Chordoma (CHDM)

Overview

Chordoma is a rare, slow-growing malignant bone tumor arising from notochordal remnants, classified under Bone Cancer in OncoTree (parent: BONE). It most commonly occurs in the skull base, spine, and sacrum. Chordomas are generally resistant to standard cytotoxic chemotherapy.

Cohorts in the corpus

Recurrent alterations

  • EGFR/ERBB2: pathway implicated by KEGG analysis in multiple chordoma samples (SARC0046_2/3, SARC0053_a, SARC0049); chordomas preferentially sensitive to TAK-285 (EGFR/ERBB2 kinase inhibitor, p=0.034). PMID:39305899

Subtypes

  • Conventional chordoma (notochordal origin); dedifferentiated chordoma variants also recognized.

Therapeutic landscape

  • everolimus: chordoma PDTOs were significantly less sensitive than other sarcomas (p=0.022); alvocidib (p=0.00046), apitolisib (p=0.0071), and bortezomib (p=1.8×10⁻⁵) also showed relative resistance. PMID:39305899
  • TAK-285 (EGFR/ERBB2 inhibitor): chordomas preferentially sensitive (p=0.034); BI-D1870 (RSK inhibitor, p=0.047) and A-769662 (AMPK activator, p=0.0015) also showed preferential sensitivity. PMID:39305899
  • Minimal ex vivo proliferation in PDTOs noted, which limited screening throughput for some chordoma samples. PMID:39305899

Sources

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

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