Hepatobiliary and pancreatic: Angiosarcoma of the liver

Abstract

A 44-year-old man presented to the Emergency Department with syncope, hypotension and abdominal pain. He had been unwell for the previous 5 weeks with epigastric discomfort, anorexia and abdominal distension. He admitted to alcohol abuse in the distant past. On examination, he had mild jaundice and ascites. Blood tests revealed mild anemia, a marginally low platelet count and a prolonged international normalized ratio. His serum bilirubin was elevated at 3.5 mg/dL (60 µmol/l) and there were minor changes in liver enzymes and a marked elevation of lactate dehydrogenase (1994 u/l). Abdominal paracentesis revealed blood in the peritoneal cavity (hemoperitoneum). A contrast-enhanced computed tomography scan showed multiple, diffuse, enhancing masses, probably in the setting of cirrhosis. Subsequently, he developed features of sepsis requiring intubation and management in Intensive Care. A dynamic gadolinium-enhanced magnetic resonance scan showed patchy heterogeneous enhancement of most of the right lobe of the liver. One of the lesions is well-defined (white arrow) and shows both peripheral and central enhancement during the arterial phase with filling-in on the delayed phase (Figure 1). He also had mild splenomegaly and esophageal varices consistent with portal hypertension. As his serum bilirubin continued to rise, a liver biopsy was performed by the transjugular approach. Histologic evaluation revealed pleomorphic cells with large hyperchromatic nuclei and multiple mitotic bodies indicative of angiosarcoma. Immunoperoxidase stains were positive for CD31 (Figure 2) and CD34. He declined further treatment and was discharged for the initiation of palliative care. Angiosarcomas are rare tumors that account for only 1.8% of primary liver tumors. Although the cause is unclear in many patients, a minority have been exposed to carcinogens such as thorium dioxide (Thorotrast), arsenicals and vinyl chloride. The typical mode of presentation is as described above but only a minority of patients have gross hemoperitoneum. With imaging, the major differential diagnosis is that of peliosis hepatis. Unfortunately, the prognosis for patients with this tumor continues to be poor. In particular, it is rare to identify local disease that may be suitable for hepatic resection. Furthermore, the tumor is resistant to chemotherapy and radiotherapy and patients are rarely considered for liver transplantation because of high recurrence rates with short survival. The efficacy or otherwise of anti-angiogenic therapies remains unclear. Contributed by