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  • Author: Steven S. Brem x
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Steven S. Brem, Philip J. Bierman, Henry Brem, Nicholas Butowski, Marc C. Chamberlain, Ennio A. Chiocca, Lisa M. DeAngelis, Robert A. Fenstermaker, Allan Friedman, Mark R. Gilbert, Deneen Hesser, Larry Junck, Gerald P. Linette, Jay S. Loeffler, Moshe H. Maor, Madison Michael, Paul L. Moots, Tara Morrison, Maciej Mrugala, Louis Burt Nabors, Herbert B. Newton, Jana Portnow, Jeffrey J. Raizer, Lawrence Recht, Dennis C. Shrieve, Allen K. Sills Jr, Frank D. Vrionis and Patrick Y. Wen

Overview In 2010, an estimated 22,020 new cases of primary brain and other nervous system neoplasms were diagnosed in the United States,1 and approximately 13,140 deaths occurred from these tumors. The incidence of primary malignant brain tumors has been increasing over the past 30 years, especially in elderly persons.2 Metastatic disease to the central nervous system (CNS) occurs much more frequently, with an estimated incidence approximately 10 times that of primary brain tumors. Between 20% and 40% of patients with systemic cancer will develop brain metastases.3 NOTE: This manuscript highlights only a portion of the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Central Nervous System Cancers. Please refer to for the complete NCCN Guidelines. Principles of Management Primary and metastatic brain tumors are a heterogeneous group of neoplasms with varied outcomes and management strategies. Primary brain tumors range from pilocytic astrocytomas, which are very uncommon, noninvasive, and surgically curable, to glioblastoma multiforme, the most common intraparenchymal brain tumor in adults, which is highly invasive and virtually incurable. Likewise, patients with metastatic brain disease may have rapidly progressive systemic disease or no systemic cancer at all. These patients may have one or dozens of brain metastases, and may have a malignancy that is either highly responsive or highly resistant to radiation or chemotherapy. Because of this marked heterogeneity, the prognostic features and treatment options for brain tumors must be carefully reviewed on an individual basis and sensitively communicated to each patient. In addition, CNS tumors are associated with...
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Louis Burt Nabors, Jana Portnow, Mario Ammirati, Joachim Baehring, Henry Brem, Nicholas Butowski, Robert A. Fenstermaker, Peter Forsyth, Jona Hattangadi-Gluth, Matthias Holdhoff, Steven Howard, Larry Junck, Thomas Kaley, Priya Kumthekar, Jay S. Loeffler, Paul L. Moots, Maciej M. Mrugala, Seema Nagpal, Manjari Pandey, Ian Parney, Katherine Peters, Vinay K. Puduvalli, John Ragsdale III, Jason Rockhill, Lisa Rogers, Chad Rusthoven, Nicole Shonka, Dennis C. Shrieve, Allen K. Sills Jr, Lode J. Swinnen, Christina Tsien, Stephanie Weiss, Patrick Yung Wen, Nicole Willmarth, Mary Anne Bergman and Anita Engh

For many years, the diagnosis and classification of gliomas have been based on histology. Although studies including large populations of patients demonstrated the prognostic value of histologic phenotype, variability in outcomes within histologic groups limited the utility of this system. Nonetheless, histology was the only proven and widely accessible tool available at the time, thus it was used for clinical trial entry criteria, and therefore determined the recommended treatment options. Research to identify molecular changes that underlie glioma progression has led to the discovery of molecular features that have greater diagnostic and prognostic value than histology. Analyses of these molecular markers across populations from randomized clinical trials have shown that some of these markers are also predictive of response to specific types of treatment, which has prompted significant changes to the recommended treatment options for grade III (anaplastic) gliomas.