Undifferentiated (embryonal) sarcoma of the liver. A clinicopathologic study of a survivor treated with combined...

Undifferentiated (embryonal) sarcoma is one of the less common primary malignant tumors of the liver that typically, although not exclusively, presents in later childhood. It has come to be recognized as an aggressive neoplasm with the potential for local recurrence and distant metastasis, despite a seemingly successful total surgical resection. This report documents our experience with an 8-year-old boy who had an 80% hepatectomy for an undifferentiated (embryonal) sarcoma, followed by cisplatin (90 mg/m2), doxorubicin (20 mg/m2/d), and radiation therapy (1950 cGy) to the tumor bed. Residual microscopic tumor was very likely present in the region of the inferior vena cava, but exploratory laparotomies for the purpose of "second-look" biopsies were performed with negative results after the completion of each course of chemotherapy. The patient is off of treatment and disease-free 30 months after the diagnosis. Because the survival at this time has been so poor for this highly malignant tumor, we suggest that the combination of therapeutic techniques and second-look laparotomy(s) to identify any subclinical tumor may be a reasonable approach. Ultrastructural and immunohistochemical studies by us and other investigators indicate that the neoplastic cells are primitive mesenchymal cells by electron microscopic study and have immunoreactivity limited to vimentin, alpha-1-antitrypsin, and alpha-1-antichymotrypsin.