Two cases of childhood undifferentiated sarcoma of the liver with long-term survival expectancy

Abstract

We report two rare cases of undifferentiated liver sarcoma (ULS). Case 1 was an 11-year-old girl presenting with fever and abdominal pain. Computed tomography (CT) revealed a large right-lobe liver tumor, and positron emission tomography-CT (PET-CT) demonstrated marked formation of tumor in the liver margin. A blood test showed a lactate dehydrogenase (LDH) level of 2145 U/ml and a CA 125 level of 178.8 U/mL, and biopsy revealed ULS. Tumor enucleation was performed after a five-course VAC regimen (namely, vincristine, actinomycin D, and cyclophosphamide). The patient underwent another eight courses of VAC postoperatively, and she is currently alive without recurrence 62 months after the completion of her treatment. Case 2 was 12-year-old boy presenting with distention of the upper right abdomen and abdominal pain. CT revealed a right-lobe liver tumor. PET-CT demonstrated the formation of tumor in the liver margin. A blood test showed an LDH level of 789 U/mL and a CA 125 level of 53.4 U/mL. With the presurgical diagnosis of ULS, the tumor was removed. The pathological finding of the excised tumor showed that the viable part of the liver showed polymorphic cell proliferation with severe hemorrhagic necrosis. Immunostaining revealed vimentin (−), α1ACT (+/−), and CD68 (−). Therefore, the final diagnosis was ULS. The patient underwent postoperative VAC (five courses), and he is currently alive without recurrence 16 months after the completion of his treatment. It is important to review the medical history when the LDH and CS 125 levels rise in older children.