目的: 分析肾上腺皮质癌伴静脉癌栓患者的临床病理特征、预后，并总结手术治疗经验。方法: 回顾性分析北京大学第三医院泌尿外科2018—2023年接受手术治疗的肾上腺皮质癌患者的相关数据，将其分为伴静脉癌栓组和不伴静脉癌栓组。采用Wilcoxon秩和检验比较定量变量，卡方检验/Fisher精确检验比较分类变量，采用Kaplan-Meier法计算生存率。结果: 共纳入27例肾上腺皮质癌患者，其中11例(40.7%)伴静脉癌栓，包括8例女性，3例男性，中位年龄49(36，58)岁，中位体重指数26.0(24.1，30.4) kg/m²；7例初诊时有症状，6例有高血压病史，2例皮质醇水平增高；中位肿瘤直径9.4(6.5，12.5) cm。3例肿瘤侧别为左侧，其中1例局限在左肾上腺中央静脉未侵入左肾静脉，2例癌栓生长达肝下下腔静脉；8例肿瘤侧别为右侧，其中1例在右肾上腺中央静脉未侵入下腔静脉，4例侵入肝下下腔静脉，3例生长达肝后下腔静脉。10例患者为欧洲肾上腺肿瘤研究协作网(European Network for the Study of Adrenal Tumors，ENSAT)临床分期Ⅲ期，1例为Ⅳ期。手术方式为开放手术6例，单纯腹腔镜手术者4例，机器人辅助腹腔镜手术1例；2例切除同侧肾脏；中位手术时间332(261，440) min，术中中位出血量900(700，2 200) mL，术后中位住院时间9(5，10) d；中位生存时间24.0个月，中位复发时间7.0个月，16例不伴静脉癌栓患者均未达到中位生存时间和中位复发时间。与不伴静脉癌栓组相比，静脉癌栓组有更差的3年总体生存(overall survival，OS)率(71.4% vs. 40.9%；Log-rank，P=0.038)和2年无复发生存(recurrence-free survival，RFS)率(53.7% vs. 9.1%；Log-rank，P=0.015)。结论: 肾上腺皮质癌伴静脉癌栓患者预后较差，肾上腺肿瘤切除术和静脉癌栓取出术治疗该疾病安全有效。

Objective: To analyze the clinicopathological features, prognostic value and surgical treatment experience in patients with adrenocortical carcinoma with venous tumor thrombus. Methods: We collected relevant data of the patients with adrenocortical carcinoma who had undergone surgery in Peking University Third Hospital from 2018 to 2023. The patients were divided into venous tumor thrombus group and non-tumor thrombus group. The Wilcoxon rank sum test was used to compare the quantitative variables. The chi-squared test and Fisher's exact test were used to compare the categorical variables. The Kaplan-Meier method was used to estimate the survival rate. Results: A total of 27 patients with adrenocortical carcinoma were included, of whom 11 cases (40.7%) had venous tumor thrombus. In the patients with venous tumor thrombus, 8 patients were female and 3 were male. The median age was 49 (36, 58) years. The median body mass index was 26.0 (24.1, 30.4) kg/m². Seven patients presented with symptoms at their initial visit. Six patients had a history of hypertension. Elevated levels of cortisol were observed in 2 cases. Three tumors were found on the left side, while 8 were found on the right side. Median tumor diameter was 9.4 (6.5, 12.5) cm. On the left, there was a case of tumor thrombus limited to the central vein of the left adrenal gland without invasion into the left renal vein, and two cases of tumor thrombus growth extending into the inferior vena cava below the liver. One case of tumor thrombus on the right adrenal central vein did not invade the inferior vena cava. Four cases of tumor thrombus invaded the inferior vena cava below the liver and three cases extended to the posterior of the liver. Ten patients were in European Network for the Study of Adrenal Tumors (ENSAT) stage Ⅲ and one was in ENSAT stage Ⅳ. Open surgery was performed in 6 cases, laparoscopic surgery alone in 4 cases and robot-assisted laparoscopic surgery in 1 case. Two patients underwent ipsilateral kidney resection. Median operative time was 332 (261, 440) min. Median intraoperative bleeding was 900 (700, 2 200) mL. Median hospital stay was 9 (5, 10) days. Median survival time for the patients with tumor thrombus was 24.0 months and median time to recurrence was 7.0 months. The median survival and recurrence time of 16 patients without tumor thrombus were not reached. The patients with tumor thrombus had worse 3-year overall survival (OS) rate (40.9% vs. 71.4%; Log-rank, P=0.038) and 2-year recurrence-free survival (RFS) (9.1% vs.53.7%; Log-rank, P=0.015) rates compared with the patients with non-tumor thrombus. Conclusion: Patients with adrenocortical carcinoma with venous tumor thrombus have poor prognosis. Different adrenal tumor resections and venous tumor thrombus removal procedures based on different tumor thrombus locations are safe and effective in treating this disease.