Changes to This Summary (08/15/2014)
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.
Revised text to state that between 1975 and 2010, childhood cancer mortality decreased by more than 50%, and added text to state that for children younger than 15 years with Wilms tumor, the 5-year survival rate has increased over the same time from 74% to 88% (cited Smith et al. as reference 3).
Revised text to state that newborns born with sporadic aniridia should undergo molecular testing for deletion analysis of PAX6 and WT1; if a deletion of WT1 is observed, the child should be screened with ultrasound every 3 months until age 8 years, and the parents should be educated about the need to identify and treat early Wilms tumor.
Revised text about tests and procedures used to evaluate and stage Wilms tumor and other childhood kidney tumors to state that in children with a renal mass that clinically appears to be stage I or stage II Wilms tumor, biopsy is not performed so that tumor cells are not spread during the biopsy, and that a biopsy would upstage such a patient to stage III. Also added text to state that biopsy of a renal mass may be indicated if the mass is atypical by radiographic appearance for Wilms tumor, and the patient is not going to undergo immediate nephrectomy. Also added that lymph node sampling is required to stage all Wilms tumor patients, except those with stage 5.
Added Smith et al. as reference 96.
Added text to state that in stage I Wilms tumor, all lymph nodes sampled are negative.
Added text to state that in stage II Wilms tumor, all lymph nodes sampled are negative.
Added text to state that the surgeon needs to be aware of the risk of intraoperative spill, especially in patients who have right-sided and large tumors (cited Gow et al. as reference 129).
Added text to state that renal-sparing surgery does not appear to be feasible in most patients at the time of diagnosis because of the location of the tumor within the kidney, even in those with very low risk (cited Ferrer et al. as reference 133).
Added text to state that in North America, the use of preoperative chemotherapy in patients with evidence of a contained preoperative rupture has been suggested to avoid intraoperative spill, but this is controversial (cited Rutigliano et al. as reference 150). Also added that the preoperative diagnosis of a contained retroperitoneal rupture on CT is difficult, even for experienced pediatric radiologists, and that the routine use of preoperative chemotherapy would lead to overtreatment in a significant number of these children.
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.