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目的:探讨子宫内膜透明细胞癌(UCCC)的临床病理特点和预后影响因素。方法:收集2004年1月1日至2014年12月31日在中国医学科学院肿瘤医院接受手术治疗且随访完整的34例UCCC患者资料，分析UCCC的临床病理特征、预后及预后影响因素。结果:34例患者中，18例(52.9%)患者术前取得UCCC病理确诊，8例(23.5%)患者按照UCCC标准接受了全面分期手术。ⅠA期17例(50.0%)，ⅠB期1例(2.9%)，Ⅱ期4例(11.8%)，ⅢA期2例(5.9%)，ⅢB期1例(2.9%)，ⅢC1期5例(14.7%)，ⅣB期4例(11.8%)。中位随访时间72个月，全组患者的5年无病生存率和5年总生存率分别为79.1%和81.3%。单因素分析显示，术前糖类抗原125(CA125)水平、淋巴结清扫范围、手术病理分期和术中腹腔细胞学是否阳性与患者的中位无病生存时间均有关(均n P<0.05)；术前CA125水平、淋巴结清扫范围、手术病理分期、术中腹腔细胞学是否阳性和淋巴脉管间隙浸润与患者的中位总生存时间均有关(均n P<0.05)。多因素分析显示，术中腹腔细胞学阳性为影响患者无病生存时间的独立危险因素，腹腔细胞学阳性患者的复发风险为阴性患者的11.47倍(n P＝0.009)，手术病理分期为ⅣB期为影响患者总生存的独立危险因素，ⅣB期患者的死亡风险为ⅠA期患者的25.29倍(n P＝0.009)。n 结论:UCCC术前病理诊断困难，会导致不全面分期手术。术中腹腔细胞学阳性、手术病理分期为ⅣB期分别为影响患者无病生存和总生存的独立危险因素，临床上应给予充分重视。“,”Objective:To analyze the clinicopathological features and prognostic factors of patients with uterine clear cell carcinoma (UCCC).Methods:UCCC patients who underwent surgery and complete follow-up at Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College between January 1, 2004 and December 31, 2014 were retrospectively reviewed. The Kaplan-Meier method and Cox regression analysis were used for survival analysis.Results:The study included 34 patients. Only 18 patients (52.9%) were diagnosed with UCCC preoperatively and 8 patients (23.5%) underwent UCCC standard comprehensive staging surgery. Among the 34 patients, stage ⅠA was 17 cases (50.0%), stage ⅠB was 1 case (2.9%), stage Ⅱ was 4 cases (11.8%), stage ⅢA was 2 cases (5.9%), stage ⅢB was 1 case (2.9%), stage ⅢC1 was 5 cases (14.7%) and stage ⅣB was 4 cases (11.8%). The median follow-up period was 72 months, 5-years disease-free survival (DFS) rate and overall survival (OS) rates for all patients were 79.1% and 81.3%, respectively. Univariate analysis result showed that preoperative CA125 level, range of lymphadenectomy, tumor stage and peritoneal cytology were significantly associated with DFS (n P<0.05). Preoperative CA125 level, range of lymphadenectomy, tumor stage, peritoneal cytology and lymph vascular space invasion were significantly associated with OS (n P<0.05). Multivariate analysis result showed that peritoneal cytology was the only independent prognostic factor for DFS, the relapse risk of peritoneal cytology positive patients was 11.47 folds higher than that of the negative patients (n P=0.009). Tumor stage was the only independent prognostic factor for OS, the death risk of ⅣB stage patients was 25.29 folds higher than that of theⅠA stage (n P=0.009).n Conclusions:The preoperative pathological diagnosis of UCCC is difficult, which results in incomplete surgical staging. Peritoneal cytology and tumor stage are independent prognostic factors for DFS and OS of UCCC patients, which deserve much more attention in clinical practice.