Chondrosarcoma (CHS)

Overview

Chondrosarcoma is a malignant cartilaginous bone tumor, classified under Bone Cancer in OncoTree (parent: BONE). It is the second most common primary bone malignancy after osteosarcoma and is generally resistant to chemotherapy and radiation, making surgery the mainstay of treatment. Subtypes include conventional, dedifferentiated (DDCHS), clear cell, and mesenchymal variants.

Cohorts in the corpus

Recurrent alterations

  • No recurrent driver alterations directly genotyped in the UCLA PDTO study; chondrosarcoma is commonly associated with IDH1/IDH2 mutations (conventional) and complex copy-number changes (dedifferentiated) in the broader literature.

Subtypes

  • Conventional chondrosarcoma (grade I–III)
  • Dedifferentiated chondrosarcoma (DDCHS)
  • Clear cell and mesenchymal chondrosarcoma (less common)

Therapeutic landscape

  • TAE226 (FAK/Pyk2 inhibitor): chondrosarcoma PDTOs showed preferential sensitivity (p=0.04). PMID:39305899
  • BX-912 (PDK1 inhibitor): preferential sensitivity in chondrosarcoma PDTOs (p=0.038). PMID:39305899
  • Patient-derived tumor organoid (PDTO) drug screening identified at least one FDA-approved or NCCN-recommended regimen for 59% of sarcoma samples overall across the 24-subtype cohort. PMID:39305899

Sources

  • PMID:39305899 — Al Shihabi et al. Cell Stem Cell 2024. UCLA sarcoma PDTO drug-sensitivity landscape.

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