Tumor de Wilms (nefroblastoma)
Visão determinística da entidade fonte YAML. A autoridade clínica permanece com os IDs de fonte citados e estado de assinatura do revisor.
| ID | DIS-WILMS |
|---|---|
| Tipo | Doença |
| Alegais | Wilms associado a Beckwith-WiedemannNefroblastomaTumor renal pediátricoWilms associados à WAGRTumor de WilmsПухлина Вільмса (нефробластома) |
| Estado | reviewed 2026-05-18 | pending_clinical_signoff |
| Doenças | Nenhuma declaração |
| Fontes | SRC-COG SRC-NCCN-PEDIATRIC-SARCOMA |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8960/3 |
|---|---|
| icd_o_3_topography | ["C64"] |
| icd_10 | C64 |
| who_classification | WHO Urinary and Male Genital Tumours 5th ed (2022): Nephroblastoma (favorable histology + anaplastic variant) |
Notas
Two major treatment philosophies: COG (North American) — upfront surgery + adjuvant chemo (± RT) based on stage + histology. SIOP (European) — pre-operative chemo (4-6 weeks) → surgery → adjuvant based on response + histology. Both achieve >90% OS in favorable- histology disease. Chemotherapy backbone: vincristine + actinomycin ± doxorubicin per stage / histology / risk group. Anaplastic histology requires intensified regimens (ifosfamide + carboplatin + etoposide). Cascade screening obligatory for syndromic carriers — abdominal US q3-4 months from birth to age 7 in Beckwith-Wiedemann / WAGR.
Usado por
Relevância terapêutica
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Doença
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Indicação
IND-BECKWITH-WIEDEMANN-CARRIER-INTENSIFIED- IND-BECKWITH-WIEDEMANN-CARRIER-INTENSIFIEDIND-BECKWITH-WIEDEMANN-CARRIER-SURVEILLANCE- IND-BECKWITH-WIEDEMANN-CARRIER-SURVEILLANCE
Sinal de alerta
RF-BECKWITH-WIEDEMANN-CONFIRMED-CARRIER- O paciente tem um diagnóstico molecular confirmado da síndrome de Beckwith-Wiedemann (BWS) — patogeni...RF-DICER1-CONFIRMED-CARRIER- Variante patogênica / patogênica provável confirmada da linha germinal no DICER1 (RNase III endonuclea...
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...