Littoral cell angioma of the spleen in a patient with hepatocellular carcinoma.

Littoral cell angioma (LCA) is a rare primitive vascular tumor of the spleen which is benign and usually associated with visceral malignancy. The diagnosis of LCA is based on histologic and immunohistologic analysis. We report a case of LCA in a 62-year-old man with a 20-year history of chronic hepatitis B. He was admitted because of passage of tarry stool and poor appetite. Dynamic computed tomography studies showed splenomegaly (20 cm in long axis) with a hypodense tumor in the upper pole and a contrast-enhanced hepatic mass in the right lobe of the liver. Under the impression of hepatocellular carcinoma (HCC) and splenic tumor, partial hepatectomy and splenectomy were performed. Histopathology showed a moderately differentiated HCC of the liver and splenic angioma with dilated vascular channels lined by plump endothelial cells. Immunohistochemical staining of the splenic tumor showed focal positive reaction for factor VIII and CD68. LCA of the spleen was diagnosed. The postoperative course was uneventful during the 18-month follow-up. Although rare, LCA may coexist with cirrhosis and HCC, and is associated with visceral malignancy.