•Treatment according to staging (see above) aims to eliminate the nephroblastoma while avoiding short-term or long-term side effects/complications

•Primary surgical resection or preoperative chemotherapy (mostly with marked tumor regression and reduction of intraoperative tumor rupture) has to be considered according to staging

•Biopsy: only in children with unclear presentation or diagnosis

•After biopsy, stage I is excluded

•Primary surgery in infants less than 6 months old and in adolescents more than 16 years of age

11.10.1 Surgical Procedures

• Transabdominal tumor resection with abdominal exploration including liver and contralateral kidney

• Biopsy of suspected tissue, especially lymph nodes

• Where there is large tumor mass, preoperative chemotherapy, probably radiotherapy (see below)

11.10.2 Chemotherapy

•Preoperative chemotherapy: vincristine and actinomycin D; in children with primary metastases, addition of anthracycline

•Postoperative chemotherapy: duration and drug combination according to stage and histology

•Toxicity: veno-occlusive disease (VOD) of the liver, especially in infants and small children

11.10.3Radiotherapy

•Nephroblastoma is radiosensitive

•Due to combined chemotherapy, irradiation is indicated only in high-risk patients

•Stage II with lymph node involvement, highly malignant, metastatic spread

•Start of radiotherapy within the first 10 postoperative days

•Dose: 15–30 Gy, with higher doses to the tumor bed and remaining tumors

11.11Therapy in Relapse

Relapse in about 15% of children with favorable, and in 50% with anaplastic histology

•Favorable prognosis in children with relapse 6 months after tumor resection, in nonirradiated areas with one organ system involvement only, without lymph node metastases, excluding highly malignant variants; chemotherapy with combination of vincristine, actinomycin D, doxorubicin, ifosfamide, carboplatin, and etoposide

•Relapse with poor prognosis: high-dose chemotherapy with autologous stem cell transplantation