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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: To summarize the clinical and histopathological features of renal giant angiomyolipoma combined with tuberous sclerosis, and discuss the diagnosis and treatment methods and prognosis. Methods: The clinical features, histopathological features, diagnosis and treatment of 1 case of renal giant angiomyolipoma complicated with tuberous sclerosis were retrospectively analyzed. At the same time, the related literatures at home and abroad in recent years were reviewed. Results: The patients underwent laparotomy in our hospital and giant nephrectomy of the right kidney. The postoperative pathology showed angiomyolipoma. Patients recovered well after surgery, symptoms improved significantly, liver and kidney function was normal. Three months after surgery, CT was reviewed. There was no change in the left kidney area compared with that before operation, and no tumor recurrence in the right kidney area. CONCLUSIONS: Surgical treatment may still be an alternative treatment for patients with giant renal angiomyolipoma with tuberous sclerosis, but its long-term prognosis remains to be seen.