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Condrossarcoma

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IDDIS-CHONDROSARCOMA
TipoDoença
Alegais
Sarcoma cartilaginosoХондросаркома
Estadoreviewed 2026-04-27 | pending_clinical_signoff
DoençasNenhuma declaração
FontesSRC-NCCN-NSCLC-2025

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Fonte YAML

icd_o_3_morphology9220/3
icd_o_3_topography["C40", "C41"]
icd_10C40, C41
who_classificationWHO Soft Tissue and Bone Tumours 5th ed (2020): Chondrosarcoma — conventional, dedifferentiated, mesenchymal, clear cell subtypes

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Cartilaginous primary bone neoplasm — historically considered chemotherapy-resistant and radiotherapy-resistant. Surgery is the principal treatment. Subtypes: - Conventional (~85%): IDH1/2 mutant ~50%; surgery + risk-adapted follow-up; metastatic disease has limited systemic options. - Dedifferentiated (~10%): biphasic — chondrosarcoma + high-grade sarcoma component; behaves like high-grade sarcoma; doxorubicin- based chemotherapy + surgery. - Mesenchymal (~3-5%): small-round-blue-cell biology; HEY1-NCOA2 fusion; pediatric / young adult; chemo-sensitive (Ewing-like protocols). - Clear cell (~2%): low-grade; surgery. Targeted therapy: - IDH1 R132 mutant (~40-50% of conventional): ivosidenib has activity in advanced unresectable IDH1-mutant chondrosarcoma (Tap et al JCO 2020) — small phase 1 cohort, durable disease control; not yet FDA-approved for chondrosarcoma but used off-label. - IDH2 mutant: enasidenib in similar exploratory setting. Niche actionability — represents a small but real precision-oncology signal in a disease historically without systemic options.

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