结节性硬化症合并双肾血管平滑肌脂肪瘤二例报告并文献复习

Tuberous sclerosis complex associated with renal angiomyolipoma: a report of two cases and review of the literature

目的进一步提高对结节性硬化症合并双肾血管平滑肌脂肪瘤的诊断和治疗水平。方法对2例结节性硬化症合并双肾血管平滑肌脂肪瘤患者的临床资料和随访情况进行回顾性分析并文献复习。结果 1例结节性硬化症合并双肾血管平滑肌脂肪瘤患者行高选择性肾血管栓塞术治疗,术后定期复查3年,无发展;1例行保守治疗后效果良好。结论结节性硬化症合并双肾血管平滑肌脂肪瘤的患者治疗必须考虑肾功能的保留问题,高选择性肾血管栓塞术为首选治疗。

Objective To investigate the clinical treatment and diagnosis of tuberous sclerosis complex （TSC） asso- ciated with renal angiomyolipoma （RAML）. Methods The clinical data and follow-up situation of 2 patients with TSC asso- ciated with RAML diagnosed and treated in our hospital were analyzed. The relevant literature was reviewed. Results One patient received superselective arterial embolization （SEA） successfully and was followed up for three years. Another patient was treated conservatively and achieved satisfactory results. Conclusion Renal dysfunction of the patients with TSC associ- ated with RAML must be taken fully into account, and SEA is the preferred therapy.