Splenic littoral cell angioma in an infant

A 1-year-old girl presented with fever, asthenia, and splenomegaly with hypersplenism. Abdominal ultrasound scan and magnetic resonance imaging showed multiple nodular cystic masses in an enlarged spleen. The histological examination of the resected spleen showed a novel type of vascular tumor called littoral cell angioma. The histopathologic and immunohistochemical features of this rare lesion are described. Distinction from other splenic vascular tumors is stressed because the clinical behavior of this new entity seems to be benign.