Bladder angiosarcoma: a systematic literature review and survival analysis.

Background: Bladder angiosarcoma is a rare type of cancer with only sporadic cases reported. Methods: We performed a systematic review to describe clinicopathological features and survival outcomes. Results: Thirty-five cases reporting 68 patients were reviewed. Among them, 35 (51.4%) were diagnosed with de novo bladder angiosarcoma and 33 (48.6%) with radiation-induced bladder angiosarcoma. The mean age at presentation was 65.255 years (±14.6), and the median tumor size was 4.6 cm (interquartile range 2.9-6.7). Hematuria was the most common symptom, reported in 52/68 patients (76.4%). Microscopic examination revealed an epithelioid-shaped morphology in 43/47 patients (91.5%), spindle-shaped cells in 13/47 (27.6%), and mixed epithelioid and spindle-shaped cells in 9/47 (19.1%). Immunohistochemical analysis showed that CD31 was positive in 36/36 patients (100%), CD34 in 17/23 (73.9%), and Factor VIII-related antigen in 17/20 (85%). Metastasis was reported in 43/60 patients (71.6%). Chemotherapy was administered to 22/68 patients (32.4%). The median follow-up time was 5 months (interquartile range 3-12). Of the 54 patients with available survival data, 16 (29.6%) survived and 38 (70.4%) died of the disease. Multivariate Cox regression analysis identified female gender (hazard ratio [HR], 2.59; Conclusions: These findings suggest that BA is an aggressive disease regardless of its etiology. Early detection and timely initiation of chemotherapy may improve patient survival.