小儿后颅窝巨大肿瘤的临床研究

Clinical research of pediatric giant posterior fossa tumors

目的探讨小儿后颅窝巨大肿瘤的临床特征及其治疗效果。方法回顾性收集温州医科大学附属第二医院神经外科自2015年1月至2020年6月收治的18例后颅窝巨大肿瘤(肿瘤任一截面最大直径超过5 cm)患儿的临床资料,并分析其手术治疗效果。结果18例患儿的肿瘤体积为(63.9±20.7)mL(33.2〜116.2mL)。术前存在不同程度梗阻性脑积水的16例患儿中,术前行Ommaya囊植入术2例、脑室腹腔分流术1例、脑室外引流术1例,术中行脑室外引流术5例,余7例术后自愈。17例患儿接受肿瘤切除术,其中10例全切除,7例次全切;术后病理回报髓母细胞瘤10例、室管膜瘤3例、毛细胞星形细胞瘤2例、少突胶质细胞瘤1例、卵黄囊瘤1例;予化疗12例、放疗9例。术后发生持续性脑积水2例,小脑性缄默综合征2例,蛛网膜下腔出血伴脑室积血1例。随访3〜67个月,13例患儿的生存期超过1年,其中10例1年内无肿瘤复发;10例肿瘤全切除患儿中8例的无进展生存期超过1年(另2例失访),7例肿瘤次全切患儿中3例的无进展生存期超过1年。结论小儿后颅窝巨大肿瘤的并发症发生率高、复发率高、预后较差,予对症治疗、尽量完整切除肿瘤、辅以放化疗是其有效的治疗方法。

Objective To explore the clinical features and treatment efficiencies of pediatric giant posterior fossa tumors.Methods A retrospective analysis was performed.The clinical data of 18 children with giant posterior fossa tumors,whose maximum diameter of any section was over 5 cm,admitted to our hospital from January 2015 to June 2020,were collected.The surgical treatment results were analyzed.Results The tumor volume of 18 children was(63.9±20.7)mL(33.2-116.2 mL).Sixteen children had different degrees of preoperative obstructive hydrocephalus;ommaya capsule implantation was performed in 2,ventriculoperitoneal shunt was performed in one,and external ventricular drainage was performed in one before surgery;intraoperative external ventricle drainage was performed in 5;and the left 7 had postoperative self-healing.Tumor resection was performed in 17 children,including 10 with total resection and 7 with subtotal resection.The postoperative pathological results indicated medulloblastoma in 10 patients,ependymoma in 3 patients,pilocytic astrocytoma in 2 patients,oligodendroglioma in 1 patient,and yolk sac tumor in 1 patient;12 patients were treated with chemotherapy and 9 with radiotherapy.Postoperative persistent hydrocephalus was noted in 2 patients,cerebellar mutism in 2 patients,and subarachnoid hemorrhage combined with ventricular hematocele in 1 patient.The follow-up period ranged from 3 to 67 months:13 children survived for more than one year(10 lived without tumor recurrence);8 of the 10 patients with total tumor resection had progression-free survival for more than 1 year(the other 2 patients were lost of follow-up),while 3 of the 7 patients with subtotal resection had progression-free survival for more than 1 year.Conclusion Pediatric giant posterior fossa tumors have high complication rate,high recurrence rate and poor prognosis;symptomatic treatment,total resection of the tumors,and adjuvant radiotherapy/chemotherapy are effective treatment methods.