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肾脂肪瘤样血管平滑肌脂肪瘤伴瘤栓延伸至下腔静脉与右心房汇合处:一例罕见病例报告并文献复习。

Renal Lipoma-Like Angiomyolipoma of Tumour Thrombus Extending to the Confluence of Inferior Vena Cava with Right Atrium: A Rare Case Report and Literature Review.

机构信息

Department of Urology, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China.

Department of Pathology, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China.

出版信息

Urol Int. 2023;107(7):742-746. doi: 10.1159/000529412. Epub 2023 Mar 8.

Abstract

Angiomyolipoma (AML) complicated with tumour thrombus extending to the confluence of inferior vena cava (IVC) with right atrium is rarely observed. We report a female AML patient admitted to our centre on January 21, 2020, with complication of tumour thrombus extending to the confluence of IVC with right atrium and had no sign of difficult breathing. She underwent whole-abdominal enhanced CT for abdominal pain and was diagnosed with a possible renal AML with tumour thrombus. Open radical nephrectomy and thrombectomy of vena cava were performed. Intraoperative transoesophageal echocardiography indicated that the tumour thrombus has reached the confluence of IVC with right atrium. The operation took 255 min with an intraoperative haemorrhage of 800mL. The patient was discharged 7 days after surgery. Pathology revealed lipoma-like AML. Immunohistochemistry showed vimentin (+), EMA (-), HMB45 (+), S-100 (-), SMA (+), TFE-3 (-), melan A (+). After 2 years of follow-up, we found that the patient showed full recovery and had no recurrence. Therefore, lipoma-like AML should also be followed closely for recurrence and metastasis. When AML involves IVC tumour thrombus, open thrombectomy and radical nephrectomy are safe and effective methods.

摘要

血管平滑肌脂肪瘤(AML)合并瘤栓延伸至下腔静脉(IVC)与右心房交汇处的情况很少见。我们报告了一名女性 AML 患者,于 2020 年 1 月 21 日入住我们中心,合并瘤栓延伸至 IVC 与右心房交汇处,且无呼吸困难迹象。她因腹痛接受了全腹部增强 CT 检查,被诊断为可能患有肾 AML 合并瘤栓。行开放性根治性肾切除术和静脉血栓切除术。术中经食管超声心动图提示肿瘤栓子已达 IVC 与右心房交汇处。手术耗时 255 分钟,术中出血 800mL。患者术后 7 天出院。病理结果显示为脂肪瘤样 AML。免疫组化显示波形蛋白(+)、EMA(-)、HMB45(+)、S-100(-)、SMA(+)、TFE-3(-)、melan A(+)。随访 2 年后,我们发现患者完全康复,无复发。因此,脂肪瘤样 AML 也应密切随访复发和转移情况。当 AML 累及 IVC 瘤栓时,开放性血栓切除术和根治性肾切除术是安全有效的方法。

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