Tumeur de Wilms (néphroblastome)
Vue déterministe de l'entité source de la LJAM. L'autorité clinique demeure avec les ID de la source cités et l'état d'approbation de l ́examinateur.
| ID | DIS-WILMS |
|---|---|
| Type | Maladie |
| Alias | Wilms associé à Beckwith-WiedemannNephroblastomeTumeur rénale pédiatriqueWilms associés à la WAGRTumeur WilmsПухлина Вільмса (нефробластома) |
| État | reviewed 2026-05-18 | pending_clinical_signoff |
| Maladies | Aucune déclaration |
| Sources | 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) |
Notes
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.
Utilisé par
Pertinence thérapeutique
BMA-WT1-GERMLINE-WILMS- WT1 germline pathogenic variants drive a spectrum of pediatric kidney predispositions: WA...
Maladie
DIS-ATRT- Téréatoïde atypique/tume rhabdoïde (ATRT)DIS-CYSTIC-NEPHROMA- Néphrome kystiqueDIS-HEPATOBLASTOMA- HépatoblastomeDIS-NEUROBLASTOMA- Neuroblastome
Indication
IND-BECKWITH-WIEDEMANN-CARRIER-INTENSIFIED- IND-BECKWITH-WIEDEMANN-CARRIER-INTENSIFIEDIND-BECKWITH-WIEDEMANN-CARRIER-SURVEILLANCE- IND-BECKWITH-WIEDEMANN-CARRIER-SURVEILLANCE
Signal d’alerte
RF-BECKWITH-WIEDEMANN-CONFIRMED-CARRIER- Le patient a un diagnostic moléculaire confirmé du syndrome de Beckwith-Wiedemann (BWS) — pathogeni...RF-DICER1-CONFIRMED-CARRIER- Variante pathogène/pathogène probable confirmée chez DICER1 (endonucléa...
Sources
SRC-BRIOUDE-BECKWITH-WIEDEMANN-2018- Document de consensus d'experts : Diagnostic clinique et moléculaire, dépistage et gestion...SRC-KALISH-BECKWITH-WIEDEMANN-2017- Recommandations de surveillance pour les enfants atteints de syndromes de surcroissance et de prédisposition...