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

  • Marc El Beaino
  • Jason Roszik
  • John A. Livingston
  • Wei-Lien Wang
  • Alexander J. Lazar
  • Behrang Amini
  • Vivek Subbiah
  • Valerae Lewis
  • Anthony P. Conley
Sarcomas (SR Patel, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Sarcomas

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.

Keywords

Mesenchymal chondrosarcoma Chondrosarcoma Fusion Translocation Notch HEY1 NCOA2 IRF2BP2 CDX1 Chromatin remodeling TGF beta Apoptosis Genomics Pathways 

Notes

Compliance with Ethical Standards

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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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Marc El Beaino
    • 1
  • Jason Roszik
    • 2
  • John A. Livingston
    • 3
  • Wei-Lien Wang
    • 4
  • Alexander J. Lazar
    • 4
  • Behrang Amini
    • 5
  • Vivek Subbiah
    • 6
  • Valerae Lewis
    • 1
  • Anthony P. Conley
    • 3
  1. 1.Department of Orthopaedic OncologyMD Anderson Cancer CenterHoustonUSA
  2. 2.Department of Melanoma Medical OncologyMD Anderson Cancer CenterHoustonUSA
  3. 3.Department of Sarcoma Medical OncologyMD Anderson Cancer CenterHoustonUSA
  4. 4.Department of PathologyMD Anderson Cancer CenterHoustonUSA
  5. 5.Department of Diagnostic RadiologyMD Anderson Cancer CenterHoustonUSA
  6. 6.Department of Investigational TherapeuticsMD Anderson Cancer CenterHoustonUSA

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