Undifferentiated (embryonal) sarcoma of the liver in childhood. The experience of the Italian Cooperative group on Pediatric Soft Tissue Sarcoma

Introduction The undifferentiated (embryonal) sarcoma of the liver (USL) is a rare mesenchymal tumor that occurs mainly in children benveen 5 and 10 years. Prognosis is reputed poor because radical surgery is difficult and the activity of chemotherapy is not well documented. Methods Ten children with USL have been enrolled in the protocols ran by the Italian Cooperative Group from 1979 to 1995. Treatment was similar to the one used for children with rhabdomyosarcoma and provided a conservative radical surgery at diagnosis or after preoperative chemotherapy and eventually radiotherapy. Results Surgery was radical in 5 patients: at diagnosis (one case) or after chemotherapy. Response to initial chemotherapy was evident in 6 out of 7 évaluable cases. Six patients are alive without evidence of disease 5 to 12 years from diagnosis. One child died for surgical complication and ftvo because of the tumor. One more patient is lost to follow up. Conclusions Our results confirm the crucial role of radical resection of the tumor. Chemotherapy may be administered to reduce the tumor size making the mass resection feasible. Improvement in surgical techniques and administration of aggressive chemotherapy, similar to that used for rhabdomyosarcom, has improved the prognosis for these patients.