Abstract
Angiosarcoma (AS) is a malignant tumor occurring in less than 2% of soft tissue sarcomas. Primary involvement of the kidney is rare, its pathogenesis remains largely unknown and it has overlapping features with other tumors of the kidney. The objective of this paper is to review the case reports of primary AS of the kidney in the literature. The search terms were primary AS of the kidney, primary renal AS and primary renal hemangiosarcoma. The total cohort of the cases reviewed was 62. The mean age of presentation was 61 years old with a predilection for the male sex. Metastatic disease at the time of diagnosis accounted for 44.9% (22/49) of the cases reported and 44.4% (12/27) of patients with non-metastatic disease at diagnosis, subsequently developed metastasis. Primary AS of the kidney is a rare malignant tumor with a poor prognosis. Local recurrence and distant metastasis is common. Primary AS of the kidney shares similar clinical presentation with other renal tumors and imaging does not allow for tumor specific diagnosis. Histopathological examination and immunohistochemistry is very important for the confirmation of the diagnosis. Current treatment options include a variable combination of surgery, radiotherapy and chemotherapy.