婴幼儿脉络丛乳头状癌的临床特点及疗效观察

Clinical characteristics and treatment outcome of choroid plexus carcinomas in infants and young children

目的探讨婴幼儿脉络丛乳头状癌(CPC)的临床特点、治疗策略及其预后.方法回顾性分析2015年1月至2020年12月在上海交通大学医学院附属上海儿童医学中心神经外科收治的6例CPC患儿的临床资料,肿瘤均位于侧脑室内,采用经侧脑室枕角三角区入路行肿瘤切除术;术后1、3、6个月,术后1年及以上每年定期进行临床随访,随访术后患儿是否进行放、化疗,是否行二次手术,有无肿瘤播散、转移,存活状态及预后情况.结果6例患儿的中位就诊年龄为29.5个月(5~38个月),就诊时除1例患儿无症状外,其余5例均伴有恶心、呕吐等颅内压增高表现.术前头颅MRI T1加权成像显示肿瘤呈等信号或低信号,T2加权成像呈稍高信号,增强序列呈不同程度强化.6例患儿的首次手术均顺利完成,4例肿瘤全切除,2例部分切除,术后均未发生手术相关并发症.术后病理学检查可见细胞核分裂明显,Ki-67阳性指数为30%~65%,均证实为CPC.6例患儿的中位随访时间为36个月(6~59个月);4例患儿(其中2例为肿瘤复发)经历二次手术,2例肿瘤全切除,2例部分切除.至末次随访,4例患儿存活,2例死亡.4例存活的患儿术后均行卡铂+长春新碱辅助化疗,2例同时辅助全脑、脊髓放疗,临床症状均改善,生长发育正常,预后良好.2例死亡的患儿术后均未行放、化疗等辅助治疗,术后脑室内多处种植、转移后死亡.结论CPC多发于婴幼儿,多伴有颅内压增高表现,手术应尽可能全切除肿瘤,如术后复发需考虑二次手术切除;对术后肿瘤残留或转移的患儿可考虑放、化疗等辅助性治疗,有助于改善预后.

Objective To explore the clinical characteristics,treatment strategies and prognosis of choroid plexus carcinomas(CPC)in infants and young children.Methods We retrospectively analyzed the clinical data of 6 children with CPC who were admitted to the Neurosurgery Department of Shanghai Children's Medical Center,School of Medicine,Shanghai Jiao Tong University from January 2015 to December 2020.The tumors were all located in the lateral ventricle,and the tumor resection was performed through the temporal occipital junction area and the lateral ventricular triangle zone.Regular clinical follow-up visits were conducted at 1,3,and 6 months after the operation,1 year after the operation,and every year thereafter to see whether the children received radiotherapy and chemotherapy after the operation,whether they underwent secondary surgery,whether there was tumor dissemination and metastasis,and the survival status and outcomes.Results The median age of the 6 children at diagnosis was 29.5 months(5-38 months).Except for 1 child who was asymptomatic at the time of treatment,the remaining 5 children were accompanied by symptoms of increased intracranial pressure such as nausea and vomiting.Preoperative brain MRI T1-weighted imaging showed that the tumor was iso-or hypointense,T2-weighted imaging showed slightly high signal,and enhancement sequences showed varying degrees of enhancement.The first operations of all 6 children were successfully completed,with total tumor resection in 4 cases and partial resection in 2 cases.No surgery-related complications occurred after operation.Postoperative pathological staining showed obvious nuclear divisions and a Ki-67 positive index of 30% to 65%.All cases were confirmed to be CPC.The median follow-up time of 6 children was 36 months(6-59 months);4 children(2 cases had tumor recurrence)underwent secondary surgery,2 cases had total tumor resection and 2 cases had partial resection.As of the last follow-up,4 patients were alive and 2 had died.The 4 surviving children all received adjuvant carboplatin+vincristine chemotherapy after surgery,and 2 patients received adjuvant whole-brain and spinal cord radiotherapy at the same time.Their clinical symptoms improved,their growth and development were normal,and their outcomes were good.The two children who died did not receive adjuvant treatment such as radiotherapy or chemotherapy after surgery.They died after multiple intraventricular dissemination and metastases.Conclusions CPC mostly occurs in infants and young children,and is often accompanied by increased intracranial pressure.The tumor should be surgically removed as completely as possible.If recurrence occurs after surgery,a second surgical resection should be considered.For children with residual or metastatic tumors after surgery,adjuvant therapy such as radiotherapy and chemotherapy can be considered,which can improve prognosis.