腹腔镜肾部分切除术治疗儿童多房囊性肾肿瘤1例报告并文献复习

Laparoscopic partial nephrectomy for children with multilocular cystic renal neoplasm:a case report and literaturereview

目的探讨腹腔镜肾部分切除术治疗儿童多房囊性肾肿瘤的安全性和有效性。方法回顾性分析华中科技大学同济医学院附属武汉儿童医院2020年8月收治的1例儿童多房囊性肾肿瘤的资料。患儿,男,12岁。因B超发现左肾囊性包块并增大1年人院。B超及CT检查示左肾中下极实质内多房囊性包块,直径约5cm;CT增强扫描见该包块CT值约10HU,其内可见分隔和点状钙化,其中一处分隔强化的壁厚>4 mm。术前诊断为左肾囊性病变(BonsiakⅡ级,TiN。M期,R.E.N.A.L.评分9X),该病变中等概率为恶性。采用腹腔镜左肾部分切除术,术中见左肾中下极有一60mm×50mm大小的囊性包块,将其完整切除。结果术后病理诊断:多房囊性肾肿瘤,形态学特征呈多房囊性改变,肾周脂肪组织及肾盏切缘均未见肿瘤,免疫组化染色检查及荧光原位杂交检测TFE3(+),符合Xpl1.2易位/TFE3基因融合相关性肾癌。术后复查血肌酐61.6μmol/L。随访3年,左肾形态恢复可;腹部CT检查未见肿瘤复发及远处转移病灶。结论按照2019版Bosniak分级标准对儿童多房囊性肾肿瘤的良恶性进行预测,采用腹腔镜肾部分切除术治疗儿童多房囊性肾肿瘤,可保留部分患肾功能,是一种安全有效的微创治疗方法。

Objective To evaluate the safety and efficacy of laparoscopic partial nephrectomy(LPN)for multilocular cystic renal neoplasm(MCRN)in children.Methods The clinical data of a case with MCRN admitted to Wuhan Children's Hospital affiliated to Tongji Medical College of Huazhong University of Science and Technology in August 2020 was retrospectively analyzed.A 12-year-old boy was found with a left cystic renal mass and enlarged by ultrasound 1 year before admission.Ultrasound and CT examination showed a 5 cm MCRN in the lower pole of left kidney.The enhanced CT showed that the mass was about 10 HU,which showed septation and punctate calcification,and a wall thickness of one separated enhancement was>4 mm.A preoperative diagnosis of left renal cystic lesion(grade Bonsiak II,stage T,NoMo,R.E.N.A.L.score 9X).The lesion had an intermediate probability of being malignant.The patient underwent a laparoscopic left partial nephrectomy.During the operation,there was a 60 mm x 50 mm cystic mass in the lower pole of left kidney,which was removed intact.Results The pathological report of the mass was MCRN.Its morphological features showed multilocular cystic changes.No tumor component was found in its perirenal adipose tissue and incisional margin of its calyces.The expression of TFE3 was detected by immunohistochemistry and fluorescence in situ hybridization.It is consistent with renal cell carcinoma associated with Xpl1.2 translocation/TFE3 gene fusion(Xpl1.2 tRCC).After 3 years of followup,the shape of the left kidney was recovered partially without recrudescence and distant metastasis.His serum creatinine was 61.6μmol/L.Conclusions According to Bosniak classification of cystic renal masses(version 2019),benign and malignant MCRN in children were predicted.LPN is a safe and effective minimally invasive treatment for MCRN in children,which can preserve part of the renal function and improve the quality of life after operation.