Treatment of Wilms Tumour: The SIOP Approach

Chapter
Part of the Pediatric Oncology book series (PEDIATRICO)

Abstract

Retrospective studies before 1950 showed a remarkable gain of survival at institutions with structured diagnostic and perioperative care. Irradiation pre-, intra- and postoperatively as well as chemotherapy were used, and pretreatment prior to surgery was widely accepted. However, the optimum timing of nephrectomy was the subject of lively debate between institutions. The improving imaging modalities and thus reliable radiologic diagnosis of nephroblastoma prepared the ground for systematic preoperative treatment. SIOP’s nephroblastoma studies starting from 1971 proved the decrease of ruptures and stage III tumours by preoperative irradiation. After demonstrating the equality of preoperative dactinomycin- and vincristine-based chemotherapy with irradiation in the following study, postoperative treatment burden was reduced from trial to trial and adjusted to the individual risk. With an overall survival reaching 81.5 % in the late 1970s during SIOP 5, the subsequent trials were focusing on reducing side effects and long-term sequelae whilst maintaining survival. Nowadays less than 20 % of patients with a localised WT who are treated according to SIOP-2001 protocol receive irradiation. Thirty-seven percent of all patients with localised WT can be cured with a 4-week pre- and 4-week post-nephrectomy dactinomycin and vincristine course only. The 5-year EFS and OS for all patients with localised nephroblastoma reach 87 and 94 %, respectively. However, patients having a local stage III, metastasis or high-risk histology WT need intensified treatment including additional doxorubicin and often etoposide, cyclophosphamide and carboplatin.

WT and nephroblastomatosis can also occur bilaterally or in combination. Under these circumstances WT patients must be treated in a multidisciplinary, chemo-response-dependent manner, aiming to preserve renal parenchyma as much as reasonably possible.

Keywords

Toxicity Europe Sarcoma Assure Stratification 

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Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Specialist in Pediatric Oncology and HematologySaarland University Hospital, Pediatric Hematology and OncologyHomburg, SaarlandGermany
  2. 2.Department of Developmental Biology and Cancer, Institute of Child HealthUniversity College LondonLondonUK

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