肾血管平滑肌脂肪瘤合并下腔静脉和右心房瘤栓1例及文献复习

Renal angiomyolipoma with inferior vena cava and right atrial embolism:A case report and literature review

肾血管平滑肌脂肪瘤(angiomyolipoma,AML)合并肾静脉、下腔静脉、右心房瘤栓是一种十分罕见的实体肿瘤,其病因及发病机制目前尚不清楚,影像学检查易误诊。中南大学湘雅二医院收治的1例AML合并肾静脉、下腔静脉、右心房瘤栓的女性患者,35岁,无明显诱因右侧腰痛3年余,CT平扫示右肾及右肾静脉−下腔静脉−右心房柱不规则、分叶状、脂肪性密度肿块影,增强扫描示各期脂肪成分无强化,实性成分轻度强化。行右肾根治加瘤栓取出术,术后1年无复发,生活质量可。肾AML沿肾静脉、下腔静脉生长并延伸至右心房者罕见,影像学检查极为重要,其CT表现具有特征性,但最终明确诊断仍需依靠病理学及免疫组织化学检查。

Renal angiomyolipoma(AML)with renal vein,inferior vena cava(IVC),and right atrial embolism is a rare solid tumor,whose etiology and pathogenesis are still unclear.Moreover,it is often misdiagnosed.One patient with renal AML complicated with renal vein,IVC,and right atrial embolism was admitted to the Second Xiangya Hospital of Central South University,who was a 35-year-old female,without any previous medical history,presented with right low back pain for more than 3 years.Computed tomography(CT)scan showed irregular lobulated fatty density mass in the right kidney,renal vein,IVC,and right atrium.The contrast-enhanced scan showed no enhancement of fat components at each phase and mild enhancement of solid components.Radical resection of the right kidney and removal of tumor thrombus were performed,and there was no recurrence 1 year after the operation.It is rare for renal AML to grow along the renal vein,IVC,and extend to the right atrium.Imaging examination is extremely important,and the CT findings of this case are characteristic,but the diagnosis eventually depends on pathological and immunohistochemical examinations.