Hepatic Angiosarcoma : Clinical & Gross

Comments:
Clinical Features: Hepatic angiosarcoma usually occurs late in life, predominantly in the 6th and 7th decades. It is more common in men (M:F = 3:1). Patients present with vague symptoms and signs of liver disease and the diagnosis is often delayed. The clinical presentation consists of abdominal pain, fatigue, weight loss, anorexia, nausea, vomiting, abdominal mass, ascites, and jaundice.

About 15%-20% of patients present to the ER with hemoperitoneum from tumor necrosis and rupture. This is considered to be a sign of malignancy because benign tumors rarely undergo spontaneous rupture. Rare cases present with acute liver failure. Many patients are asymptomatic and the tumor is discovered incidentally at autopsy.

Patients may develop hematologic complications, including thrombocytopenia (secondary to platelet entrapment in the tumor), microangiopathic hemolytic anemia (due to RBC fragmentation in the tumor), and disseminated intravascular coagulation (due to local intravascular destruction of clotting factors).

Gross Pathology: The tumor can show four growth patterns macroscopically: multiple nodules involving both lobes, large dominant mass, mixed pattern of dominant mass with nodules, and diffusely infiltrating micronodular tumor. The lesions consist of variably-sized hemorrhagic-looking spongy areas with ill-defined margins scattered throughout the liver. Some areas may have well-demarcated borders. High-grade foci are more solid with gray-white appearance. Large cystic spaces with hemorrhagic contents as well as grossly necrotic areas may also be present. Image copyright: pathorama.ch