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

Purpose: This study was undertaken to review the authors' clinical experien ce 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 treatm ent 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 la rge 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 t umor recurrence at 12 and 29 months; both of these patients died of their d isease. Another patient died of complications related to chemotherapy. The other 4 patients are alive with no evidence of disease after 19 to 150 mont hs' follow-up. Conclusions: Undifferentiated embryonal sarcoma of the liver presents as a large hepatic tumor. Operative resection is difficult, but combined with ad juvant chemotherapy offers the best hope for cure. Copyright (C) 1999 by W. B. Saunders Company.