目的:总结肾血管平滑肌脂肪瘤(RAML)合并下腔静脉(IVC)癌栓的临床特点,并探讨该系列患者行肾部分切除和取栓的可行性。
方法:回顾性分析2014年4月至2023年3月北京大学第三医院泌尿外科诊断为RAML合并IVC癌栓患者的临床资料。通过电子病历系统记录并收集RAML合并IVC癌栓患者的人口统计学和围手术期数据,包括年龄,性别,手术方法,和随访时间,等。比较了经典血管平滑肌脂肪瘤(CAML)合并IVC癌栓的患者和上皮样血管平滑肌脂肪瘤(EAML)合并IVC癌栓的患者之间的临床特征,以确定这些患者的临床特征。
结果:本研究共纳入11例患者,包括7例CAML合并IVC癌栓和4例EAML合并IVC癌栓。女性9人(9/11,81.8%),男性2人(2/11,18.2%),平均年龄(44.0±17.1)岁。9例(9/11,81.8%)出现临床症状,包括腹痛在内的局部症状,血尿,腹部肿块,全身症状包括体重减轻和发热;2例(2/11,18.2%)RAML和IVC癌栓未出现临床症状,这是通过体检发现的。在11名患者中,10例接受根治性肾切除术伴血栓切除术,谁,3例行开放手术(3/10,30.0%),2例行腹腔镜手术(2/10,20.0%),机器人辅助腹腔镜手术5例(5/10,50.0%)。此外,1例患者行开放性肾部分切除术和血栓切除术。EAML合并IVC癌栓患者的全身临床症状比例较高(100%vs.0%,P=0.003),术中出血更多[400(240,3050)mL与50(50,300)mL,P=0.036],肿瘤坏死的比例更高(75%vs.0%,P=0.024)与CAML合并IVC癌栓患者比拟。然而,手术时间[(415.8±201.2)minvs.(226.0±87.3)min,两组间P=0.053。
结论:与CAML和IVC癌栓患者相比,EAML和IVC癌栓患者的全身症状和肿瘤坏死发生率较高。此外,在选定的CAML合并IVC肿瘤血栓的患者中,可以进行部分肾切除术和肿瘤血栓切除术,以更好地保护肾功能。
OBJECTIVE: To summarize the clinical characteristics of patients with renal angiomyolipoma (RAML) combined with inferior vena cava (IVC) tumor thrombus, and to explore the feasibility of partial nephrectomy and thrombectomy in this series of patients.
METHODS: The clinical data of patients diagnosed with RAML combined with IVC tumor thrombus in the Department of Urology of the Peking University Third Hospital from April 2014 to March 2023 were retrospectively analyzed, and demographic and perioperative data of RAML patients with IVC tumor thrombus were recorded and collected from Electronic Medical Record System, including age, gender, surgical methods, and follow-up time, etc. The clinical characteristics between classic angiomyolipoma (CAML) patients with IVC tumor thrombus and epithelioid angiomyolipoma (EAML) patients with IVC tumor thrombus were compared to determine the clinical characteristics of these patients.
RESULTS: A total of 11 patients were included in this study, including 7 patients with CAML with IVC tumor thrombus and 4 patients with EAML with IVC tumor thrombus. There were 9 females (9/11, 81.8%) and 2 males (2/11, 18.2%), with an average age of (44.0±17.1) years. 9 patients (9/11, 81.8%) experienced clinical symptoms, including local symptoms including abdominal pain, hematuria, abdominal masses, and systemic symptoms including weight loss and fever; 2 patients (2/11, 18.2%) with RAML and IVC tumor thrombus did not show clinical symptoms, which were discovered by physical examination. Among the 11 patients, 10 underwent radical nephrectomy with thrombectomy, of whom, 3 underwent open surgery (3/10, 30.0%), 2 underwent laparoscopic surgery (2/10, 20.0%), and 5 underwent robot-assisted laparoscopic surgery (5/10, 50.0%). In addition, 1 patient underwent open partial nephrectomy and thrombectomy. The patients with EAML combined with IVC tumor thrombus had a higher proportion of systemic clinical symptoms (100% vs. 0%, P=0.003), more intraoperative bleeding [400 (240, 3 050) mL vs. 50 (50, 300) mL, P =0.036], and a higher proportion of tumor necrosis (75% vs. 0%, P=0.024) compared to the patients with CAML combined with IVC tumor thrombus. However, there was no statistically significant difference in operation time [(415.8±201.2) min vs. (226.0±87.3) min, P=0.053] between the two groups.
CONCLUSIONS: Compared with the patients with CAML and IVC tumor thrombus, the patients with EAML and IVC tumor thrombus had a higher rate of systemic symptoms and tumor necrosis. In addition, in the selected patients with CAML with IVC tumor thrombus, partial nephrectomy and tumor thrombectomy could be performed to better preserve renal function.