Tumor de Wilms (nefroblastoma)
Vista determinista de la entidad fuente YAML. La autoridad clínica sigue con los ID de fuente citados y estado de firma del revisor.
| ID | DIS-WILMS |
|---|---|
| Tipo | Enfermedad |
| Aliaces | Wilms asociada a Beckwith-WiedemannNefroblastomaTumor de riñón pediátricoWilms asociados a la WAGRTumor de WilmsПухлина Вільмса (нефробластома) |
| Situación | reviewed 2026-05-18 | pending_clinical_signoff |
| Enfermedades | Ninguna declarada |
| Fuentes | 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.
Utilizado por
Relevancia terapéutica
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Enfermedad
DIS-ATRT- Atípico teratoide/tumor rabdoide (ATRT)DIS-CYSTIC-NEPHROMA- Nefroma císticaDIS-HEPATOBLASTOMA- HepatoblastomaDIS-NEUROBLASTOMA- Neuroblastoma
Indicación
IND-BECKWITH-WIEDEMANN-CARRIER-INTENSIFIED- IND-BECKWITH-WIEDEMANN-CARRIER-INTENSIFIEDIND-BECKWITH-WIEDEMANN-CARRIER-SURVEILLANCE- IND-BECKWITH-WIEDEMANN-CARRIER-SURVEILLANCE
Señal de alerta
RF-BECKWITH-WIEDEMANN-CONFIRMED-CARRIER- El paciente tiene un diagnóstico molecular confirmado del síndrome de Beckwith-Wiedemann (BWS) - patogeni...RF-DICER1-CONFIRMED-CARRIER- Variante patógena/potible patogénica confirmada de la línea germinal en DICER1 (endónuclea de RNase III...
Fuentes
SRC-BRIOUDE-BECKWITH-WIEDEMANN-2018- Documento de consenso de expertos: Diagnóstico clínico y molecular,...SRC-KALISH-BECKWITH-WIEDEMANN-2017- Recomendaciones de vigilancia para niños con síndromes de sobrecrecimiento y predisposición a...