儿童巨大肾血管平滑肌脂肪瘤1例报告及文献复习

A giant renal angiomyolipoma in a child:a case report and literature review

目的探讨儿童肾脏血管平滑肌脂肪瘤(angiomyolipoma,AML)的临床特征、诊断及治疗方法。方法通过回顾分析1例术前误诊为肾母细胞瘤的儿童巨大肾脏AML的临床、影像学及病理资料,结合相关文献复习,讨论其临床生物学行为、诊断及治疗方法。结果 CT及MRI检查提示左肾肾母细胞瘤,由于肿瘤体积巨大,行剖腹探查及左肾切除术,术后病理诊断为肾AML,随访6个月未见复发。结论对于小体积及儿童肾AML,仅仅依靠CT影像资料做诊断可能存在一定困难,体积巨大而肿瘤脂肪成分低的患者容易被误诊,大体积的儿童肾AML可以行手术治疗以避免不必要的化疗。

Objective To explore the clinical characterstics and analyze the diagnosis and treatment of renal angiomyolipoma（AML）in children. Methods We retrospectively reported a case of 11 years girl with a giant renal AML,which was misdiagnosed as Wilms tumor,and reviewed the relevant literatures.The biological behavior of AML was recorded,the diagnosis and treatment of these lesions were discussed. Results Wilms tumor was suspected by both of computed tomography（CT）and magnetic resonance imaging（MRI）,and the patient underwent surgical exploration and subsequent nephrectomy due to large size of the tumor.The diagnosis of AML was confirmed by histopathological examination.At 6 months follow-up postoperatively,the child was well without signs of recurrence. Conclusions Demonstration of fat on imaging examination can diagnose renal AML in most of the cases.However,depending solely on CT demonstration of lipid density in the renal mass to diagnose AML may be difficult in small tumors and tumors in children.In our case,the large size along with low fat content resulted in our misdiagnosis.We recommend conservative surgery for pediatric patients with a large size AML to avoid chemotherapy.