肾原发性血管肉瘤：病例分析与文献复习
Primary angiosarcoma of the kidney: case analysis and literature review.
作者信息
Zhang Hai-Min, Yan Yang, Luo Ming, Xu Yun-Fei, Peng Bo, Zheng Jun-Hua
机构信息
Department of Urology, Shanghai 10th People's Hospital, Tongji University School of Medicine Shanghai 200072, China.
出版信息
Int J Clin Exp Pathol. 2014 Jun 15;7(7):3555-62. eCollection 2014.
OBJECTIVE
To study the clinical presentation, diagnosis, treatment and prognosis of primary angiosarcoma of the kidney.
METHODS
We treated a patient with primary angiosarcoma, then searched the published papers with the terms of 'primary angiosarcoma of the kidney' and 'primary renal angiosarcoma' in PubMed database, found 27 patients with detailed data, and analyzed their characters in the clinical presentation, diagnosis, treatment and prognosis.
RESULTS
The primary angiosarcoma occurred mainly from 50 years old to 69 years old, predominated in male patients. The clinical presentation was flank pain and hematuria, and the nephrectomy was the mainstay of the treatment; the maximum diameter and the metastasis status at the time of diagnosis had important prognostic value.
CONCLUSIONS
The primary angiosarcoma is a rare carcinoma and lacks of specific presentation. Accurate diagnosis depends on pathological examination. Surgery is the mainstay of the treatment, but the prognosis is poor.
目的
研究肾原发性血管肉瘤的临床表现、诊断、治疗及预后。
方法
我们治疗了1例肾原发性血管肉瘤患者，然后在PubMed数据库中以“肾原发性血管肉瘤”和“原发性肾血管肉瘤”为关键词检索已发表的论文，找到27例有详细数据的患者，并分析他们在临床表现、诊断、治疗及预后方面的特征。
结果
肾原发性血管肉瘤主要发生于年龄50岁至69岁，以男性为主。临床表现为侧腹痛和血尿，治疗以肾切除术为主；诊断时的最大直径和转移状态具有重要的预后价值。
结论
肾原发性血管肉瘤是一种罕见的癌，缺乏特异性表现。准确诊断依赖病理检查。手术是主要治疗方法，但预后较差。
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