Desmoplastic Small-Round-Cell Tumor (DSRCT)

Overview

Desmoplastic small-round-cell tumor is a rare, highly aggressive soft-tissue sarcoma characterized by the EWSR1–WT1 fusion translocation. It predominantly affects adolescent males and typically presents in the peritoneum. It is classified under Soft Tissue Sarcoma in OncoTree (parent: SOFT_TISSUE). Prognosis is poor despite multimodal therapy.

Cohorts in the corpus

Recurrent alterations

  • EWSR1–WT1 fusion (canonical driver; not directly profiled in the UCLA PDTO study but defining molecular feature of the disease).
  • PIPseq cohort: EWSR1-WT1 fusion identified as diagnostic of desmoplastic small round cell tumor in a pediatric patient PMID:28007021

Subtypes

  • Monomorphic entity defined by EWSR1–WT1 fusion; no established molecular subtypes.

Therapeutic landscape

  • Patient-derived tumor organoid (PDTO) drug screening was applied across all 24 sarcoma subtypes in the UCLA cohort, including DSRCT. Across the full cohort, 80.4% of specimens screened had at least one significant drug response (top 5% viability rank), and 59% had at least one FDA-approved or NCCN-recommended top-five regimen. PMID:39305899

Sources

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

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