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Wilms’ Tumor

(Nephroblastoma)

by David N. Korones, MD

Wilms’ tumor is an embryonal cancer of the kidney composed of blastemal, stromal, and epithelial elements. Genetic abnormalities have been implicated in the pathogenesis, but familial inheritance accounts for only 1 to 2% of cases. Diagnosis is by ultrasonography and abdominal CT and is confirmed by biopsy. Treatment may include surgical resection, chemotherapy, and radiation therapy.

Wilms’ tumor usually manifests in children < 5 yr but occasionally in older children and rarely in adults. Wilms’ tumor accounts for about 6% of cancers in children < 15 yr. Bilateral synchronous tumors occur in about 5% of patients; bilateral disease is more common among very young children, especially girls.

A chromosomal deletion of WT1, the Wilms’ tumor suppressor gene, has been identified in some cases. Other associated genetic abnormalities include deletion of WT2 (a 2nd Wilms’ tumor suppressor gene), deletion of chromosome 16, and duplication of chromosome 12.

About 10% of cases manifest with other congenital abnormalities, especially GU abnormalities, but also commonly hemihypertrophy (asymmetry of the body). WAGR syndrome is the combination of Wilms’ tumor (with WT1 deletion), aniridia, GU malformations (eg, renal hypoplasia, cystic disease, hypospadias, cryptorchidism), and mental retardation (intellectual disablity).

Symptoms and Signs

The most frequent finding is a painless, palpable abdominal mass. Less frequent findings include abdominal pain, hematuria, fever, anorexia, nausea, and vomiting. Hematuria (occurring in 15 to 20%) indicates invasion of the collecting system. Hypertension may occur if compression of the renal pedicle or renal parenchyma causes ischemia.

Diagnosis

  • Abdominal ultrasonography and CT

  • Biopsy

Abdominal ultrasonography determines whether the mass is cystic or solid and whether the renal vein or vena cava is involved. Abdominal CT is needed to determine the extent of the tumor and check for spread to regional lymph nodes, the contralateral kidney, or liver. The diagnosis is confirmed by biopsy of the mass. Renal arteriography, vena cavography, retrograde urography, or excretory urography is seldom required. Chest CT is recommended to detect metastatic pulmonary involvement at initial diagnosis.

Prognosis

Prognosis depends on

  • Histology (favorable or unfavorable)

  • Stage at diagnosis

  • Patient’s age (younger is better)

The outcome for children with Wilms’ tumor is excellent. Cure rates for lower-stage disease (localized to the kidney) range from 85% to 95%. Even children with more advanced disease fare well; cure rates range from 60% (unfavorable histology) to 90% (favorable histology).

The cancer may recur, typically within 2 yr of diagnosis. Cure is possible in children with recurrent cancer. Outcome after recurrence is better for children who present initially with lower-stage disease, whose tumors recur at a site that has not been irradiated, who relapse > 1 yr after presentation, and who receive less intensive treatment initially.

Treatment

  • Surgery and chemotherapy

  • Sometimes radiation therapy

The National Wilms’ Tumor Study Group has established staging criteria and guidelines for treatment. Prompt surgical exploration of potentially resectable lesions is indicated, with examination of the contralateral kidney. If the cancer is unilateral and limited to the kidney or if extension is minimal, complete resection by nephrectomy is done, followed by treatment with vincristine and actinomycin D. If a unilateral cancer has spread extensively or if the disease is bilateral, chemotherapy with actinomycin D and vincristine, with or without radiation therapy, is used. Children with more advanced disease also receive doxorubicin. Other frequently used drugs include cyclophosphamide, ifosfamide, and etoposide.

Children with very large nonresectable tumors or bilateral tumors are candidates for chemotherapy followed by reevaluation and possibly resection.

Drugs Mentioned In This Article

  • Drug Name
    Select Trade
  • CYTOXAN (LYOPHILIZED)
  • IFEX
  • MARQIBO KIT
  • ETOPOPHOS

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