Dedifferentiated Chondrosarcoma (DDCHS)

Overview

Dedifferentiated chondrosarcoma is a high-grade variant of chondrosarcoma characterized by a well-differentiated cartilaginous component juxtaposed with an abrupt transition to a high-grade non-cartilaginous sarcomatous component. It is classified under Bone Cancer in OncoTree (parent: CHS). Prognosis is poor, with 5-year survival typically <15%.

Cohorts in the corpus

  • sarcoma_ucla_2024: represented as a subtype within the 24-subtype UCLA sarcoma PDTO biobank (194 specimens from 126 patients, Feb 2018–May 2022). PMID:39305899

Recurrent alterations

  • No specific alterations for DDCHS directly genotyped in the UCLA PDTO study. TP53, RB1, and complex copy-number events are reported in the broader literature.

Subtypes

  • Subtype of CHS; the dedifferentiated component may resemble osteosarcoma, undifferentiated pleomorphic sarcoma, or fibrosarcoma histologically.

Therapeutic landscape

  • Patient-derived tumor organoid (PDTO) screening was applied across all 24 sarcoma subtypes in the UCLA cohort, including DDCHS, identifying FDA-approved or NCCN-recommended regimens for 59% of samples overall. PMID:39305899

Sources

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

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