Primary renal angiosarcoma mimicking urothelial carcinoma - A case report and literature reviews.

Angiosarcoma (AS) is a rare aggressive tumor originating from endothelial cells. We reported a 66-year-old female with primary renal angiosarcoma (PRA) who presented as urothelial carcinoma with hematuria and dysuria. Based on ureterorenoscopic tumor biopsy, the initial diagnosis suggested low-grade non-invasive urothelial carcinoma. However, the specimen retrieved from nephroureterectomy confirmed the diagnosis of primary renal angiosarcoma. Primary renal angiosarcoma could uncommonly present as urothelial carcinoma in renal pelvis. Surgical resection remains to be the most effective therapy but there is no consensus about adjuvant therapies. The overall prognosis of primary renal angiosarcoma is dismal.