He was a former drinker without a history of chemical or radiation exposure, and no other comorbidities.
Magnetic resonance imaging of the liver showed a large solid mass measuring 12 × 10 × 12 cm, filling segments 6 and 7 of the right lobe.
Percutaneous computerized tomography-guided biopsy revealed a hepatic epithelioid hemangioendothelioma (HEH) that was positive for the markers CD31 and CD34, and reacted with the monoclonal antibody D2-40, indicating a vascular endothelial origin. No metastases were detected.
Briongos-Figuero and Zamora-Martínez explain that HEH is a rare liver tumor of vascular origin with an incidence of less than 0.1 per 100,000 individuals. It has an unpredictable course that ranges from benign hemangioma to angiosarcoma.
Factors associated with HEH include exposure to oral contraceptives, vinyl chloride, asbestos, thorium dioxide, major trauma to the liver, viral hepatitis, primary biliary cirrhosis, and alcohol consumption.
Development of these tumors involves activation of the vascular endothelial growth factor signaling pathway.
Briongos-Figuero and Zamora-Martínez say that findings from computed tomography can include multiple lesions or a large peripherally hypodense mass, peripheral enhancement of contrast, and hypervascularized lesions. Magnetic resonance imaging shows a hypo-intense mass on T1-weighted, and a hyperintense mass on T2-weighted, images. Ascites and portal hypertension rarely are observed, but extra-hepatic metastasis are frequent.
Surgical resection and liver transplantation are the most common treatments for HEH, which seems to be resistant to chemotherapy and radiotherapy.
The patient of Briongos-Figuero and Zamora-Martínez could not undergo liver resection or transplantation because of his deteriorated physical state, the size of his tumor, and his risk for acute liver failure. He was treated with thalidomide but suddenly developed severe hematemesis; he died 4 weeks after the start of therapy.