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Tumor miofibroblástico inflamatório (IMT)

Visão determinística da entidade fonte YAML. A autoridade clínica permanece com os IDs de fonte citados e estado de assinatura do revisor.

IDDIS-IMT
TipoDoença
Alegais
IMTTumor miofibroblástico inflamatóriopseudotumor inflamatório (histórico, agora distinto)Granuloma de células plasmáticas (histórico)Запальна міофібробластична пухлина
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_morphology8825/1
icd_o_3_topography["C76", "C16", "C25", "C34", "C49", "C56"]
icd_10D48.1
who_classificationWHO Soft Tissue Tumours 5th ed (2020): Inflammatory myofibroblastic tumour (intermediate, rarely metastasizing)

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Notas

Mesenchymal neoplasm of intermediate biological behavior — locally invasive, ~25% recurrence rate, <5% metastatic. Fusion-driven biology (ALK most common) makes IMT one of the few sarcomas with broadly actionable targets. Therapy: - Localized: complete surgical resection — curative in most. - Unresectable / metastatic ALK+: crizotinib FDA-approved (pediatric + adult); lorlatinib for crizotinib-resistant. - ROS1+: entrectinib (FDA tumor-agnostic) or crizotinib. - NTRK+: larotrectinib / entrectinib (FDA tumor-agnostic). - Fusion-negative: surgery, radiation, sometimes corticosteroids / NSAIDs (historical), or chemotherapy by sarcoma protocol. IMT is one of the canonical examples of "rare disease, common driver" — small absolute numbers but high actionable-fraction; supports a case for universal molecular profiling at diagnosis.

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