Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy.

Background/purpose: Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignancy found in older children, and the most appropriate treatment strategy has been controversial. The authors report the tumor's clinical characteristics and the results of management in 6 children and recommend the best treatment plans based on these findings.

Methods: Six USL pathologically verified at Seoul National University Children's Hospital between 1990 and 2000 were reviewed retrospectively.

Results: The mean age at diagnosis of USL was 10.3 years (range, 7 to 13 years). There were 2 girls and 4 boys; 5 of 6 children presented with large abdominal mass, one with weight loss. Tumors ranged in size from 8.5 x 9.0 cm to 15.0 x 15.0 cm. Location of the tumor varied from right lobe in 4 to left lobe in one, and both lobes in one. Three children underwent primary complete resection without preoperative chemotherapy. Partial resection of the tumor was done in one child because of encasing inferior vena cava and hepatic vein. However, complete resection was possible at second-look operation after chemotherapy. Two children underwent complete resection after chemotherapy. Preoperative chemotherapy allowed successful resection of 3 USL, which were initially considered unresectable. Postoperative recovery for all children was uneventful. Chemotherapy according to the the Third Intergroup Rhabdomyosarcoma Study (IRS III) was administered to all except one child who refused further postoperative chemotherapy after having had severe complications during the first cycle of chemotherapy. One child with partial resection died of sepsis at 22 months after diagnosis during postoperative chemotherapy after complete surgical removal of the tumor. Five children are alive without recurrence at 40, 45, 48, 60, and 122 months, respectively, after diagnosis.

Conclusion: The combined therapy of surgery and chemotherapy in USL can improve the prognosis.