本文报道一例因血尿确诊肾肉瘤样癌的病例。患者因"间歇性全程肉眼血尿1月余"在茂名市人民医院就诊。泌尿系CTU提示左肾团块状占位，肿瘤与感染性病变待鉴别；左肾上腺局部增粗，未除外增生。CTA图像示病灶乏血供，由左侧肾动脉分支供血。经广东省医学会泌尿外科疑难病例多学科会诊专家建议行肾穿刺活检术明确肿物性质。穿刺结果回报提示穿刺组织内见异形梭形细胞伴坏死，符合恶性肿瘤，倾向肉瘤样改变。综合广东省医学会泌尿外科疑难病例多学科会诊专家的意见，成功为患者行经腹膜后途径腹腔镜下左肾根治性切除+左肾上腺切除术。术后随访患者恢复良好，复查肾功能正常，未见肿瘤复发和转移。因此针对肾肉瘤样癌等术前不能明确诊断的肾占位病变，可以考虑穿刺活检以获得明确的病理类型，以指导治疗。肾肉瘤样癌侵袭性强，建议行肾根治性切除术，术后必须进行严密随访。Ⅱ期以上的肿瘤患者可考虑联合靶向、免疫治疗或放疗等综合治疗。

This article reports a case of diagnosed sarcomatoid renal cell carcinoma due to hematuria. The patient was admitted to Maoming People's Hospital due to intermittent gross hematuria in the whole process of urination for more than 1 month. CT scan showed that mass lesion in the left kidney which required further differential diagnosis between tumor and infection, the left adrenal gland was partially thickened, and hyperplasia was not excluded. CTA images showed that the lesion lacked blood supply and was supplied by the left renal artery branches. After multidisciplinary consultations on difficult cases in the Urology department of Guangdong Medical Association, experts suggested to conduct renal biopsy to determine the nature of the lesion. The puncture pathological result indicated the presence of irregular spindle shaped cells with necrosis in the puncture tissue, which was consistent with malignant tumors and tended to be sarcomatoid changes. Based on the opinions of multidisciplinary consultations on difficult cases in the Urology department of Guangdong Medical Association, the patient underwent laparoscopic left radical nephrectomy and left adrenalectomy via the retroperitoneal approach. The patient recovered well during postoperative follow-up, with normal renal function and no tumor recurrence or metastasis. Therefore, for certain renal masses lesions such as sarcomatoid renal cell carcinoma that cannot be clearly diagnosed before surgery, preoperative needle biopsy can be considered to obtain a clear pathological type to guide treatment. Sarcomatoid renal cell carcinoma is highly invasive, and radical nephrectomy is recommended. Close follow-up is necessary after surgery. Comprehensive treatments such as combined targeted therapy, immunotherapy, or radiotherapy may be considered for patients with stage II or above.