Objectives. To present a new case of angiosarcoma of the bladder, revi
ew 9 other previously reported cases, and discuss the unique features
of our case with regard to presentation, treatment, and clinical cours
e of patients with this exceedingly rare tumor. The utility of multimo
dality therapy is emphasized. Methods. We report the latest case of an
giosarcoma of the bladder. We also reviewed the world literature (MEDL
INE) 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 pree
xisting bladder hemangioma. Two patients had a history of prior gyneco
logic malignancies treated with external beam radiotherapy, with subse
quent sarcoma formation within the past treatment field. Two other pat
ients presented with skin lesions that predated the discovery of bladd
er 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 wa
s 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 sur
vival of these 5 was 10.6 months. The 2 most recent patients (includin
g ours) were alive and tumor free at 8 and 32 months, respectively. Bo
th of these patients underwent multimodality oncologic approaches as p
art of their treatment regimen. Conclusions. Angiosarcoma of the bladd
er is exceedingly rare and usually fatal. Prognosis is poorer than tha
t of angiosarcomas in more traditional sites. Regional lymph nodes are
typically spared, but local recurrence with eventual distant metastas
is is the rule. Optimal therapy has not been determined, but it most l
ikely should involve a multimodal approach combining radical surgery w
ith chemotherapy and radiotherapy. (C) 1998, Elsevier Science Inc. All
rights reserved.