婴幼儿松果体区肿瘤临床特点及治疗策略

Clinical characteristics and treatment strategies of infantile pineal tumors

目的探讨婴幼儿松果体区肿瘤患者的临床特点、手术的可行性及手术入路的选择。方法回顾性分析2014年10月至2018年10月期间在广州市妇女儿童医疗中心神经外科接受手术治疗并经病理证实的11例婴幼儿松果体区肿瘤患者的临床资料,包括临床特点、影像学、手术入路、术后病理、并发症等。其中男6例,女5例;年龄3~36个月,平均年龄17.4个月;术前影像学提示均合并梗阻性脑积水。所有患儿均接受手术治疗,其中7例采用经纵裂胼胝体穹隆间入路,1例采用经右侧额中回皮层造瘘入路,1例采用枕下小脑幕上入路,1例采用颞顶皮层造瘘侧脑室三角区入路,1例采用枕下后正中入路。结果11例患儿中,8例患儿达到显微镜下全切除,2例大部分切除,1例仅行活检术。术后病理提示非典型畸胎瘤样/横纹肌样瘤(atypical teratoid/rhabdoid tumor,AT/RT)3例,松果体母细胞瘤2例,松果体细胞瘤2例,毛细胞星形细胞瘤1例,未成熟畸胎瘤1例,松果体原基瘤1例,混合性胚胎性肿瘤1例。术后脑积水缓解不理想需要行脑室腹腔分流术(ventriculoperitoneal shunt,VP shunt)5例,术后出现硬膜下积液8例,其中行硬脑膜下外引流术好转2例,6例自行吸收,无死亡病例。结论婴幼儿松果体区肿瘤临床病理特点明显与青少年及成人不同,治疗策略制定宜个体化,经纵裂胼胝体穹隆间入路适合大多数婴幼儿患者,明确病理诊断有利于制定下一步的治疗方案。

Objective To summarize the clinical characteristics of infant patients with pineal region tumors,and to explore the feasibility of active surgery and the choice of surgical approaches.Methods The clinical data were retrospectively analyzed for of 11 surgical infants with pathologically confirmed pineal region tumors who underwent surgery and were confirmed by pathology in the Department of Neurosurgery,Guangzhou Women and Children's Medical Center from October 2014 to October 2018 were retrospectively analyzed.The relevant data,including clinical characteristics,imaging studies,surgical approaches,postoperative pathology,and complications.There were 6 boys and 5 girls,aged 3 to 36 months,with a mean age of 17.4(3-36)months,and preoperative imaging revealed obstructive hydrocephalus.All children receivedThe surgical treatment,approaches including 7 cases using the transcallosal interfornical route approach through the longitudinal fissure(n=7),1 case using the right middle frontal gyrus corticostomy approach(n=1),1 case using the suboccipital supracerebellar approach(n=1),1 case using the temporal parietal corticostomy lateral ventricle trigone approach,(n=1)and 1 case using the suboccipital posterior midline(n=1)approach.Results Of the 11 children,the procedures included 8 children achieved microscopic total resection(n=8),2 had major resection,(n=2)and 1 underwent biopsy alone(n=1).Postoperative pathology revealed atypical teratoid/rhabdoidtumor(AT/RT,n=3)in 3 cases,pineoblastoma(n=2)in 2 cases,pineocytoma(n=2)in 2 cases,pilocytic astrocytoma(n=1)in 1 case,immature teratoma(n=1)in 1 case,pineal primordium tumor(n=1)in 1 case,and mixed embryonal tumor(n=1)in 1 case.There were 5 cases of ventriculoperitoneal shunt(VP shunt)requiring for a non-unsatisfactory relief of postoperative hydrocephalus.Among 8 cases of postoperative subdural effusion,2 cases were improved through subdural external drainage while the remaining,6 cases were absorbed spontaneously.There was no occurrence of death.Conclusions The clinical and pathological features of pineal tumors in infants and toddlers are obviously different from those in adolescents and adults.Treatment strategies should be individualized.The transcallosal anterior interforniceal approach approach is suitable for most of infants with pineal tumors.Making a definite pathological diagnosis is beneficial to the formulation of the subsequent treatment protocolplan.