儿童肾上皮样血管平滑肌脂肪瘤1例及文献复习

摘要

Objective:To investigate the clinical manifestation,diagnosis,imaging and treatment and prognosis of renal epithelioid angiomyolipoma(EAML). Methods:The clinical data of a case of renal epithelioid angiomyolipoma in a child were observed,and the related literature were reviewed. Results:The patient was a 6 - year - old girl with abdominal pain,and a big mass on the left side of the abdomen. Radical left nephrectomy was used for treatment. Grossly,a tumor with the size of 15cm × 10cm × 8cm was found in the lower pole of the kidney,its envelope was com-plete,and the boundary was clear. Microscopically,the main component of the tumor were epithelial cells,nuclear fis-sion as rare,multinucleated tumor giant cells. Immunohistochemical results supported the renal epithelioid angiomyoli-poma. During the follow - up of 6 months,no tumor recurrence. Conclusion:The renal epithelioid angiomyolipoma was a rare mesenchymal tumors. It was no specificity in clinical manifestations. The diagnosis rely on the histopathological characteristics and immunohistochemical features. Surgical excision was major method but recurrence or metastases were possible,longer follow - up is needed.%目的：探讨肾上皮样血管平滑肌脂肪瘤（epithelioid angiomyolipoma，EAML）的临床特征、诊断、治疗与预后。方法：通过1例儿童肾 EAML 的报道，分析其临床资料并复习相关文献。结果：本例患儿女性，6岁，临床表现为腹部胀痛，左侧腹部可触及一巨大肿物。经肾肿瘤根治术，肉眼检查见肿瘤位于肾下极，大小约15cm ×10cm ×8cm，其包膜完整，与周围组织界限清楚。镜检示瘤体主要成分为上皮样细胞，核分裂象罕见，多核瘤巨细胞多见。免疫组化结果支持肾上皮样血管平滑肌脂肪瘤。术后随访6月，未见肿瘤复发。结论：肾 EAML 是一种罕见的肾脏间叶肿瘤，儿童病例报道甚少。临床表现缺乏特异性，诊断依靠病理学检查。手术切除为主要治疗方法，术后有复发和转移可能，宜严密随访。