Hepatoblastoma
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| ID | DIS-HEPATOBLASTOMA |
|---|---|
| Tipo | Doença |
| Alegais | Hepatoblastoma associado a Beckwith-WiedemannHepatoblastoma infantilTumor embrionário do fígado pediátricoГепатобластома |
| Estado | reviewed 2026-05-19 | pending_clinical_signoff |
| Doenças | Nenhuma declaração |
| Fontes | SRC-AASLD-HCC-2023 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8970/3 |
|---|---|
| icd_o_3_topography | ["C22.0"] |
| icd_10 | C22.2 |
| who_classification | WHO Digestive System Tumours 5th ed (2019): Hepatoblastoma |
Notas
Treatment philosophy combines pre-operative cisplatin-based chemo (SIOPEL PLADO / PLATO regimens) + surgical resection or liver transplantation. AFP is a key tumor marker — used for diagnosis, monitoring response, and surveillance in Beckwith-Wiedemann carriers (q3mo AFP from birth to age 3-4). High-risk subset (PRETEXT IV, +V involvement, metastatic) receives intensified regimens. Five-year EFS approaches ~70-80% across all risk groups in current SIOPEL / COG-AHEP cooperatives.
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Fontes
SRC-BRIOUDE-BECKWITH-WIEDEMANN-2018- Documento de consenso de peritos: Diagnóstico clínico e molecular, triagem e manejo de...SRC-KALISH-BECKWITH-WIEDEMANN-2017- Recomendações de Vigilância para Crianças com Síndromes de Sobrecrescimento e...