Primary angiosarcoma of the bladder.

CONTEXT

Primary bladder angiosarcomas are extremely rare, and their clinical and pathologic features are not well described.

OBJECTIVE

To further refine the clinical features of primary bladder angiosarcomas and define their pathologic spectra.

DATA SOURCES

Relevant sources were identified using MEDLINE and a subsequent bibliographic search of all pertinent reports and reviews. We also searched the M. D. Anderson pathology archives.

STUDY SELECTION

After excluding 4 cases that likely secondarily involved the bladder, we identified 9 true primary bladder angiosarcomas.

DATA EXTRACTION

Data were extracted on the following: demographics, clinical presentation, predisposing factors, gross pathology, microscopic pathology, immunophenotype, therapy, and outcomes.

DATA SYNTHESIS

Primary bladder angiosarcomas were found at a mean age of 64.2 years, with a male-female ratio of 8:1. Two cases arose in a postirradiation setting. Primary bladder angiosarcomas typically presented with hematuria and were grossly hemorrhagic, raised masses (mean size, 6.7 cm) of the trigone and/or dome. Histologically, most showed classic anastomosing channels lined by plump hyperchromatic cells, though many showed variant histology such as solid growth and epithelioid cytology. Three (43%) of 7 patients died within a year, but only 1 patient died with evidence of disease. The remaining patients were alive at the time of publication of their respective cases (mean, 22 months).

CONCLUSIONS

Primary angiosarcomas of the bladder are typically rare tumors of middle-aged and elderly men that present with locally advanced disease and show a wide histologic spectrum. However, their prognosis may be better than previously thought.