Objective:To investigate the clinical and pathological features,prognosis and differential diagnosis of tubulocystic carcinoma of the kidney(TCC).Methods:Retrospectively analysis the clinical dataof a case of TCC and review pertinent literatures with statistical analysis.Results:TCC is a rare disease in clinic.Review pertinent litera-tures of 83 TCC cases,fifty-one of which was elected and analyzed with statistical methods.The results showed an average age of (57.43±16.07)years(range from18 to 94,median 61).The ratio of incidence between men and women was 8∶1.The tumor ranged in size from 0.2 to 17.0 cm in its greatest dimension with a mean size of (28± 3.01)cm.By sex group,the age of onset and size of tumor showed no statistical difference.However,the age of on-set was found to be significantly lower on patients with clinical symptoms (39.40±16.24 vs 59.39±14.95,P<0.05).In our case,there showed no uniqueness in clinical and imaging features,with the pathological and Immuno-histochemical observations matching the document description.A follow-up of 1 9 months showed no recurrence and metastasisafter radical resection of right kidney.Conclusions:TCC is a rare entity of renal cell carcinoma with a male preponderance of 8∶1 .There was no characteristic of clinical manifestation with other renal neoplasms.Confirmed diagnosis depends on the examination of pathology and immunoh is tochemistry.Surgical resection is the preferred treat ment of TCC,with favorable postoperative prognosis and low malignant potential.%目的：：探究肾管状囊性癌的临床表现、病理特征、鉴别诊断及预后.方法：回顾1例肾管状囊性癌的诊疗过程,从国内外数据库搜索同类患者文献资料并进行统计分析.结果：肾管状囊性癌临床罕见,汇总国内外报道83例,筛选有效病例共51例进行统计学分析,结果显示肾管状囊性癌发病年龄18~94岁,平均(57.43±16.07)岁,男女发病率之比为8∶1,肿瘤大小0.2~17.0 cm,平均(4.28±3.01)cm.以性别分组,男女发病年龄及肿瘤大小差异无统计学意义(P＞0.5)；以有无临床症状分组,发现有临床症状者发病年龄(39.40±16.24)显著低于无临床症状者(59.39±14.95),P＝0.049.本资料1例肾管状囊性癌,病理观察及免疫组化与文献描述相符,肾切除术后随访19个月无复发及转移.结论：肾管状囊性癌好发于男性,临床表现与其他肾肿瘤相比无特异性,病理学及免疫组织化学检查为主要确诊方法,手术切除是主要治疗手段,预后良好,具有低度恶性潜能.
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