The AGA Journals Blog highlights the latest discoveries in gastroenterology and hepatology research.

Researchers describe a rare liver tumor of vascular origin in the May issue of Clinical Gastroenterology and Hepatology.

Laisa Socorro Briongos-Figuero and Tomás Zamora-Martínez describe the case of a 59-year-old man with chronic pancreatitis who was was admitted to the hospital with vomiting and jaundice, without fever or diarrhea. He had a previous history of cachexia, recurrent vomiting, and malnutrition related to cholecystectomy and a Roux-en-Y gastric bypass more than 10 years ago.

He was a former drinker without a history of chemical or radiation exposure, and no other comorbidities.

LiverTumorUltrasonography showed a large mass (11 × 12 cm) with internal vascularity, based on color Doppler ultrasound (see figure) and moderate ascites.

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.

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Kristine Novak

Kristine Novak

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About The Author:

Dr. Kristine Novak

Dr. Kristine Novak

Dr. Kristine Novak is a science writer and editor based in San Francisco. She has extensive experience covering gastroenterology, hepatology, immunology, oncology, clinical, and biotechnology research discoveries.

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