目的:探讨肾癌的罕见类型肾黏液样小管和梭形细胞癌(MTSCC)的临床病理特点、治疗和预后。方法:回顾性分析2009年11月、2013年10月收治的2例MTSCC患者的临床资料,并结合文献进行讨论。结果:2例患者均为女性,发病年龄分别为47、57岁,均由体检时发现,无临床表现。均为单侧单发,直径均在4 cm左右,影像学资料提示乏血供肿瘤,均采用后腹腔镜肾根治术,病理报告显示免疫组化CK7、CK19均为阳性,CD10阳性、阴性各1例。1例随访6年,1例随访1年,术后均无复发转移。结论:MTSCC是一类罕见的肾细胞癌,恶性程度较低,预后较好。影像学资料显示乏血供的肾占位病变在MTSCC的诊断中占有重要地位,需要与乳头状肾细胞癌、肾梭形细胞癌、间叶型梭形细胞肉瘤鉴别。治疗方法可选择根治性切除术和保留肾单位手术,根据肿瘤大小、位置、肾功能进行选择,微创手术优于开放手术。尽管预后相对好,仍有伴或不伴肉瘤样改变的MTSCC发生远处转移的报道,因此应重视术后的随访。 Objective: To investigate the clinicopathological features, treatment and prognosis of renal cell carcinoma of the rare type of renal mucinous tubules and spindle cell carcinoma (MTSCC). Methods: The clinical data of 2 patients with MTSCC who were treated in November 2009 and October 2013 were analyzed retrospectively, and discussed in the literature. Results: Both of the two patients were female. The age of onset was 47 and 57 respectively, both of which were found by physical examination and had no clinical manifestations. All single unilateral, diameter of about 4 cm, imaging data suggest lack of blood for the tumor, are used laparoscopic radical nephrectomy, pathological reports showed immunohistochemical CK7, CK19 were positive, CD10 positive and negative 1 case. One case was followed up for 6 years and one case was followed up for 1 year. No recurrence and metastasis was found in one case. Conclusion: MTSCC is a rare type of renal cell carcinoma with low malignant degree and good prognosis. Imaging data show that lack of blood supply of renal space occupying lesions in the diagnosis of MTSCC occupies an important position, need and papillary renal cell carcinoma, spindle cell carcinoma of the lung, mesenchymal spindle cell sarcoma identification. Treatment options radical resection and nephron surgery can be retained, according to tumor size, location, choice of renal function, minimally invasive surgery is better than open surgery. Despite the relatively good prognosis, there are reports of distant metastases in MTSCC with or without sarcomatoid changes, so follow-up should be emphasized.