A 7 year-old girl presented with an abdominal mass in the epigastrium associated with abdominal pain, nausea, vomiting and fever. Laboratory findings showed slight anemia and liver dysfunction, but serum alpha-fetorotein and total bilirubin levels were within the normal range. Suddenly she had developed shock when the tumor ruptured into the peritoneal cavity. After blood transfusion she underwent a left hepatic lobectomy with dissection of the lymphnodes. The tumor was composed predominantly of necrotic tissue, blood and gelatinous matter, and was pseudocapsulated by fibrous connective tissues. It was histologically diagnosed as undifferentiated (Embryonal) Sarcoma. The post operative course had been satisfactory until three months later, when distension of the abdomen appeared. She died of cachexia 6 months after the onset of symptoms. Autopsy showed recurrence (probably implantation) of the tumor associated with hemorrhagic ascitet (about 3,200ml) but there was no metastatic lesions. Thirty-one cases reported by Stocker and Ishak, and nine cases reported in the Japanese literature were compared in this report.
