Wilms Tumor Ppt New Access

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:

    1. Favorable histology – triphasic; excellent prognosis.
    2. Diffuse anaplasia – large, hyperchromatic nuclei, atypical mitoses; high risk.
    3. Focal anaplasia – intermediate.
    4. New entity: Cystic partially differentiated nephroblastoma (benign behavior; no chemo).
    5. 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"

wilms tumor ppt new