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Henry G. Kaplan

tumors Discuss the potential benefits of vemurafenib in the presence of a BRAF mutation Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon cancers that can demonstrate extremely aggressive behavior. Primary treatment involves surgical

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Javaughn Corey R. Gray, Jongho Kim, Michael Digianvittorio, Nancy K. Feeley, Paul J. Scheel, Stanley S. Siegelman, Elliot K. Fishman, and Steven P. Rowe

main therapeutic approach to ECD was interferon-α–based regimens. Now, there is increasing evidence that almost all patients carrying the BRAF V600E mutation have excellent responses to the now FDA-approved BRAF inhibitor vemurafenib and other BRAF

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Shailender Bhatia and John A. Thompson

disease met with failure in the past, and a pressing need existed for successful new therapies. However, this changed recently with the successful investigations of ipilimumab and vemurafenib, with both therapies associated with significantly improved

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Letizia Gandolfi, Sarah Adamo, Alessandro Pileri, Alessandro Broccoli, Lisa Argnani, and Pier Luigi Zinzani

intralesional “cytokine storm.” Recently, detection the BRAF V600E mutation changed the perspective of LCH, suggesting a possible use of BRAF inhibitors (ie, vemurafenib) in its treatment. 12 , 13 Case Report A previously healthy 40-year-old woman

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Michelle T. Ashworth and Adil Daud

specimen. Given the indolent clinical course to date, it was thought that rendering the patient free of visible disease could provide additional months to years of disease-free survival. A clinical trial of BRAF inhibitor (BRAFi) PLX4032 (vemurafenib

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Al B. Benson III, Alan P. Venook, Mahmoud M. Al-Hawary, Lynette Cederquist, Yi-Jen Chen, Kristen K. Ciombor, Stacey Cohen, Harry S. Cooper, Dustin Deming, Paul F. Engstrom, Ignacio Garrido-Laguna, Jean L. Grem, Axel Grothey, Howard S. Hochster, Sarah Hoffe, Steven Hunt, Ahmed Kamel, Natalie Kirilcuk, Smitha Krishnamurthi, Wells A. Messersmith, Jeffrey Meyerhardt, Eric D. Miller, Mary F. Mulcahy, James D. Murphy, Steven Nurkin, Leonard Saltz, Sunil Sharma, David Shibata, John M. Skibber, Constantinos T. Sofocleous, Elena M. Stoffel, Eden Stotsky-Himelfarb, Christopher G. Willett, Evan Wuthrick, Kristina M. Gregory, and Deborah A. Freedman-Cass

of EGFR and RAS in the MAPK signaling pathway. The BRAF V600E mutation results in constitutive MAPK signaling that encourages cellular proliferation. 37 Vemurafenib selectively inhibits the V600E-mutated form of the BRAF kinase, thereby reducing

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John A. Thompson

stage IV M1b or M1c disease, “the differences go away,” he noted. Molecularly Targeted Therapy With half of patients with metastatic melanoma harboring an activating mutation in BRAF , therapies targeting this mutation—vemurafenib and dabrafenib

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Melanoma, Version 2.2013

Featured Updates to the NCCN Guidelines

Daniel G. Coit, Robert Andtbacka, Christopher J. Anker, Christopher K. Bichakjian, William E. Carson III, Adil Daud, Dominick DiMaio, Martin D. Fleming, Valerie Guild, Allan C. Halpern, F. Stephen Hodi Jr., Mark C. Kelley, Nikhil I. Khushalani, Ragini R. Kudchadkar, Julie R. Lange, Anne Lind, Mary C. Martini, Anthony J. Olszanski, Scott K. Pruitt, Merrick I. Ross, Susan M. Swetter, Kenneth K. Tanabe, John A. Thompson, Vijay Trisal, Marshall M. Urist, Nicole McMillian, and Maria Ho

contentious. Advances in cancer immunotherapy and molecular targeting in melanoma have yielded 2 novel agents, ipilimumab and vemurafenib, both of which NCCN Guidelines Insights : Metastatic Colon Cancer, Version 2.2013 Version 2.2013 © National

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Douglas B. Johnson and Jeffrey A. Sosman

administered in 5 fractions to his atrial mass and 3500 cGy to the retroorbital mass. He subsequently initiated vemurafenib at 960 mg twice daily. Two months after starting vemurafenib, restaging scans showed a slight decrease in his lymphadenopathy but

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Daniel G. Coit and Anthony J. Olszanski

therapy. Within only 6 months, the second drug ever to show a survival advantage in patients with metastatic melanoma was approved by the FDA. The BRAF inhibitor vemurafenib produced a survival benefit over dacarbazine in the BRIM-3 trial, in which