90例儿童颅内残留或复发性室管膜瘤的手术疗效分析

Surgical outcome analysis of 90 children with residual or recurrent intracranial ependymoma

目的探讨儿童颅内残留或复发性室管膜瘤的手术治疗效果。方法回顾性分析2014年3月至2023年8月北京大学第一医院小儿外科收治的90例行外科手术治疗颅内残留或复发性室管膜瘤患儿的临床资料,其中32例为首次手术后肿瘤残留,58例为肿瘤复发;肿瘤位于后颅窝60例,小脑幕上23例,后颅窝肿瘤转移至侧脑室7例。术后通过门诊或电话方式进行随访,末次随访时间为2023年10月,获知患儿生存及后续治疗情况,通过头颅MRI检查评估患儿肿瘤是否再次复发或转移。结果90例患儿手术均顺利完成,其中肿瘤扩大全切除10例,全切除71例,近全切除9例。术后78例(86.7%)患儿出现并发症,其中发热70例,皮下积液39例,出现声音嘶哑、吞咽困难等后组脑神经损伤症状16例,面瘫13例;术后13例(14.4%)患儿因继发性脑积水行脑室-腹腔分流术,其中2例分别出现消化道大出血、心力衰竭于术后24h死亡;1例因术后并发肺炎出院后死亡。术后经病理学证实,69例患儿的肿瘤病理学诊断与首次诊断相同,其中世界卫生组织(WHO)2级5例,3级62例,2~3级2例;21例肿瘤病理学诊断与首次诊断不同,其中7例由WHO2级转为3级,1例由1级转为2级,9例由2~3级转为3级,4例由3级转为2级。87例患儿均获得临床随访,随访时间[M(范围)]为16个月(3~116个月)。49例患儿术后行首次或二次放疗,3例行单纯化疗3例行放疗和化疗。末次随访显示,27例患儿死于肿瘤复发或转移;存活的60例患儿中,局部再复发5例,脊髓转移2例。结论儿童颅内残留或复发性室管膜瘤的治疗效果欠佳,目前仍以手术切除辅助术后放疗为主要治疗手段。

Objective To investigate the surgical treatment of residual or recurrent intracranial ependymoma in children.Methods The clinical data of 90 children with residual or recurrent ependymoma admitted to the Department of Pediatric Surgery at Peking University First Hospital from March 2014 to August 2023 were retrospectively analyzed.Among them,32 cases had residual tumors after initial operation,and 58 cases had recurrent tumors.The tumor was located in the posterior cranial fossa in 60 cases and supratentorial region in 23 cases,and metastasis of the original posterior cranial fossa tumor to the lateral ventricle was identified in 7 cases.After surgery,patients were followed up either at the outpatient department or through telephone interviews to assess survival status,follow-up treatments,the last follow-up time was in October 2023,and recurrence/metastasis using head MRI scans.ResultsAll 90 cases underwent successful operations,including extended resection in 10 cases,total resection in 71 cases and near-total resection in 9 cases.Postoperative complications occurred in 78 cases(86.7%)including fever in 70 cases,subcutaneous effusion in 39 cases,hoarseness and dysphagia in 16 cases,and facial paralysis in 13 cases.Thirteen cases(14.4%)underwent ventriculoperitoneal shunt surgery for secondary hydrocephalus after surgery,and two of them died 24 h after surgery due to gastrointestinal bleeding and heart failure,respectively.One case died of postoperative pneumonia after discharge.After surgery,the pathological diagnosis of 69 cases remained consistent with initial diagnosis,comprising 5 cases classified as WHO grade 2,62 cases classified as WHO grade 3,and 2 cases classified as WHO grade 2-3.The pathological diagnosis of 21 cases differed from the initial diagnosis,with 7 cases upgrading from WHO grade 2 to grade 3,1 case upgrading from grade 1 to grade 2,9 cases upgrading from grade 2-3 to grade 3,and 4 cases downgrading from grade 3 to grade 2.All 87 patients were followed up,with a follow-up time[M(range)]of 16 months(3-116 months).Among them,49 patients received primary or salvage radiotherapy,while 3 patients received chemotherapy alone and another 3 patients received both radiotherapy and chemotherapy.At the last follow-up visit,tumor recurrence or metastasis led to the demise of 27 patients.Among the 60 surviving children,local recurrence was observed in 5 patients and spinal cord metastasis occurred in 2 patients.Conclusion The treatment effect of residual or recurrent intracranial ependymoma in children remains to be not ideal,and surgical resection assisted by postoperative radiotherapy is still the main treatment method.