Title Slide
Wilms Tumor (Nephroblastoma) An Embryonal Renal Malignancy of Childhood
Answer per new guidelines: Stage III (tumor thrombus) + molecular high-risk → intensify to Regimen DD-4A + flank radiation (19.8 Gy) despite favorable histology.
Slide 4: Clinical Presentation
- Classic Triad (Abdominal Mass):
- Preoperative chemo (SIOP) reduces surgical complications; immediate nephrectomy (COG) provides pristine staging.
- New high-risk markers: 1q gain, TP53 mutation, blastemal type post-chemo.
- Relapse is not hopeless – incorporate B7-H3 or checkpoint inhibitors in trials.
- Reduce doxorubicin in infants; use proton therapy.
- Every survivor gets cardiac and renal surveillance.
Highlighting associated genetic syndromes adds scientific depth to your presentation:
- Favorable histology – triphasic; excellent prognosis.
- Diffuse anaplasia – large, hyperchromatic nuclei, atypical mitoses; high risk.
- Focal anaplasia – intermediate.
- New entity: Cystic partially differentiated nephroblastoma (benign behavior; no chemo).
- New: Blastemal-predominant subtype after SIOP chemo – poor prognosis regardless of stage → upgrade to high-risk therapy.
The "Rule of 10s": A highly effective mnemonic for presentations, noting that roughly 10% of cases are bilateral, 10% have unfavorable histology, 10% show vascular invasion, and 10% present with pulmonary metastases.
Secondary Malignancies: Risk from prior radiation or alkylating agents.
Radiotherapy: Reserved for Stage III and IV patients or those with unfavorable histology. 6. Recent Advances & "What's New"