Undifferentiated sarcoma of the liver in childhood: a curable disease.

Abstract BACKGROUND: Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare childhood hepatic tumor, and it is generally considered an aggressive neoplasm with an unfavorable prognosis. METHODS: The Soft Tissue Sarcoma Italian and German Cooperative Groups enrolled 17 children with UESL in studies conducted between 1979 and 1995. They were treated using the same multimodal approach as for patients with sarcomas including conservative surgery at diagnosis, multiagent chemotherapy, and second-look operation in cases of residual disease. Radiotherapy was occasionally used (2 of 17 patients). RESULTS: Twelve patients are alive with follow-up ranging from 2.4 to 20 years. Eight underwent complete tumor resection either at diagnosis or after preoperative chemotherapy, and all are currently alive. After initial chemotherapy tumor reduction was evident in six of nine evaluable cases. Overall three patients died of disease and one of a surgical complication. One child died in second complete remission for a non-disease-related cause. CONCLUSIONS: The current prognosis of UESL no longer should be regarded as poor. Modern multimodal treatment and supportive therapy have improved survival. Copyright 2002 American Cancer Society.