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Explore: Usually starts with immediate nephrectomy followed by chemotherapy.
For more in-depth study, you can explore these specific presentations:
: Often called nephroblastoma; it arises from immature kidney cells.
Overgrowth disorder (macroglossia, hemihypertrophy) with an increased risk of embryonal tumors. Clinical Presentation
| Gene | Location | Function | Associated Condition | |------|----------|----------|----------------------| | WT1 | 11p13 | Transcription factor | WAGR, Denys-Drash | | WT2 (IGF2) | 11p15 | Growth regulation | Beckwith-Wiedemann | | CTNNB1 | 3p22 | Wnt signaling | Sporadic Wilms | | TP53 | 17p13 | Tumor suppressor | Anaplastic histology |
: Associated with chromosome 11 deletions (WT1, WT2) and syndromes like WAGR , Beckwith-Wiedemann , and Denys-Drash .
| Risk Group | Agents | Duration | |------------|--------|----------| | Low risk (Stage I, FH) | Vincristine + Dactinomycin | 4 weeks | | Standard risk (Stage II-IV, FH) | Add Doxorubicin | 24 weeks | | High risk (Anaplastic) | Add Cyclophosphamide, Etoposide, Carboplatin | 24-34 weeks |