Undifferentiated Embryonal Sarcoma of the Liver : Report of an Infantile Case and a Review of 48 Cases Reported in Japan

Abstract

A 5-year-old boy was admitted to our hospital because of abdominal distention for a month. Ultrasonography and magnetic response imaging (MRI) revealed a large cystic tumor which occupied the entire right lobe of the liver. Slight elevation of plasma CA125, NSE, IAP and CA72-4 were found though the AFP level stayed in the normal range. Complete resection of the tumor was achived by right hepatic lobectomy. The tumor was 19×19×19 cm in size and weighed 2, 400 gm. The tumor was composed of solid and cystic mass containing clear yellowish fluid, and histological examination revealed immature tumor cells with the diagnosis of undifferentiated embryonal sarcoma of the liver. The postoperative course was uneventful without recurrence for 24 months after the operation. Undifferentiated embryonal sarcoma of the liver is a rare tumor in children, and the present case was reviewed toghether with other 48 reported cases in children in Japan. Ultrasonography and CT scan showed a cystic mass in liver. It is important to attempt to diagnose this disease. It should be stressed that cystic lesions of the liver may account for this rare condition of the liver.