儿童嗜酸性实性和囊性肾细胞癌1例并文献分析  

Eosinophilic solid and cystic renal cell carcinoma in children:a case review and literature analysis

作　　者：邵佳文 周平江 周玲玲[2] 李仲荣[1] Shao Jiawen;Zhou Pingjiang;Zhou Lingling;Li Zhongrong(Department of Pediatric Surgery,Second Affiliated Hospital&Yuying Children's Hospital,Wenzhou Medical University,Wenzhou 325000,China;Department of Pathology,Second Affiliated Hospital&Yuying Children's Hospital,Wenzhou Medical University,Wenzhou 325000,China)

机构地区：[1]温州医科大学附属第二医院,育英儿童医院小儿外科,温州325000 [2]温州医科大学附属第二医院,育英儿童医院病理科,温州325000

出　　处：《临床小儿外科杂志》2023年第3期283-287,共5页Journal of Clinical Pediatric Surgery

摘　　要：目的探讨儿童嗜酸性实性和囊性肾细胞癌的临床特点、诊治及预后。方法回顾性分析温州医科大学附属第二医院收治的1例儿童嗜酸性实性和囊性肾细胞癌患儿临床资料。以"eosinophilic solid and cystic renal cell carcinoma"或"eosinophilic solid and cystic renal cell cancer"为检索词,检索PubMed、Web of Science数据库相关文献;以"嗜酸性实性和囊性肾细胞癌"为检索词检索万方数据库及中国知网相关文献;检索时间2000年1月1日至2021年12月;排除重复文献后进行文献复习,总结该类肿瘤的临床、病理特征以及预后情况。结果患儿女,9岁,因"右侧腹部隆起3 d"入院,术前影像学检查提示右肾巨大占位;行右肾巨大肿瘤根治性切除术,病理诊断为儿童嗜酸性实性和囊性肾细胞癌;免疫组化提示:CK20(+)、PAX-8(+)、CD117(+)、CK7(-)。术后未行放化疗,截至目前已随访6个月,未见复发及转移。共获得3篇符合要求文献,共报道6例嗜酸性实性和囊性肾细胞癌患儿,其中男4例,女2例;平均年龄14岁(9~17岁),2例因腹痛、腹部肿块、双下肢疼痛就诊,均未表现出结节性硬化症(tuberous sclerosis complex,TSC)相关临床症状;1例为双侧,其余为单侧(2例右侧,1例左侧,2例未注明侧别)。1例行部分肾切除术,其余行根治性肾肿瘤切除术,其中1例出现下腔静脉受累,合并肺栓塞,术后辅助化疗2年后出现肝转移。TSC基因突变5例,均为TSC2基因突变。中位随访时间18个月(6~132个月),无一例肿瘤复发。结论儿童嗜酸性实性和囊性肾细胞癌罕见,一般无特异度临床症状,辅助检查亦无明显特殊表现,但具有独特的病理学表现,部分病例存在TSC基因突变;行根治性手术治疗预后较好。Objective To explore the characteristics,diagnosis,treatment and prognosis of eosinophilic solid and cystic renal cell carcinoma in children.Methods Retrospective analysis was performed for clinical data of a child with eosinophilic solid and cystic renal cell carcinoma.The databases of PubMed and Web of Science were searched with the key words of"eosinophilic solid and cystic renal cell carcinoma"or"eosinophilic solid and cystic renal cell cancer"or"eosinophilic solid and cystic renal cell carcinoma"in Wanfang and CNKI.The searching cutoff was up to December 2021.Duplicate literatures were excluded and clinicopathological features and prognosis of this kind of tumor summarized.Results This 9-year-old girl was hospitalized for"detecting a bulge of right abdomen for 3 days".Preoperative imaging examination indicated that right kidney occupied a large space.Then radical resection of giant tumor of right kidney was performed.Pathological diagnosis was eosinophilic solid and cystic renal cell carcinoma in childhood.Immunohistochemical hint:CK20(+);PAX-8(+);CD117(+);CK7(-).There were no postoperative radiochemotherapy.No recurrence or metastasis occurred during 6-month follow-ups.Six cases from the literature search and this one had a total of 6 children with eosinophilic solid and cystic renal cell carcinoma,including 4 boys and 2 girls with an average age of 14(9-17)years.Two cases had abdominal pain,abdominal mass or pain in both lower extremities and none of them showed clinical features of tuberous sclerosis complex(TSC).The involved side was bilateral(n=1),left(n=1),right(n=2)and non-specified(n=2).Partial(n=1)and radical(n=6)nephrectomy were performed.One child had an involvement of inferior vena cava plus pulmonary embolism and liver metastasis occurred 2 years after adjuvant chemotherapy.TSC gene mutation was detected in 5 cases,all of which were TSC2 gene mutation.There was no tumor recurrence during a median follow-up period of 18(6-132)months.Conclusion As a rare disease in children,eosinophilic solid rena

关 键 词：癌 肾细胞 结节性硬化症 嗜酸性细胞 外科手术 儿童 

分 类 号：R737.11[医药卫生—肿瘤]