Case 1; A 5-year-old male, who had complained of abdominal pain, came to our hospital. Echogram demonstrated a tumor in the right hepatic lobe, in which multiple cystic areas were visualized. Celiac arteriogram disclosed tumor vessels with central hypovascularity distal to the right hepatic artery. AFP was negative. He underwent right hepatic lobectomy. Standard VAC therapy was added postoperatively for 12 weeks, followed by cyclophosphamide given orally for another 1 year. He is doing well without recurrence at the time of follow-up of 35 months postoperatively. Case 2; An 8-year-old male was admitted, suffering from severe abdominal pain and distension due to a large hepatic tumor in the right lobe and left medial segment. Hematological data showed liver dysfunction and negative AFP. Emergency laparotomy revealed the tumor invasion to the diaphragm. He underwent right hepatic trisegmentectomy. CCA (cis-platinum, cyclophosphamide, adriamycin) therapy was started on the 10th postoperative day every 3 months. There is no evidence of recurrence 17 months after surgical intervention. Pathological diagnosis was undifferentiated sarcoma of the liver in both cases, with positive Sudan III in case 1, and positive α_1-antitrypsin and α_1-antichymotrypsin in both. We reviewed 19 pediatric and 3 adult cases reported in Japan. Among the 5 pediatric survivors, 4 have survived over 2 years without recurrence. Survival of 17 months without recurrence in case 2 is the longest among 4 cases with tumor ruptured at the primary operation, while tumor-free period is only 2 〜 8 months in the others. This review suggests that CCA therapy is the most effective for the non-resectable undifferentiated sarcoma of the liver.
