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Mesenchymal Chondrosarcoma: a Review with Emphasis on its Fusion-Driven Biology

  • Sarcomas (SR Patel, Section Editor)
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Abstract

Mesenchymal chondrosarcoma is a rare but deadly form of chondrosarcoma that typically affects adolescents and young adults. While curative intent is possible for patients with localized disease, few options exist for patients in the unresectable/metastatic setting. Thus, it is imperative to understand the fusion-driven biology of this rare malignant neoplasm so as to lead to the future development of better therapeutics for this disease. This manuscript will briefly review the clinical and pathologic features of mesenchymal chondrosarcoma followed by an appraisal of existing data linked to the fusions, HEY1-NCOA2 and IRF2BP2-CDX1, and the associated downstream pathways.

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Authors and Affiliations

  1. Department of Orthopaedic Oncology, MD Anderson Cancer Center, Houston, TX, 77030, USA

    Marc El Beaino & Valerae Lewis

  2. Department of Melanoma Medical Oncology, MD Anderson Cancer Center, Houston, TX, 77030, USA

    Jason Roszik

  3. Department of Sarcoma Medical Oncology, MD Anderson Cancer Center, Houston, TX, 77030, USA

    John A. Livingston & Anthony P. Conley

  4. Department of Pathology, MD Anderson Cancer Center, Houston, TX, 77030, USA

    Wei-Lien Wang & Alexander J. Lazar

  5. Department of Diagnostic Radiology, MD Anderson Cancer Center, Houston, TX, 77030, USA

    Behrang Amini

  6. Department of Investigational Therapeutics, MD Anderson Cancer Center, Houston, TX, 77030, USA

    Vivek Subbiah

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  1. Marc El Beaino
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  2. Jason Roszik
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  3. John A. Livingston
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  4. Wei-Lien Wang
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  5. Alexander J. Lazar
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  6. Behrang Amini
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  7. Vivek Subbiah
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  8. Valerae Lewis
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  9. Anthony P. Conley
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Corresponding author

Correspondence to Anthony P. Conley.

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Conflict of Interest

Marc El Beaino declares that he has no conflict of interest.

Jason Roszik declares that he has no conflict of interest.

John A. Livingston declares that he has no conflict of interest.

Wei-Lien Wang declares that he has no conflict of interest.

Alexander J. Lazar declares that he has no conflict of interest.

Behrang Amini declares that he has no conflict of interest.

Vivek Subbiah has received research support through grants from Novartis, Bayer, AbbVie, and Roche/Genentech.

Valerae Lewis declares that she has no conflict of interest.

Anthony P. Conley declares that he has no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Sarcomas

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El Beaino, M., Roszik, J., Livingston, J.A. et al. Mesenchymal Chondrosarcoma: a Review with Emphasis on its Fusion-Driven Biology. Curr Oncol Rep 20, 37 (2018). https://doi.org/10.1007/s11912-018-0668-z

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  • DOI: https://doi.org/10.1007/s11912-018-0668-z

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