Undifferentiated embryonal sarcoma of the liver: results of clinical management in one center

PURPOSE: This study was undertaken to review the authors' clinical experience with undifferentiated embryonal sarcoma of the liver (UES) in children, focusing on the clinical presentation and results of treatment. METHODS: A retrospective analysis of all children who have undergone treatment for UES during the 15-year period from 1984 through 1998 was performed. RESULTS: Seven patients (4 boys and 3 girls) ranging in age from 20 months to 12 years at the time of diagnosis were identified. All presented with large abdominal masses and normal liver function test results. All underwent complete tumor resection; trisegmentectomy was required in 4 of these cases. All patients received postoperative chemotherapy. Two patients suffered tumor recurrence at 12 and 29 months; both of these patients died of their disease. Another patient died of complications related to chemotherapy. The other 4 patients are alive with no evidence of disease after 19 to 150 months' follow-up. CONCLUSIONS: Undifferentiated embryonal sarcoma of the liver presents as a large hepatic tumor. Operative resection is difficult, but combined with adjuvant chemotherapy offers the best hope for cure.