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Key Features

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  • Approximately 460 new cases occur each year in the United States, representing 5–6% of cancers in children younger than 15 years

  • Second most common abdominal tumor in children after neuroblastoma

  • Occurrence is sporadic

  • Median age at diagnosis is related both to gender and laterality

    • Diagnosed earlier in males than females

    • Bilateral tumors present at a younger age than unilateral tumors

  • Occurs most commonly between ages 2 and 5 years; it is unusual after age 6 years

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Clinical Findings

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  • Increasing abdominal size is presenting symptom in most children

  • Abdominal mass can be asymptomatic and discovered incidentally by a parent or health care provider

  • Mass is usually smooth and firm, well demarcated, and rarely crosses the midline, though it can extend inferiorly into the pelvis

  • About 25% of patients are hypertensive at presentation

  • Gross hematuria is an uncommon presentation, although microscopic hematuria occurs in approximately 25% of patients

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Diagnosis

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  • Complete blood cell count is usually normal; some patients have anemia secondary to hemorrhage into the tumor

  • Blood urea nitrogen and serum creatinine are usually normal

  • Urinalysis may show some blood or leukocytes

  • Ultrasonography or CT of the abdomen

    • Should establish the presence of an intrarenal mass

    • Contralateral kidney should be assessed for presence and function as well as synchronous Wilms tumor

  • Ultrasonography with Doppler flow should be done to evaluate the inferior vena cava for the presence and extent of tumor propagation

  • The liver should be imaged for the presence of metastatic disease

  • Chest CT scan should be obtained to determine whether pulmonary metastases are present

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Treatment

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  • First step is surgical exploration of the abdomen via an anterior surgical approach

    • Allows for inspection and palpation of the contralateral kidney

    • Liver and lymph nodes are inspected and suspicious areas biopsied or excised

    • En bloc resection of the tumor is performed

  • Patients with stage III or IV Wilms tumor

    • Require radiation therapy to the tumor bed and to sites of metastatic disease

    • Chemotherapy is optimally begun within 5 days after surgery

    • Radiation therapy should be started within 10 days

  • Stage V (bilateral Wilms tumor) disease

    • Possible bilateral renal biopsies followed by chemotherapy and second-look renal-sparing surgery

    • Radiation therapy may also be necessary

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