Liposarcoma (LIPO)

Overview

Liposarcoma is the most common soft-tissue sarcoma of adults, classified under Soft Tissue Sarcoma in OncoTree (parent: SOFT_TISSUE). It encompasses several distinct subtypes including well-differentiated (WDLS), dedifferentiated (DDLS), myxoid/round-cell, and pleomorphic liposarcoma, with different molecular drivers and clinical behavior.

Cohorts in the corpus

Recurrent alterations

  • CDK4: prevalent CDK aberration in liposarcoma literature (cited as motivation for palbociclib testing); not directly genotyped in this study. PMID:39305899
  • MDM2: amplification is a defining feature of well-differentiated/dedifferentiated liposarcoma; confirmed by FISH in WDLS SARC0120 in both parent tumor and PDTO, illustrating genomic fidelity of PDTO models. PMID:39305899

Subtypes

  • Well-differentiated liposarcoma (WDLS) — MDM2/CDK4 amplified
  • Dedifferentiated liposarcoma (DDLS) — MDM2/CDK4 amplified, high-grade
  • Myxoid/round-cell liposarcoma — FUS–DDIT3 or EWSR1–DDIT3 fusion
  • Pleomorphic liposarcoma — complex karyotype

Therapeutic landscape

  • palbociclib: CDK4/6 inhibitor tested across UCLA PDTO sarcoma cohort; CDK4 prevalence in liposarcoma motivates this approach. PMID:39305899
  • Patient-derived tumor organoid (PDTO) drug screening identified ≥1 FDA-approved or NCCN-recommended regimen for 59% of sarcoma 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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