SIOP

Source: Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: April 15, 2025 · Captured 2026-09-09.

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Context: Wilms Tumor / Treatment of Wilms Tumor / Treatment of stage IV Wilms tumor / Liver metastases at diagnosis

The SIOP/Gesellschaft für Pädiatrische Hämatologie und Onkologie (GPOH) group has recommended a more aggressive surgical approach. This recommendation was based on the reported outcomes of 29 patients treated on the SIOP93-01/GPOH and SIOP2001/GPOH trials that enrolled 1,365 patients between 1994 and 2004. Two of the patients had diffuse anaplasia.[283]

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The 5-year OS rate was 62.6% for patients with hepatic metastases, whereas the OS rate was 76.3% for all patients with stage IV disease. Within the group of patients with liver metastases, 14 did not undergo liver resections. Four of the patients (13%) responded to chemotherapy and were still alive at the time of the publication.

Fifteen patients underwent liver surgery (11 primary liver resections, 6 complete resections, and 5 incomplete resections). All six patients who had a complete resection survived.

As a result of these findings, the authors recommended an aggressive initial surgical approach for patients with liver disease. However, it is important to remember that SIOP patients receive preoperative chemotherapy before nephrectomy and/or resection of metastatic lesions.

The French cohort enrolled on the SIOP2001 trial included 131 patients with stage IV FH Wilms tumor. Of these patients, 18 (14%) had liver metastases at diagnosis, including 4 (3%) with isolated liver metastases.[286]

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The 5-year EFS rate was 83%, and the OS rate was 88% for this cohort of patients with liver metastases.

The authors concluded that liver involvement does not appear to be an adverse prognostic marker in patients with metastatic Wilms tumors, although there was a small number of patients in this study.

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