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肾间叶性软骨肉瘤是一种罕见的恶性软组织肿瘤。本文报道1例，患者因体检发现左肾肿瘤7d入院，行机器人辅助腹腔镜肾部分切除术，术后经病理和基因检测诊断为左肾间叶性软骨肉瘤。术后6个月复查，未见肿瘤局部复发和转移。“,”Renal mesenchymal chondrosarcoma is a rare malignant soft tissue tumor. This paper reports a patient who was admitted to hospital because of left renal tumor, and underwent robot-assisted laparoscopic partial nephrectomy. The patient was diagnosed as left renal mesenchymal chondrosarcoma by pathological and genetic examination after operation. There was no local recurrence and metastasis six months after operation.