PURPOSE: Remarkable progress could be achieved in the treatment of nephroblastoma within the last decades. In all children with Wilms' tumor, 5-year overall survival rate reaches more than 90% in the SIOP/GPOH Study Group. Despite this fact, there is a small group of patients who have tumor lesions in the liver primarily representing a challenge in treatment. Data of this group are analyzed. METHODS: To define survival and success of treatment in this group of patients, we reviewed the records of 29 of 1365 patients enrolled in the SIOP 93-01/GPOH Study and the SIOP 2001/GPOH Study between April 1, 1994, and September 30, 2004. RESULTS: Median age at diagnosis was 10.61 years (range, 0.19-34.16 years). All patients but two underwent nephrectomy. Liver metastases were operated in 11 children at time of nephrectomy, in 4 patients secondarily, whereas in 11 patients, liver lesions were treated alone with chemotherapy and radiotherapy, respectively. In 3 children, no treatment could be initiated. Sixteen patients received radiotherapy additionally. Median follow-up was 64 months (range, 3-157 months). Eleven patients died in the course at a median of 13.07 months (range, 0.25-42 months) after initial diagnosis. These included 7 patients who never had surgery for their liver lesions and 4 patients who had incomplete and/or atypical resections of their metastatic liver lesions. All patients who underwent complete resection of hepatic metastases (n = 9) survived. Eight children survived with a nonsurgical treatment. Overall survival was below 60% in the whole group up to now. CONCLUSIONS: Liver metastases are much less frequent than metastases to other sites. Our report suggests that Wilms' tumor complicated by metastases of the liver primarily has a less favorable outcome. Chemotherapy and radiotherapy play an important role in treatment. Radical surgery for nephrectomy as well as surgery of liver lesions cannot be overemphasized to prevent local and distant recurrence.
PURPOSE: Remarkable progress could be achieved in the treatment of nephroblastoma within the last decades. In all children with Wilms' tumor, 5-year overall survival rate reaches more than 90% in the SIOP/GPOH Study Group. Despite this fact, there is a small group of patients who have tumor lesions in the liver primarily representing a challenge in treatment. Data of this group are analyzed. METHODS: To define survival and success of treatment in this group of patients, we reviewed the records of 29 of 1365 patients enrolled in the SIOP 93-01/GPOH Study and the SIOP 2001/GPOH Study between April 1, 1994, and September 30, 2004. RESULTS: Median age at diagnosis was 10.61 years (range, 0.19-34.16 years). All patients but two underwent nephrectomy. Liver metastases were operated in 11 children at time of nephrectomy, in 4 patients secondarily, whereas in 11 patients, liver lesions were treated alone with chemotherapy and radiotherapy, respectively. In 3 children, no treatment could be initiated. Sixteen patients received radiotherapy additionally. Median follow-up was 64 months (range, 3-157 months). Eleven patients died in the course at a median of 13.07 months (range, 0.25-42 months) after initial diagnosis. These included 7 patients who never had surgery for their liver lesions and 4 patients who had incomplete and/or atypical resections of their metastatic liver lesions. All patients who underwent complete resection of hepatic metastases (n = 9) survived. Eight children survived with a nonsurgical treatment. Overall survival was below 60% in the whole group up to now. CONCLUSIONS: Liver metastases are much less frequent than metastases to other sites. Our report suggests that Wilms' tumor complicated by metastases of the liver primarily has a less favorable outcome. Chemotherapy and radiotherapy play an important role in treatment. Radical surgery for nephrectomy as well as surgery of liver lesions cannot be overemphasized to prevent local and distant recurrence.
Authors: Peter F Ehrlich; Fernando A Ferrer; Michael L Ritchey; James R Anderson; Daniel M Green; Paul E Grundy; Jeffrey S Dome; John A Kalapurakal; Elizabeth J Perlman; Robert C Shamberger Journal: Ann Surg Date: 2009-10 Impact factor: 12.969