Primary splenic angiosarcoma: case report and literature review

Abstract

This paper describes a case of an aggressive primary splenic angiosarcoma in a 29-year-old man and makes a review of its clinicai findings, pathologic reports, treatment and prognosis. Angiosarcomas comprise less than 1% of soft tissue sarcomas, and only a small percentage of these tumors arise in the spleen. These tumors usually represent a diagnostic challenge for the pathologist in view of its variegated histology, and the development of new immu-nohistochemical markers for vascular tumors like CD31 (platelet-endothelial cell adhesion molecule) helps to rule out other diagnoses. There is no standard chemotherapy treatment for angiosarcomas, and the prognosis of splenic angiosarcoma is particularly poor.