Wilms Tumor

• • Associated with:

  • -Beckwith-Wiedemann syndrome (macroglossia, gigantism, visceromegaly, hypoglycemia, abdominal wall defects)

    -WAGR syndrome (Wilms, aniridia, ambiguous genitalia, mental retardation)

    -Neurofibromatosis

    -Denys-Drash syndrome

    -Perlman familial nephroblastomatosis

    -Other genital abnormalities

  • 1-2% incidence of bilateral disease

  • As high as 7% multicentricity

Epidemiology

• • 6.9% of cancers diagnosed before age 15

  • Incidence highest in blacks

  • Peak incidence between ages 2 and 3

Symptoms and Signs

• • Abdominal mass

  • Pain

  • Fever

  • Hematuria

Imaging Findings

• • CT scan: Shows extent of disease in kidney and extent of distant or nodal disease

• • Stage, bilateral disease, major vessel involvement

• • History and physical exam

  • Abdominal CT scan

  • Chest x-ray

  • Doppler US when concern of major vessel involvement

Surgery

• • Resection of tumor with inspection of contralateral kidney and sampling of regional nodes

  • Resection performed after chemotherapy/radiation therapy if bilateral disease to spare renal function or in presence of major vessel involvement (inferior vena cava, renal veins, etc)

Medications

• • Chemotherapy (vincristine and actinomycin D with doxorubicin added for stage III and IV)

  • Radiation for stage III, pulmonary or hepatic metastases or all stages if unfavorable histology

Complications

• • Damage to major neurovascular structures

  • Recurrence

Prognosis

• • 4-year survival ranging from 78% to 97% (stage dependent)

References