Liver metastases at diagnosis

The liver is an infrequent site of metastases at diagnosis for patients with stage IV FH Wilms tumor, but it is the most common site after the lung. In 634 patients with stage IV FH Wilms tumor from the NWTS-4 and -5 studies, 96 (15%) presented with liver involvement.[239] In the AREN0533 study of 47 patients (14%) with FH Wilms tumor who presented with extrapulmonary metastases, there were 37 patients with isolated liver metastases and 10 patients with liver metastases in combination with other metastatic sites. Thirty-eight patients presented with lung and extrapulmonary sites. All patients were treated with regimen M and abdominal radiation therapy depending on local tumor stage. The 4-year EFS rate was 76%, and the OS rate was 89%. Only 2 patients had a resection of the liver metastases (both of whom also received liver radiation therapy). Of the patients who received liver radiation therapy (27 of 39), none relapsed in the liver.[279]

The impact of liver metastases at diagnosis on patient care management has not been studied in a prospective manner. Most reported experiences come from single institutions and/or retrospective studies that do not include more modern surgical approaches or contemporary risk stratification parameters. In aggregate, there are not sufficient data to support liver metastases as an unfavorable site for metastatic disease, although there were some early SIOP studies that conflicted with this finding.[239,282-286]