Objective: To study the clinical presentation, diagnosis, treatment and prognosis of primary angiosarcoma of the kidney.
Methods: We treated a patient with primary angiosarcoma, then searched the published papers with the terms of 'primary angiosarcoma of the kidney' and 'primary renal angiosarcoma' in PubMed database, found 27 patients with detailed data, and analyzed their characters in the clinical presentation, diagnosis, treatment and prognosis.
Results: The primary angiosarcoma occurred mainly from 50 years old to 69 years old, predominated in male patients. The clinical presentation was flank pain and hematuria, and the nephrectomy was the mainstay of the treatment; the maximum diameter and the metastasis status at the time of diagnosis had important prognostic value.
Conclusions: The primary angiosarcoma is a rare carcinoma and lacks of specific presentation. Accurate diagnosis depends on pathological examination. Surgery is the mainstay of the treatment, but the prognosis is poor.
Keywords: Primary angiosarcoma of the kidney; diagnosis; prognosis; treatment.