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目的总结双肾血管平滑肌脂肪瘤(RAML)伴结节性硬化症(TSC)的发病特点,以提高该病的诊治水平。方法回顾性分析4例双RAML伴TSC患者的临床资料。结果4例患者经B超、CT检查诊断。1例因左肾肿瘤出血行肾切除,1例因右肾肿瘤巨大行肾切除。另2例患者因肿瘤小且症状轻微行保守治疗,随访12~48个月,肿瘤大小无变化,无症状复发。结论B超、CT检查结合临床表现是诊断RAML伴TSC的重要手段。治疗方案应根据双肾肿瘤的大小、分布、发展情况及症状决定。手术时应注意保留正常肾组织,保护肾功能。
Objective To summarize the incidence of renal angiomyolipoma (RAML) with tuberous sclerosis (TSC) in order to improve the diagnosis and treatment of this disease. Methods The clinical data of 4 patients with dual RAML and TSC were retrospectively analyzed. Results 4 patients were diagnosed by B-ultrasound and CT. One patient underwent nephrectomy due to left renal tumor hemorrhage, and one had a nephrectomy due to a massive nephrectomy. The other 2 patients due to small tumors and conservative treatment of mild symptoms, followed up for 12 to 48 months, no change in tumor size, asymptomatic recurrence. Conclusion Ultrasonography, CT combined with clinical manifestations is an important means of diagnosing RAML with TSC. Treatment options should be based on the size of renal tumors, distribution, development and symptoms. Surgery should pay attention to retain normal kidney tissue and protect renal function.