Progress in treatment of bilateral Wilms'tumor / 中国肿瘤临床

ABSTRACT

Bilateral Wilms'tumor (BWT) affects approximately 5% of children with WT. The goal of treatment in BWT is to preserve normal renal function as much as possible, in order to postpone and reduce the risk of end-stage renal disease and other long-term complications. For neoadjuvant chemotherapy, vincristine/dactinomycin/doxorubicin is used in cases with favorable histology. Cases with diffuse anaplasia or other unfavorable histology receive an intensified regimen, including additional agents such as ifosfamide, cy-clophosphamide, carboplatin, and others. Nephron-sparing surgery is performed to preserve long-term renal function. Radiotherapy is given according to local staging, when margins or lymph nodes are positive, or tumor spillage has occurred, or pathological type is ana-plasia. The survival rate of relapsed BWT is low. Some experts suggest that patients with recurrent BWT are most likely to benefit from high-dose therapy including stem-cell transplantation, but this remains controversial.