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

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27 Scopus citations

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.

Original languageEnglish (US)
Article number37
JournalCurrent oncology reports
Volume20
Issue number5
DOIs
StatePublished - May 1 2018

Keywords

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

ASJC Scopus subject areas

  • Oncology

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