Undifferentiated (Embryonal) Sarcoma of the Liver in Children

ABSTRACT

Undifferentiated (embryonal) sarcoma is a rare malignancy of the liver in children and young adults. Seven cases of undifferentiated (embryonal) sarcoma of the liver pathologically verified at Seoul National University Children's Hospital between 1986 and 1999 were retrospectively analyzed. There were three girls and four boys, and their mean age at diagnosis was 12.1years (range, 7 - 13years). Six patients presented with an abdominal mass and/or pain, and one with weight loss. Tumor size ranged from 8.0x8.0cm to 15.0x15.0cm. Four tumors were located in right lobe, two in left lobe and one in both. One patient died during preparation for chemotherapy. Initial complete resection was performed in three patients. Two patients received the complete resection after chemotherapy. Five patients with complete resection survived without evidence of disease for 8, 11, 13, 28, 84 months. A patient with partial resection and chemotherapy died of sepsis during chemotherapy 19 months after complete surgical resection. Adjuvant chemotherapy and radiotherapy were performed in all patients after complete surgical resection. In conclusion, though undifferentiated (embryonal) sarcoma of the liver is highly malignant, we think that the combination therapy of surgery, chemotherapy and radiotherapy can get good results.