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Systemic Therapy for Chondrosarcoma

  • Sarcoma (SH Okuno, Section Editor)
  • Published:
Current Treatment Options in Oncology Aims and scope Submit manuscript

Opinion statement

Clinical trial enrollment should be actively encouraged in all patients diagnosed with advanced, surgically unresectable chondrosarcoma (CS) due to the lack of consensus treatment recommendations. In the absence of an appropriate clinical trial, treatments are determined based on histologic subtype of CS with consideration given to targetable mutations (i.e., IDH1). Conventional CS is inherently resistant to cytotoxic chemotherapy and patients may benefit from antiangiogenic therapy including off-label use of pazopanib. Individuals harboring an IDH1 mutation may derive clinical benefit from ivosidenib, an IDH1 inhibitor. Upon progression and with functional status permitting, alternative options include mTOR inhibitors (sirolimus, temsirolimus) or other tyrosine kinase inhibitors (dasatinib), though no clear sequencing data exists. For dedifferentiated CS, conventional chemotherapies with osteosarcoma-like regimens are upfront options although prospective data is limited with minimal overall benefit. Alternative treatment options include immunotherapy with pembrolizumab or ivosidenib in IDH1-mutant, dedifferentiated CS, but questionable efficacy was observed in small sample sizes with either approach. In mesenchymal CS, treatment with Ewing sarcoma-like chemotherapy regimens may be considered, although data supporting its use is even more limited given its rarity.

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  1. Harbor-UCLA Medical Center, 1000 W. Carson St, Torrance, CA, 90502, USA

    Adam Rock MD & Sana Ali MD

  2. University of California Irvine, 101 The City Drive South, Orange, CA, 92868, USA

    Warren A. Chow MD, FACP

  3. UCI Health, 101 The City Drive South, Building 63, Room 412, ZOT 4061, Orange, CA, 92868, USA

    Warren A. Chow MD, FACP

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Adam Rock reports other financial activities from Bristol Myers Squibb, Merck & Co Inc., Cigna Corporation, Forian, outside the submitted work. Sana Ali declares that she has no conflict of interest. Warren Chow reports other financial activities from Advenchen, Inhibrx, GSK, outside the submitted work.

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Rock, A., Ali, S. & Chow, W.A. Systemic Therapy for Chondrosarcoma. Curr. Treat. Options in Oncol. 23, 199–209 (2022). https://doi.org/10.1007/s11864-022-00951-7

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