Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (08/10/2017)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Wilms Tumor

Added text to state that in patients with congenital anomalies and Wilms tumor, nephrogenic rests have been reported in 60% of cases (cited Vujanić et al. as reference 5).

Added text to state that preoperative chemotherapy does not appear to affect the overall prevalence of nephrogenic rests. Congenital anomalies have been reported in 12% of patients with nephrogenic rests, including in 9% of patients with unilateral Wilms tumor and in 33% of patients with bilateral disease.

Added text to state that intralobar nephrogenic rests have been associated with stromal-type Wilms tumor and younger age at diagnosis.

Revised Table 3 to update survival data and treatment options for patients younger than 24 months with favorable histology (FH), stage I Wilms tumor (cited Fernandez et al. as reference 196).

Added text to state that the Children's Oncology Group validated the hypothesis that nephrectomy only is appropriate therapy for patients younger than 2 years at diagnosis with stage I, FH Wilms tumor that weighed less than 550 g in the AREN0532 (NCT00352534) trial.

Added text about the results of the AREN0532 trial for patients younger than 2 years at diagnosis with stage I, FH Wilms tumor that weighed less than 550 g.

Renal Cell Carcinoma (RCC)

Added Ezekian et al. as reference 15.

Added text about the results of a study that evaluated 304 children and adolescents with RCC who were identified in the National Cancer Data Base (cited Rialon et al. as reference 41).

Added text to state that disease regression and improvement in symptoms have been reported with the use of cabozantinib in pediatric patients with translocation-positive RCC expressing MET (cited Wedekind et al. as reference 48).

Congenital Mesoblastic Nephroma

This section was extensively revised.

Multilocular Cystic Nephroma

Added text to state that anaplastic sarcoma of the kidney has also been associated with the DICER1 mutation (cited Wu et al. as reference 1).

Treatment of Recurrent Childhood Kidney Tumors

Added text to state that the most common site of relapse is lung, followed by abdomen/flank and liver. Recurrence in the brain is rare in children with Wilms tumor (cited Venkatramani et al. as reference 2).

Added text about the APEC1621 (NCT03155620) trial as a treatment option under clinical evaluation for recurrent Wilms tumor.

Added Treatment options under clinical evaluation for recurrent clear cell sarcoma of the kidney as a new subsection.

Added Treatment of Recurrent Congenital Mesoblastic Nephroma as a new subsection.

Special Considerations for the Treatment of Children With Cancer

Added this new section.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)—Health Professional Version - National Cancer Institute