Clinical and pathological features of primary renal angiosarcoma.

We review all cases of renal angiosarcoma described in the medical literature to describe its clinical and histological features.Methods: The search term was “angiosarcoma of the kidney.” All articles or case reports in English, French, German or Spanish published until March 15, 2013 were considered. When available, data that focused on clinical and pathological features were extracted.Results: In total, the final cohort included 42 cases. The median overall survival (OS) was 5.0 months (95% confidence interval [CI], 2.1-7.9). Patients with metastatic disease had more than a threefold increase in the risk of death compared to patients without metastatic disease (hazard ratio: 3.27, 95% CI, 1.48-7.24; p = 0.004). Non-metastatic patients had dismal disease-free survival (DFS) rates, with a median DFS of 6.0 months (95% CI, 4.3-7.7); despite this, chemotherapy was effective to increase survival in eligible patients (4.0 vs. 1.0 months; p = 0.001). Microscopic examination found epithelioid and spindled cell in 44% and 56% of cases, respectively; there was a statistically insignificant increase in survival in patients with epithelioid patterns compared to spindled ones (9.0 vs. 4.0 months; p = 0.077). The tumour grading was related to OS; the lower the grade, the longer the survival (13 vs.4 months; p = 0.029).Conclusions: Renal angiosarcoma is a rare disease with defined clinical and pathological features. It has a very poor prognosis in patients with metastatic disease and a high recurrence rate in patients with non-metastatic disease.