Primary Splenic Angiosarcoma: Clinical and Imaging Manifestations of This Rare Aggressive Neoplasm

Abstract

Primary splenic angiosarcoma is a rare and fatal neoplasm arising from vascular endothelial cells within the spleen. With an incidence of 2 cases per 10 million people worldwide, the diagnosis and treatment of this rare entity is unfamiliar and challenging. We describe the case of a previously healthy 45-year-old woman who presented with vague upper-abdominal pain and was found to have a splenic mass on computed tomography. The patient underwent laparoscopic splenectomy and was found to have splenic angiosarcoma on microscopic evaluation. Although specific radiologic diagnosis is not possible, bringing the possibility of primary splenic angiosarcoma to the ordering clinician's attention has the potential to hasten treatment and improve patient outcomes. This case highlights the importance for radiologists to be aware of this rare neoplasm and to consider it in the differential when encountering a heterogeneously enhancing splenic mass.