Supplementary Material for: 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

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.

伴肿瘤血栓延伸至下腔静脉（inferior vena cava, IVC）与右心房汇合处的血管平滑肌脂肪瘤（angiomyolipoma, AML）临床较为罕见。本文报告1例于2020年1月21日入住我中心的女性AML患者，该患者合并肿瘤血栓延伸至IVC与右心房汇合处，无呼吸困难症状。患者因腹痛接受全腹部增强CT检查，初步诊断为疑似肾源性AML伴肿瘤血栓。遂为患者实施开放性根治性肾切除术及腔静脉血栓切除术。术中行经食管超声心动图（transoesophageal echocardiography）检查，结果显示肿瘤血栓已抵达下腔静脉与右心房汇合处。手术时长255分钟，术中出血量为800毫升。患者于术后7天出院。术后病理检查提示为脂肪瘤样AML。免疫组织化学（immunohistochemistry）检测结果显示：波形蛋白（vimentin）阳性（+）、上皮膜抗原（EMA）阴性（-）、HMB45阳性（+）、S-100蛋白（S-100）阴性（-）、平滑肌肌动蛋白（SMA）阳性（+）、TFE-3阴性（-）、黑素A抗原（melan A）阳性（+）。经2年随访，患者恢复良好，未出现肿瘤复发。因此，脂肪瘤样AML亦应接受密切随访，以监测其复发与转移情况。当AML合并下腔静脉肿瘤血栓时，开放性血栓切除术联合根治性肾切除术是安全且有效的治疗方案。