总结分析3例肾脏原发少见肿瘤的临床特征及诊治方法，提高对肾脏原发少见肿瘤的认识。

回顾性分析2018年5月至9月于中山大学附属第七医院泌尿外科诊治的术后病理证实的3例肾脏原发少见肿瘤的临床资料。

3例患者分别为：（1）54岁女性，因体检发现左肾占位1周入院，CT提示左肾巨大软组织肿块及肾门多个肿大淋巴结，行腹腔镜下根治性左肾切除术+淋巴结清扫术，术后病理：肾黏液管状与梭形细胞癌，术后3个月随访肿瘤无复发转移；（2）44岁男性，因左侧腰腹部胀痛1个月入院，MR提示左肾巨大占位病变，开放探查见肿瘤与降结肠粘连明显，遂行根治性左肾切除+降结肠部分切除术，术后病理：肾脏癌肉瘤，术后1个月局部复发并腹腔内多发转移，入肿瘤内科后辅以紫杉醇+多柔比星化疗及阿帕替尼靶向治疗，目前未观察到肿瘤继续恶化；（3）70岁男性，因左侧腰痛伴肉眼血尿3个月入院，CT提示左肾上极不规则低密度灶及腹膜后多发肿大淋巴结，行腹腔镜下根治性左肾切除+淋巴结清扫术，术后病理：高级别浸润性尿路上皮癌，拟二次手术，患者拒绝，术后6个月随访肿瘤无复发转移。

肾脏原发少见肿瘤临床症状及影像学表现常无明显特异性，容易误诊，应注意鉴别诊断，治疗以根治性切除为主，部分肿瘤预后不良，术后需密切随访，必要时尽早辅以放化疗及靶向治疗。

To analyze the clinical characteristics and methods of diagnosis and treatment of primary rare kidney neoplasms, in order to improve the understanding of primary rare kidney neoplasms.

The data of three cases of primary rare kidney neoplasms confirmed pathologically in the Seventh Affiliated Hospital of Sun Yat-sen University from May 2018 to September 2018 were analyzed retrospectively.

Case 1 was a 54-year-old woman, CT scan showed a large soft tissue mass in the left kidney and several enlarged lymph nodes around renal hilum in a routine check-up a week ago, laparoscopic radical left nephrectomy and lymphadenectomy were then conducted and the mass was pathologically proved to be mucinous tubular and spindle cell carcinoma, no recurrence or metastasis was observed during a 3-month follow-up. Case 2 was a 44-year-old man, presented with a 1-month history of left flank abdominal pain, MR examination revealed a huge space-occupying lesion in the left kidney. In consideration of inconvenient laparoscopic operation, open exploratory surgery was performed. During the operation the descending colon was discovered adhered to the lesion apparently, consequently radical left nephrectomy and partial resection of descending colon were conducted, and the histopathology was proved to be carcinosarcoma. Local relapse and extensive intraperitoneal metastasis were observed a month later, auxiliary chemotherapy with paclitaxel combined doxorubicin and targeted therapy with apatinib were executed according to oncologist’s advices, no further progression was observed till now. Case 3 was a 70-year-old man, presented with left flank pain and gross hematuria for three months, CT scan displayed irregular low-density focus in the upper pole of the left kidney and multiple retroperitoneal enlarged lymph nodes. Laparoscopic radical left nephrectomy and lymphadenectomy were carried out and the histopathology was unexpectedly proved to be high grade invasive urothelial carcinoma, the further nephroureterectomy was recommended but the patient refused it, neither recurrence nor metastasis was observed during a 6-month follow-up.

The clinical symptoms and imaging manifestations of primary rare kidney neoplasms are frequently lack of specific and easily to be misdiagnosed, more attention should be paid to distinguish from other diseases. Radical excision is the main therapeutic method, some types of neoplasms have a poor prognosis and close follow-up is essential, auxiliary radiotherapy, chemotherapy or targeted therapy should be performed postoperatively as early as possible if it is necessary.