Wilms tumor (nephroblastoma)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-WILMS |
|---|---|
| Type | Disease |
| Aliases | Beckwith-Wiedemann-associated WilmsNephroblastomaPediatric kidney tumorWAGR-associated WilmsWilms tumorПухлина Вільмса (нефробластома) |
| Status | reviewed 2026-05-18 | pending_clinical_signoff |
| Diseases | None declared |
| 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.
Used By
Actionability
BMA-WT1-GERMLINE-WILMS- WT1 germline pathogenic variants drive a spectrum of pediatric kidney predispositions: WA...
Disease
DIS-ATRT- Atypical teratoid/rhabdoid tumor (ATRT)DIS-CYSTIC-NEPHROMA- Cystic nephromaDIS-HEPATOBLASTOMA- HepatoblastomaDIS-NEUROBLASTOMA- Neuroblastoma
Indication
IND-BECKWITH-WIEDEMANN-CARRIER-INTENSIFIED- IND-BECKWITH-WIEDEMANN-CARRIER-INTENSIFIEDIND-BECKWITH-WIEDEMANN-CARRIER-SURVEILLANCE- IND-BECKWITH-WIEDEMANN-CARRIER-SURVEILLANCE
Red flag
RF-BECKWITH-WIEDEMANN-CONFIRMED-CARRIER- Patient has a confirmed Beckwith-Wiedemann syndrome (BWS) molecular diagnosis — pathogeni...RF-DICER1-CONFIRMED-CARRIER- Confirmed germline pathogenic / likely-pathogenic variant in DICER1 (RNase III endonuclea...
Sources
SRC-BRIOUDE-BECKWITH-WIEDEMANN-2018- Expert consensus document: Clinical and molecular diagnosis, screening and management of...SRC-KALISH-BECKWITH-WIEDEMANN-2017- Surveillance Recommendations for Children with Overgrowth Syndromes and Predisposition to...