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Essential Features

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  • • 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

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Epidemiology

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  • • 6.9% of cancers diagnosed before age 15

    • Incidence highest in blacks

    • Peak incidence between ages 2 and 3

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Clinical Findings

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Symptoms and Signs

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  • • Abdominal mass

    • Pain

    • Fever

    • Hematuria

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Imaging Findings

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

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Diagnostic Considerations

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  • • Stage, bilateral disease, major vessel involvement

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Work-up

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  • • History and physical exam

    • Abdominal CT scan

    • Chest x-ray

    • Doppler US when concern of major vessel involvement

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Treatment and Management

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Surgery

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  • • 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)

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Medications

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  • • 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

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Complications

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  • • Damage to major neurovascular structures

    • Recurrence

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Prognosis

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

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Resources

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References

Capra ML et al. Wilms' tumor: a 25-year review of the role of preoperative chemotherapy. J Pediatr Surg. 1999;34:579.  [PubMed: 10235327]
Haase GM, Ritchey ML. Nephroblastoma. Semin Pediatr Surg. 1997;6:11.  [PubMed: 9117268]

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