Objective To improve the understanding,diagnosis and treatment of multilocular cystic nephroma.Methods Clinical manifestations and auxiliary examination data of a patient with multilocular cystic nephroma were retrospectively analyzed to investigate the diagnosis and treatment of multilocular cystic nephroma based on the review of literature.Results Ultrasonog-raphy showed right renal cyst and cystic renal cell carcinoma.CT showed right kidney placehold-er,suggesting a possibility of cystic renal cell carcinoma.Pathological report showed multilocular cystic nephroma.Because this patient had multilocular cystic nephroma with a history of surgery, tumor resection and partial nephrectomy could not be performed.Therefore,this patient received right kidney resection under general anesthesia.After a 3-year follow-up,no recurrence and me-tastasis were found in this patient.Conclusion Multilocular cystic nephroma should be pathologi-cally differentiated from cyst and cystic renal cell carcinoma.%目的：提高对多房性囊性肾瘤认识、诊断与治疗水平。方法对1例多房性囊性肾瘤患者的临床表现和辅助检查资料进行回顾性分析，结合文献探讨多房性囊性肾瘤的诊断和治疗。结果超声检查示：右肾囊肿、囊性肾癌待排；CT 检查示：右肾占位，考虑囊性肾癌可能性大；病理报告示：多房性囊性肾瘤。在全身麻醉下行右肾切除术，因患者既往有手术病史，且囊肿分隔多，术中无法行肿块切除及肾部分切除，最终行肾切除。术后随访3年，未见复发及转移。结论部分多房性囊性肾瘤应与囊肿、囊性肾癌进行病理鉴别诊断。
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