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肾血管平滑肌脂肪瘤合并下腔静脉瘤栓的临床诊断与治疗

[Clinical diagnosis and treatment of renal angiomyolipoma with inferior vena cava tumor thrombus].

作者信息

Chen Kewei, Liu Zhuo, Deng Shaohui, Zhang Fan, Ye Jianfei, Wang Guoliang, Zhang Shudong

机构信息

Department of Urology, Peking University Third Hospital, Beijing 100191, China.

出版信息

Beijing Da Xue Xue Bao Yi Xue Ban. 2024 Dec 18;56(4):617-623. doi: 10.19723/j.issn.1671-167X.2024.04.012.

Abstract

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% . 0%, =0.003), more intraoperative bleeding [400 (240, 3 050) mL . 50 (50, 300) mL, =0.036], and a higher proportion of tumor necrosis (75% . 0%, =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 . (226.0±87.3) min, =0.053] between the two groups.

CONCLUSION

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.

摘要

目的

总结肾血管平滑肌脂肪瘤(RAML)合并下腔静脉(IVC)肿瘤血栓患者的临床特征,并探讨在这一系列患者中行部分肾切除术及血栓切除术的可行性。

方法

回顾性分析2014年4月至2023年3月在北京大学第三医院泌尿外科确诊为RAML合并IVC肿瘤血栓患者的临床资料,从电子病历系统记录并收集RAML合并IVC肿瘤血栓患者的人口统计学和围手术期数据,包括年龄、性别、手术方式及随访时间等。比较经典型血管平滑肌脂肪瘤(CAML)合并IVC肿瘤血栓患者与上皮样血管平滑肌脂肪瘤(EAML)合并IVC肿瘤血栓患者的临床特征,以明确这些患者的临床特点。

结果

本研究共纳入11例患者,其中7例为CAML合并IVC肿瘤血栓,4例为EAML合并IVC肿瘤血栓。女性9例(9/11,81.8%),男性2例(2/11,18.2%),平均年龄为(44.0±17.1)岁。9例患者(9/11,81.8%)出现临床症状,包括局部症状如腹痛、血尿、腹部肿块,以及全身症状如体重减轻和发热;2例RAML合并IVC肿瘤血栓患者未表现出临床症状,通过体格检查发现。11例患者中,10例行根治性肾切除术及血栓切除术,其中3例行开放手术(3/10,30.0%),2例行腹腔镜手术(2/10,20.0%),5例行机器人辅助腹腔镜手术(5/10,50.0%)。此外,1例患者行开放性部分肾切除术及血栓切除术。与CAML合并IVC肿瘤血栓患者相比EAML合并IVC肿瘤血栓患者全身临床症状比例更高(100%对0%,P=0.003),术中出血更多[400(240,3050)mL对50(50,300)mL,P=0.036],肿瘤坏死比例更高(75%对0%,P=0.024)。然而,两组手术时间差异无统计学意义[(415.8±201.2)分钟对(226.0±87.3)分钟,P=0.053]。

结论

与CAML合并IVC肿瘤血栓患者相比,EAML合并IVC肿瘤血栓患者全身症状及肿瘤坏死发生率更高。此外,在选择的CAML合并IVC肿瘤血栓患者中,可行部分肾切除术及肿瘤血栓切除术以更好地保留肾功能。

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