Objective: To investigate the clinical features and prognosis of rare subtypes of renal cell carcinoma. Methods: This retrospective study collected the data of 52 rare subtypes of renal cell carcinoma of patients who underwent surgery from January 2002 to December 2014 at Department of Urology, Peking University First Hospital. There were 12 patients with collecting duct carcinoma, 5 patients with Xp11.2 translocation renal cell carcinoma, 5 patients with mucinous tubular and spindle cell carcinoma, 30 patients with unclassified renal cell carcinoma. The study group included 25 male and 27 female patients, with mean age of 52 years. The mean tumour size was (6.5±3.9) cm (range: 1.5 to 21.0 cm). The basic clinical features, gross appearance, Fuhrman nuclear grade, TNM staging and prognosis of rare subtypes of RCC were studied. The OS curves were obtained for rare subtypes of renal cell carcinoma using the Kaplan-Meier method and compared using a Log-rank test. Results: The rate of lymph node and distant metastasis were 34.6% (18/52) and 17.3% (9/52). Malignancies were screened and detected by color Doppler ultrasonography or CT scan, however, no case was diagnosed before operation or aspiration, all cases were confirmed by the pathological examination. The average period of postoperative follow-up process was 65 months, and the mean survival time was (34±23) months. Conclusion: The clinical features of rare subtypes of renal cell carcinoma are similar to those of clear cell renal cell carcinoma, while the imaging changes will be helpful for diagnosis before operation.
目的： 探讨透明细胞癌、乳头状癌、嫌色细胞癌以外的少见类型肾细胞癌的临床特征及预后。 方法： 回顾性分析2002年1月至2014年12月就诊于北京大学第一医院泌尿外科的52例少见类型肾细胞癌患者的资料，包括集合管癌12例，Xp11.2易位/TFE3基因融合相关性肾细胞癌5例，黏液小管状和梭形细胞癌5例，未分类癌30例。患者男性25例，女性27例，平均年龄(52±17)岁。肿瘤位于右肾27例，位于左肾25例。肿瘤最大径1.5～21.0 cm，平均(6.5±3.9)cm。统计患者基本信息、肿瘤大小、肿瘤病理分型、Fuhrman分级、TNM分期等资料，并采用Kaplan－Meier曲线对少见类型肾细胞癌的预后进行分析。 结果： 18例发现淋巴结转移，9例发现远处转移。泌尿系统超声、腹部CT增强扫描为主要影像学诊断手段，但无一例于术前确诊，均依靠手术或穿刺获得标本进行病理学确诊。术后平均随访65个月，平均生存时间(34±23)个月。少见类型肾细胞癌患者的1、3、5年总体生存率分别为73%、59%、54%。 结论： 少见类型肾癌临床表现与透明细胞癌类似，影像学改变有助于术前定性诊断，手术为主要治疗方式。.
Keywords: Carcinoma, renal cell; Prognosis; Treatment.