Developments in Systemic Therapy for Soft Tissue and Bone Sarcomas

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Suzanne George
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The past year has led to significant changes in systemic therapies used to treat soft tissue sarcomas, mainly dominated by the removal of the recently approved drug olaratumab as part of combination therapy with doxorubicin from the NCCN Guidelines for Soft Tissue Sarcoma, according to Dr. Suzanne George. Several histology-specific drugs have entered the space, including pazopanib and pembrolizumab, the latter of which was approved as a category 2B recommendation for alveolar soft part sarcoma, highlighting the rather limited role of immunotherapy in sarcomas. Dr. George also discussed updated data for sorafenib in the treatment of desmoid tumors, as well as the importance of larotrectinib in TRK fusion–positive tumors.

Correspondence: Suzanne George, MD, Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA 02215. Email: sgeorge2@partners.org
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  • 1.

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  • 2.

    Phase III ANNOUNCE trial. Available at: https://investor.lilly.com/news-releases/news-release-details/lilly-reports-results-phase-3-soft-tissue-sarcoma-study. Accessed May 16, 2019.

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  • 3.

    US Food & Drug Administration. Olaratumab (LARTRUVO). FDA statement January 24, 2019. Available at: https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm525878.htm. Updated January 25, 2019. Accessed April 18, 2019.

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    Mir O, Cropet C, Toulmonde M, et al. Pazopanib plus best supportive care versus best supportive care alone in advanced gastrointestinal stromal tumours resistant to imatinib and sunitinib (PAZOGIST): a randomised, multicentre, open-label phase 2 trial. Lancet Oncol 2016;17:632641.

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    Stacchiotti S, Mir O, Le Cesne A, et al. Activity of pazopanib and trabectedin in advanced alveolar soft part sarcoma. Oncologist 2018;23:6270.

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    Groisberg R, Hong DS, Behrang A et al. Characteristics and outcomes of patients with advanced sarcoma enrolled in early phase immunotherapy trials. J Immunother Cancer 2017;5:100.

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    Gounder MM, Mahoney MR, Van Tine BA, et al. Sorafenib for advanced and refractory desmoid tumors. N Engl J Med 2018;379:24172428.

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    Drilon A, Laetsch TW, Kummar S, et al. Efficacy of larotrectinib in TRK fusion-positive cancers in adults and children. N Engl J Med 2018;378:731739.

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    Heinrich M, von Mehren M, Jones RL, et al. Avapritinib is highly active and well-tolerated in patients (pts) with advanced GIST driven by diverse variety of oncogenic mutations in KIT and PDGFRA [abstract]. Presented at 2018 CTOS Annual Meeting; November 14–17, 2018; Rome, Italy. Paper 012.

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  • 11.

    George S, Heinrich M, Chi P, et al. Initial results of phase 1 study of DCC-2618, a broad-spectrum KIT and PDGFRA inhibitor, in patients (PTS) with gastrointestinal stromal tumor (GIST) by number of prior regimens. Presented at the 2018 CTOS Annual Meeting; November 14–17, 2018; Rome, Italy. Paper 013.

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