Abstract

ABSTRACT: Four patients had resection for primary hepatic sarcoma: one with malignant fibrous histiocytoma (MFH), two with poorly differentiated fibrosarcoma, and one with leiomyosarcoma. Age ranged from 40 to 69 years. One patient had a cousin and a grandmother who had died of hepatic tumors. At presentation, all patients had pain; one had tumor rupture, and one had mental changes and hypoglycemia. None had hepatitis or cirrhosis. Results of laboratory evaluation were nonspecific, including normal carcinoembryonic antigen and α-fetoprotein levels. Computed tomography showed hypodense masses with enhancement. Angiography showed a hypervascular mass in three patients and an avascular mass in the patient with MFH. Despite large tumors (8 to 32 cm), portal and hepatic veins were not invaded. The pattern of vascularization and lack of venous invasion helps differentiate primary hepatic sarcomas from hepatocellular carcinoma, especially in noncirrhotic patients. All patients had extensive hepatic resections, with one operative death. Immunohistochemical stains of the tumors were positive for vimentin but negative for epithelial markers, differentiating these lesions from other hepatic tumors. The patient with MFH died with recurrence at 10½ months. The patient with the ruptured fibrosarcoma had a second resection and chemotherapy, but died with recurrence at 3 years. The patient with the leiomyosarcoma had a second resection and was disease free at 4 years. Resection of primary hepatic sarcoma is warranted, with potential survival measured in years.