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Subtipo adverso o característica molecular en el condrosarcoma: subtipo desdiferenciado (high-gr...

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IDRF-CHONDROSARCOMA-HIGH-RISK-BIOLOGY
TipoSeñal de alerta
Situaciónreviewed 2026-04-26 | pending_clinical_signoff
EnfermedadesDIS-CHONDROSARCOMA
FuentesSRC-NCCN-NSCLC-2025

Origen de la bandera roja

DefiniciónAdverse subtype or molecular feature in chondrosarcoma: dedifferentiated subtype (high-grade sarcoma component), mesenchymal subtype (HEY1-NCOA2 fusion, Ewing-like biology), grade III conventional histology, IDH1 R132 mutation in advanced unresectable disease (ivosidenib-eligible), or CDKN2A loss — each shifts treatment from observation/surgery alone to systemic therapy or trial enrollment.
Dirección clínicaintensificar
Categoríahigh-risk-biology

Lógica de activación

{
  "any_of": [
    {
      "finding": "chondrosarcoma_subtype",
      "value": "dedifferentiated"
    },
    {
      "finding": "chondrosarcoma_subtype",
      "value": "mesenchymal"
    },
    {
      "finding": "chondrosarcoma_grade",
      "value": "III"
    },
    {
      "all_of": [
        {
          "finding": "BIO-IDH-MUTATION",
          "value": "positive"
        },
        {
          "finding": "unresectable_or_metastatic",
          "value": true
        }
      ]
    },
    {
      "finding": "BIO-CDKN2A",
      "value": "loss"
    }
  ],
  "type": "biomarker"
}

Notas

Chondrosarcoma decision-making is dominated by subtype + grade. Dedifferentiated (~10%) — biphasic with high-grade sarcoma component, behaves aggressively (5-yr OS ~30%) → doxorubicin + ifosfamide adjuvant by extrapolation from soft-tissue sarcoma protocols. Mesenchymal (~3–5%, young adults) — HEY1-NCOA2 fusion-positive small-round-blue-cell biology, treated like Ewing (VDC/IE protocols) — chemo-sensitive. Grade III conventional — consider adjuvant systemic therapy on trial. IDH1 R132 mutant (~40–50% of conventional) — ivosidenib has signal in advanced unresectable disease (Tap et al JCO 2020); off-label / trial. CDKN2A loss is prognostic in dedifferentiated subtype.

Utilizado por

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