Undifferentiated embryonal sarcoma is the third most common malignant tumor of the liver in children, accounting for 13% of hepatic malignancies in this age group. It has been considered an aggressive neoplasm with very poor prognosis until the late 1980s, when long-term survivors were reported after multiagent chemotherapy followed by resection. We, herein, report two pediatric cases of undifferentiated embryonal sarcoma treated successfully with surgical resection after neoadjuvant chemotherapy based on therapy used in childhood soft tissue sarcomas and in childhood hepatic malignancies.
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