Objectives. To present a new case of angiosarcoma of the bladder, review 9 other previously reported cases, and discuss the unique features of our case with regard to presentation, treatment, and clinical course of patients with this exceedingly rare tumor. The utility of multimodality therapy is emphasized. Methods. We report the latest case of angiosarcoma of the bladder. We also reviewed the world literature (MEDLINE) and discovered 9 previously reported cases of angiosarcoma of the bladder. Presentation, treatment, and clinical course were analyzed. Results. Of the 10 cases, 2 were considered to have arisen from a preexisting bladder hemangioma. Two patients had a history of prior gynecologic malignancies treated with external beam radiotherapy, with subsequent sarcoma formation within the past treatment field. Two other patients presented with skin lesions that predated the discovery of bladder lesions. Only 4 patients presented with primary bladder lesions and no preexisting disease or previous carcinogenic exposure (except for tobacco use). Hematuria was a universal presentation, and treatment was widely variant. Of the 10 patients, 8 died during a period of follow-up of 23 months. Five patients died of tumor-related causes. Mean survival of these 5 was 10.6 months. The 2 most recent patients (including ours) were alive and tumor free at 8 and 32 months, respectively. Both of these patients underwent multimodality oncologic approaches as part of their treatment regimen. Conclusions. Angiosarcoma of the bladder is exceedingly rare and usually fatal. Prognosis is poorer than that of angiosarcomas in more traditional sites. Regional lymph nodes are typically spared, but local recurrence with eventual distant metastasis is the rule. Optimal therapy has not been determined, but it most likely should involve a multimodal approach combining radical surgery with chemotherapy and radiotherapy.
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