婴儿颅内巨大肿瘤的临床分析

The clinical analysis of giant infantile intracranial tumors

目的分析婴儿颅内巨大肿瘤的临床特点并探讨其治疗方法及预后。方法2014年1月至2017年12月复旦大学附属儿科医院神经外科共收治18例婴儿颅内巨大肿瘤患者,回顾性分析其临床特征、治疗方法及预后。18例患儿中,8例行肿瘤切除手术,2例行脑室-腹腔分流术,1例行脑室外引流术,7例拒绝行治疗。通过临床随访和影像学随访评估患儿的预后。结果8例行肿瘤切除手术的患儿中,5例行肿瘤全切除,1例行大部分切除,1例仅行活组织检查术,1例术中发生失血性休克而死亡。术后发生硬膜下积液4例,脑积水1例,经对症处理后好转。术后病理学结果显示:脉络丛乳头状瘤3例(其中1例为非典型脉络膜乳头状瘤),髓母细胞瘤1例,原始胚胎性肿瘤1例,非典型畸胎样/横纹肌样瘤1例,神经上皮性肿瘤1例。7例行肿瘤切除手术的患儿中,2例术后失随访;余5例至末次随访时,1例生长发育较同龄人落后,1例有轻微的神经系统体征,余3例无异常。10例未行开颅手术的患儿中,2例失访,余8例均在出院后2个月内死亡。总体随访时间为2.0个月至3.7年。结论婴儿颅内巨大肿瘤罕见,其总体预后欠佳。应综合考虑肿瘤特点、手术风险及并发症等因素个体化制定治疗方案,从而改善患儿的预后。

Objective To analyze the characteristics of giant intracranial tumors in infants and to explore their treatment and prognosis. Methods This study retrospectively enrolled 18 cases of giant intracranial tumors in infants admitted to Neurosurgery Department of Children′s Hospital of Fudan University from January 2014 to December 2017. The clinical characteristics, treatment and prognosis were documented and analyzed. Clinical follow-up and imaging follow-up were applied to evaluate the prognosis. Among the 18 cases, 8 underwent excision of tumor and the other 10 cases refused tumor excision surgery. Ventricular-peritoneal shunt was undergone in 2 patients of them and 1 patient underwent external brain drainage. Results Of the 8 patients who underwent tumor resection, gross total resection was achieved in 5 cases, subtotal resection in 2 and biopsy in 1. One patient died from intraoperative hemorrhagic shock. Among the 7 patients with the surgery of tumor excision, 4 developed subdural effusion and 1 patient developed hydrocephalus. The clinical manifestation was improved after symptomatic treatment. Pathology results revealed 3 cases of choroid plexus (including 1 case of atypical choroidal papilloma), 1 case of medulloblastoma, 1 case of primordial embryonic tumor, 1 case of atypical teratoid/rhabdoid tumor and 1 case of nerve epithelial tumor. Five cases in tumor excision group were followed up including 1 patient developing growth retardation and 1 suffering from mild malfunction of nervous system. Eight in the group without tumor excision surgery died within 2 months after discharge and 2 of them were lost to follow-up. The range of follow-up duration was from 2 months to 3.7 years. Conclusions Giant intracranial tumors in infants are rare with an overall poor prognosis. To improve the prognosis, the characteristics of tumor, risk of treatment and complications should be taken into consideration to make therapeutic strategy.