Snapshot A 75-year-old man presents to the clinic with complaints of generalized fatigue and weight loss. He states that over the past 3 years he lost a total of 15 lbs. without changes to his diet or his exercise. He notes that he can no longer copmlete his daily 2 mile walks because he would get extremely tired half way. Laboratory studies revealed mild normocytic anemia and mild elevations in transaminases. Physical examination reveals moderate tenderness to palpation at the right upper quadrant. Introduction Overview hepatic angiosarcoma is a rare, high-grade malignant vascular neoplasm Epidemiology incidence most common sarcoma arising in the liver 0.1-2% of all primary tumors of the liver demographics occurs in older patients (> 60 years of age) more common in men risk factors vinyl chloride used to make polyvinyl chloride (PVC) pipes, wire coatings, plastic kitchen ware, and insulation arsenic anabolic steroids radiation thorium dioxide Pathogenesis majority of cases are of unknown etiology malignant neoplasm of endothelial cells that line the walls of blood vessels of lymphatic vessels can easily metastasize to distant sites common sites of metastasis include spleen, lymph nodes, lung, bone, and the adrenals Associated conditions Secondary Budd-Chiari syndrome thrombosis of the hepatic vein that drain the liver due to compression of the veins by angiosarcoma Prognosis very poor prognosis as diagnosis is often very late in the disease process mean survival time of 5 months Presentation Symptoms weight loss jaundice weakness pain RUQ or epigastric constant dull Physical exam hepatomegaly palpable mass distension (ascites) Imaging Computed tomography best initial imaging study findings numerous poorly defined variably sized nodules entire liver is frequently involved Studies Serum labs often accompanied by thrombocytopenia usually normal liver function until late in disease process Tumor biopsy immunostaining positive for vascular markers ERG transcription factor CD31 CD34 factor VIII antigen Histology grossly hemorrhagic and necrotic tissue tumor composed of pleomorphic spindle or epithelioid cells, often with bizarre or multinucleated forms and mitosis Differential Hepatocellular carcinoma histology appears as hepatocytes with differing degree of differentiation Cholangiocarcinoma laboratory studies often reveal cholestatic pattern Treatment Medical treatment adjuvant chemotherapy as needed Surgical and interventional partial or complete hepatectomy depending on presentation or location of tumor liver transplantation high recurrence rate Complications Liver failure Intraabdominal bleeding secondary to liver rupture