Wilms and Other Pediatric Renal Tumors
Wilms tumor or nephroblastoma is the most common pediatric abdominal, malignant tumor, representing about 5–6% of all childhood cancer.
The North American approach to these tumors involves initial nephrectomy followed by chemotherapy with or without radiotherapy (RT). In Europe, preoperative chemotherapy followed by surgery and postoperative chemotherapy with or without RT is the usual sequence of treatment. Despite the difference between the two approaches, survival for Wilms tumor is excellent for children in North America and Europe with more than 90% alive at 5 years . Most of these patients will have favorable histology subtype with only 10% having anaplastic Wilms tumor.
In addition to histology, loss of heterozygosity (LOH) in 1p and 16q has been associated with worse outcome . Other renal tumors such as clear cell sarcoma and rhabdoid tumor have been included in studies from the National Wilms Tumor Study (NWTS) and Children’s Oncology Group (COG), but they are not subtypes of Wilms tumor.
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