Engel J D, Kuzel T M, Moceanu M C, Oefelein M G, Schaeffer A J
Department of Urology, Northwestern University Medical School, Chicago, Illinois, USA.
Urology. 1998 Nov;52(5):778-84. doi: 10.1016/s0090-4295(98)00286-6.
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.
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.
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.
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.
报告一例膀胱血管肉瘤新病例，回顾其他9例先前报道的病例，并讨论该极为罕见肿瘤患者在表现、治疗及临床病程方面的独特特征。强调多模式治疗的效用。
我们报告膀胱血管肉瘤的最新病例。我们还检索了世界文献（MEDLINE），发现9例先前报道的膀胱血管肉瘤病例。分析其表现、治疗及临床病程。
10例病例中，2例被认为起源于先前存在的膀胱血管瘤。2例患者有既往妇科恶性肿瘤接受体外放疗史，随后在过去的放疗区域内形成肉瘤。另外2例患者在发现膀胱病变之前出现皮肤病变。仅4例患者表现为原发性膀胱病变，无既往疾病或先前致癌暴露史（除吸烟外）。血尿是普遍表现，治疗方式差异很大。10例患者中，8例在23个月的随访期内死亡。5例死于肿瘤相关原因。这5例患者的平均生存期为10.6个月。最近的2例患者（包括我们的病例）分别在8个月和32个月时存活且无肿瘤。这2例患者均接受了多模式肿瘤治疗方法作为其治疗方案的一部分。
膀胱血管肉瘤极为罕见，通常致命。预后比更常见部位的血管肉瘤更差。区域淋巴结通常未受累，但局部复发并最终远处转移是常见情况。尚未确定最佳治疗方法，但很可能应采用将根治性手术与化疗和放疗相结合的多模式方法。
