肾脏巨大血管平滑肌脂肪瘤合并结节性硬化1例报告并文献复习

Giant Angiomyolipoma of the Kidney Associated with Tuberous Sclerosis:a Case Report and Review of the Literature

目的:总结肾脏巨大血管平滑肌脂肪瘤合并结节性硬化的临床及组织病理特点,探讨其诊疗方法及预后情况。方法:回顾性分析1例罕见的肾脏巨大血管平滑肌脂肪瘤合并结节性硬化的临床特征、组织病理学特点以及诊断、治疗方法,同时复习近年来的国内外相关文献。结果:患者于我院行剖腹探查,右肾巨大肿瘤切除术,术后病理示血管平滑肌脂肪瘤。手术后患者恢复良好,症状明显改善,肝肾功能正常。术后3个月复查CT:左肾区与术前比无变化,右肾区未见肿瘤复发。结论:对于巨大肾脏血管平滑肌脂肪瘤合并结节性硬化的患者,手术治疗仍可作为可选择的治疗方法,但其远期预后仍待观察。

Objective： Summary of the clinical and pathological features of renal huge angiomyolipoma associated with tuberous sclerosis,diagnosis,treatment and prognosis.Methods： We analysed a rare case of huge renal angiomyolipoma associated with tuberous sclerosis clinical features,histopathologic features,and diagnosis,treatment,and reviewed relevant literature in recent years.Results： Patients with laparotomy in our hospital,the right kidney huge tumor resection,Pathological examination shows of AML.Good recovery in patients after surgery,symptoms improved significantly,liver and kidney function is normal.After 3 months to review CT： left kidney district preoperative unchanged,the right kidney area no recurrence.Conclusions： For a giant kidney AML with TSC patients,surgery is still available as treatment options,but its long-term prognosis remains to be seen.