y. Wen, Nicole McMillian, and Maria Ho

Primary and metastatic tumors of the central nervous system are a heterogeneous group of neoplasms with varied outcomes and management strategies. Recently, improved survival observed in 2 randomized clinical trials established combined chemotherapy and radiation as the new standard for treating patients with pure or mixed anaplastic oligodendroglioma harboring the 1p/19q codeletion. For metastatic disease, increasing evidence supports the efficacy of stereotactic radiosurgery in treating patients with multiple metastatic lesions but low overall tumor volume. These guidelines provide recommendations on the diagnosis and management of this group of diseases based on clinical evidence and panel consensus. This version includes expert advice on the management of low-grade infiltrative astrocytomas, oligodendrogliomas, anaplastic gliomas, glioblastomas, medulloblastomas, supratentorial primitive neuroectodermal tumors, and brain metastases. The full online version, available at NCCN. org, contains recommendations on additional subtypes.

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Survivorship: Cognitive Function, Version 1.2014

Crystal S. Denlinger, Jennifer A. Ligibel, Madhuri Are, K. Scott Baker, Wendy Demark-Wahnefried, Debra L. Friedman, Mindy Goldman, Lee Jones, Allison King, Grace H. Ku, Elizabeth Kvale, Terry S. Langbaum, Kristin Leonardi-Warren, Mary S. McCabe, Michelle Melisko, Jose G. Montoya, Kathi Mooney, Mary Ann Morgan, Javid J. Moslehi, Tracey O'Connor, Linda Overholser, Electra D. Paskett, Muhammad Raza, Karen L. Syrjala, Susan G. Urba, Mark T. Wakabayashi, Phyllis Zee, Nicole R. McMillian, and Deborah A. Freedman-Cass

Cognitive impairment is a common complaint among cancer survivors and may be a consequence of the tumors themselves or direct effects of cancer-related treatment (eg, chemotherapy, endocrine therapy, radiation). For some survivors, symptoms persist over the long term and, when more severe, can impact quality of life and function. This section of the NCCN Guidelines for Survivorship provides assessment, evaluation, and management recommendations for cognitive dysfunction in survivors. Nonpharmacologic interventions (eg, instruction in coping strategies; management of distress, pain, sleep disturbances, and fatigue; occupational therapy) are recommended, with pharmacologic interventions as a last line of therapy in survivors for whom other interventions have been insufficient.

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Melanoma, Version 2.2016, NCCN Clinical Practice Guidelines in Oncology

Daniel G. Coit, John A. Thompson, Alain Algazi, Robert Andtbacka, Christopher K. Bichakjian, William E. Carson III, Gregory A. Daniels, Dominick DiMaio, Marc Ernstoff, Ryan C. Fields, Martin D. Fleming, Rene Gonzalez, Valerie Guild, Allan C. Halpern, F. Stephen Hodi Jr, Richard W. Joseph, Julie R. Lange, Mary C. Martini, Miguel A. Materin, Anthony J. Olszanski, Merrick I. Ross, April K. Salama, Joseph Skitzki, Jeff Sosman, Susan M. Swetter, Kenneth K. Tanabe, Javier F. Torres-Roca, Vijay Trisal, Marshall M. Urist, Nicole McMillian, and Anita Engh

This selection from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Melanoma focuses on adjuvant therapy and treatment of in-transit disease, because substantial changes were made to the recommendations for the 2016 update. Depending on the stage of the disease, options for adjuvant therapy now include biochemotherapy and high-dose ipilimumab. Treatment options for in-transit disease now include intralesional injection with talimogene laherparepvec (T-VEC), a new immunotherapy. These additions prompted re-assessment of the data supporting older recommended treatment options for adjuvant therapy and in-transit disease, resulting in extensive revisions to the supporting discussion sections.

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Distress Management

Jimmie C. Holland, Barbara Andersen, William S. Breitbart, Luke O. Buchmann, Bruce Compas, Teresa L. Deshields, Moreen M. Dudley, Stewart Fleishman, Caryl D. Fulcher, Donna B. Greenberg, Carl B. Greiner, Rev. George F. Handzo, Laura Hoofring, Charles Hoover, Paul B. Jacobsen, Elizabeth Kvale, Michael H. Levy, Matthew J. Loscalzo, Randi McAllister-Black, Karen Y. Mechanic, Oxana Palesh, Janice P. Pazar, Michelle B. Riba, Kristin Roper, Alan D. Valentine, Lynne I. Wagner, Michael A. Zevon, Nicole R. McMillian, and Deborah A. Freedman-Cass

The integration of psychosocial care into the routine care of all patients with cancer is increasingly being recognized as the new standard of care. These NCCN Clinical Practice Guidelines in Oncology for Distress Management discuss the identification and treatment of psychosocial problems in patients with cancer. They are intended to assist oncology teams identify patients who require referral to psychosocial resources and to give oncology teams guidance on interventions for patients with mild distress to ensure that all patients with distress are recognized and treated.

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Pediatric Aggressive Mature B-Cell Lymphomas, Version 2.2020, NCCN Clinical Practice Guidelines in Oncology

Kimberly Davies, Matthew Barth, Saro Armenian, Anthony N. Audino, Phillip Barnette, Branko Cuglievan, Hilda Ding, James B. Ford, Paul J. Galardy, Rebecca Gardner, Rabi Hanna, Robert Hayashi, Alexandra E. Kovach, Andrea Judit Machnitz, Kelly W. Maloney, Lianna Marks, Kristin Page, Anne F. Reilly, Joanna L. Weinstein, Ana C. Xavier, Nicole R. McMillian, and Deborah A. Freedman-Cass

Pediatric aggressive mature B-cell lymphomas are the most common types of non-Hodgkin lymphoma in children, and they include Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL). These diseases are highly aggressive but curable, the treatment is complex, and patients may have many complicated supportive care issues. The NCCN Guidelines for Pediatric Aggressive Mature B-Cell Lymphomas provide guidance regarding pathology and diagnosis, staging, initial treatment, disease reassessment, surveillance, therapy for relapsed/refractory disease, and supportive care for clinicians who treat sporadic pediatric BL and DLBCL.

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Pediatric Central Nervous System Cancers, Version 2.2023, NCCN Clinical Practice Guidelines in Oncology

Amar Gajjar, Anita Mahajan, Mohamed Abdelbaki, Clarke Anderson, Reuben Antony, Tejus Bale, Ranjit Bindra, Daniel C. Bowers, Kenneth Cohen, Bonnie Cole, Kathleen Dorris, Ralph Ermoian, Andrea Franson, Jeffrey Helgager, Daniel Landi, Chi Lin, Laura Metrock, Ronica Nanda, Joshua Palmer, Sonia Partap, Ashley Plant, Sumit Pruthi, Renee Reynolds, Paul Ruggieri, Duncan Stearns, Phillip Storm, Anthony Wang, Katherine Warren, Nicholas Whipple, Wafik Zaky, Nicole R. McMillian, and Lenora A. Pluchino

Central nervous system (CNS) cancers account for approximately one quarter of all pediatric tumors and are the leading cause of cancer-related death in children. More than 4,000 brain and CNS tumors are diagnosed each year in children and teens, and the incidence rate has remained stagnant in recent years. The most common malignant pediatric CNS tumors are gliomas, embryonal tumors consisting of predominately medulloblastomas, and germ cell tumors. The inaugural version of the NCCN Guidelines for Pediatric Central Nervous System Cancers focuses on the diagnosis and management of patients with pediatric diffuse high-grade gliomas. The information contained in the NCCN Guidelines is designed to help clinicians navigate the complex management of pediatric patients with diffuse high-grade gliomas. The prognosis for these highly aggressive tumors is generally poor, with 5-year survival rates of <20% despite the use of combined modality therapies of surgery, radiation therapy and systemic therapy. Recent advances in molecular profiling has expanded the use of targeted therapies in patients whose tumors harbor certain alterations. However, enrollment in a clinical trial is the preferred treatment for eligible patients.

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Soft Tissue Sarcoma, Version 2.2012

Featured Updates to the NCCN Guidelines

Margaret von Mehren, Robert S. Benjamin, Marilyn M. Bui, Ephraim S. Casper, Ernest U. Conrad III, Thomas F. DeLaney, Kristen N. Ganjoo, Suzanne George, Ricardo Gonzalez, Martin J. Heslin, John M. Kane III, Joel Mayerson, Sean V. McGarry, Christian Meyer, Richard J. O'Donnell, Benjamin Paz, John D. Pfeifer, Raphael E. Pollock, R. Lor Randall, Richard F. Riedel, Scott Schuetze, Karen D. Schupak, Herbert S. Schwartz, Sridhar Shankar, Brian A. Van Tine, Jeffrey Wayne, Hema Sundar, and Nicole R. McMillian

The major changes to the 2012 and 2011 NCCN Guidelines for Soft Tissue Sarcoma pertain to the management of patients with gastrointestinal stromal tumors (GISTs) and desmoid tumors (aggressive fibromatosis). Postoperative imatinib following complete resection for primary GIST with no preoperative imatinib is now included as a category 1 recommendation for patients with intermediate or high risk of recurrence. The panel also reaffirmed the recommendation for preoperative use of imatinib in patients with GISTs that are resectable with negative margins but associated with significant surgical morbidity. Observation was included as an option for patients with resectable desmoid tumors that are small and asymptomatic, not causing morbidity, pain, or functional limitation. Sorafenib is included as an option for systemic therapy for patients with desmoid tumors.

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Merkel Cell Carcinoma, Version 1.2014

Christopher K. Bichakjian, Thomas Olencki, Murad Alam, James S. Andersen, Daniel Berg, Glen M. Bowen, Richard T. Cheney, Gregory A. Daniels, L. Frank Glass, Roy C. Grekin, Kenneth Grossman, Alan L. Ho, Karl D. Lewis, Daniel D. Lydiatt, William H. Morrison, Kishwer S. Nehal, Kelly C. Nelson, Paul Nghiem, Clifford S. Perlis, Ashok R. Shaha, Wade L. Thorstad, Malika Tuli, Marshall M. Urist, Timothy S. Wang, Andrew E. Werchniak, Sandra L. Wong, John A. Zic, Karin G. Hoffmann, Nicole R. McMillian, and Maria Ho

Merkel cell carcinoma is a rare, aggressive cutaneous tumor that combines the local recurrence rates of infiltrative nonmelanoma skin cancer with the regional and distant metastatic rates of thick melanoma. The NCCN Guidelines for Merkel Cell Carcinoma provide recommendations on the diagnosis and management of this aggressive disease based on clinical evidence and expert consensus. This version includes revisions regarding the use of PET/CT imaging and the addition of a new section on the principles of pathology to provide guidance on the analysis, interpretation, and reporting of pathology results.

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Esophageal and Esophagogastric Junction Cancers, Version 1.2015

Jaffer A. Ajani, Thomas A. D’Amico, Khaldoun Almhanna, David J. Bentrem, Stephen Besh, Joseph Chao, Prajnan Das, Crystal Denlinger, Paul Fanta, Charles S. Fuchs, Hans Gerdes, Robert E. Glasgow, James A. Hayman, Steven Hochwald, Wayne L. Hofstetter, David H. Ilson, Dawn Jaroszewski, Kory Jasperson, Rajesh N. Keswani, Lawrence R. Kleinberg, W. Michael Korn, Stephen Leong, A. Craig Lockhart, Mary F. Mulcahy, Mark B. Orringer, James A. Posey, George A. Poultsides, Aaron R. Sasson, Walter J. Scott, Vivian E. Strong, Thomas K. Varghese Jr, Mary Kay Washington, Christopher G. Willett, Cameron D. Wright, Debra Zelman, Nicole McMillian, and Hema Sundar

Esophageal cancer is the sixth most common cause of cancer deaths worldwide. Adenocarcinoma is more common in North America and Western European countries, originating mostly in the lower third of the esophagus, which often involves the esophagogastric junction (EGJ). Recent randomized trials have shown that the addition of preoperative chemoradiation or perioperative chemotherapy to surgery significantly improves survival in patients with resectable cancer. Targeted therapies with trastuzumab and ramucirumab have produced encouraging results in the treatment of advanced or metastatic EGJ adenocarcinomas. Multidisciplinary team management is essential for patients with esophageal and EGJ cancers. This portion of the NCCN Guidelines for Esophageal and EGJ Cancers discusses management of locally advanced adenocarcinoma of the esophagus and EGJ.