Primary embryonal sarcoma of liver is rare malignant tumor in pediatric age. This tumor has been reported as malignant nmesenchymoma, undifferentiated sarcoma, primary sarcoma and fibromyxosarcoma. Histologicaly, they are characterized by mixture
of
epithelial lined cystic structures, resembling the bile duct, and undifferentiated sarcomatous component. PAS positive eosinophilic globules are seen around the sarcomatous cells.
We reported a 6 years old boy who had palpable abdominal mass for 10 days. The mass origninated from the left lobe of liver, proved by CT and MRI. The patients underwent the left lateral segmentectomy of liver and the postoperative course was
uneventful. The pathologic report showed that the mass was primary embryonal sarcoma of liver. The postoperative combined chemotherapy was perfomed with vincristine, actinomycin D and cyclophosphamide. The patients is disease free one year after
the
diagnosis.
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