CAJ | 학술논문

目的探讨小于3岁儿童弥漫性桥脑胶质瘤的影像学检查和病理学特点及治疗效果.方法回顾性分析10例年龄小于3岁的儿童桥脑胶质瘤患儿临床表现、肿瘤部位、MRI影像学检查结果和肿瘤组织病理学特点,并以生存期为标准与3岁以上患儿疗效进行对比.结果 10 例确诊患儿的平均年龄为2.2岁(0.8～2.7岁).从出现症状到确诊的平均间间隔时间为2.5个月.所有患儿出现颅神经麻痹,其巾7例合并其他脑干的神经功能缺损症状.MRI检查显示所有患儿为桥脑肿瘤,占脑干肿瘤的50％以上.其巾MRI表现不典型的2例患儿已通过病理学检查证实其为桥脑肿瘤.所有患儿接受治疗,其中2例单纯放疗,6例放疗结合化疗,2例单纯化疗.4例患儿死于肿瘤进展,平均存活时间为0.7年(0.5～3.7年);余6例患儿平均生存时间为2.3年(0.9～8年).3岁以内患儿的3年整体存活率和肿瘤无进展率分别为45％±19％和69％±19％,均高于3岁以上患儿.结论尽管使用相同的治疗方法,年龄小于3岁的脑干胶质瘤儿童较年长患儿预后好,推测可能与年幼患儿脑干胶质瘤病理学类型有关.
목적 탐토소우3세인동미만성교뇌효질류적영상학검사화병이학특점급치료효과.방법 회고성분석10례년령소우3세적인동교뇌효질류환인림상표현、종류부위、MRI영상학검사결과화종류조직병이학특점,병이생존기위표준여3세이상환인료효진행대비.결과 10 례학진환인적평균년령위2.2세(0.8～2.7세).종출현증상도학진적평균간간격시간위2.5개월.소유환인출현로신경마비,기건7례합병기타뇌간적신경공능결손증상.MRI검사현시소유환인위교뇌종류,점뇌간종류적50％이상.기건MRI표현불전형적2례환인이통과병이학검사증실기위교뇌종류.소유환인접수치료,기중2례단순방료,6례방료결합화료,2례단순화료.4례환인사우종류진전,평균존활시간위0.7년(0.5～3.7년);여6례환인평균생존시간위2.3년(0.9～8년).3세이내환인적3년정체존활솔화종류무진전솔분별위45％±19％화69％±19％,균고우3세이상환인.결론 진관사용상동적치료방법,년령소우3세적뇌간효질류인동교년장환인예후호,추측가능여년유환인뇌간효질류병이학류형유관.
Objective Diffuse pontine glioma (DPG) is rare among young children and the outcome of affected patients is unknown.This study thus aimed to investigate the pathological characteristics,imaging and treatment effects of patients under 3 years old.Methods The authors reviewed clinical,imaging tests and pathology characteristic,and compared the outcomes with those more than 3 years old.Results The median age at diagnosis in the 10 patients was 2.2 years (range,0.8-2.7 years).The median interval between the onset of symptoms and diagnosis was 2.5 months.All patients presented with cranial nerve palsy,7 also had associated neurologic deficits attributable to hrainstem involvement.All patients had pons-based tumors involving ＞50 ％ of the brainstem segment.Histological confirmation was attempted in 2 patients who had atypical radiological features at diagnosis.Four patients initially were initially observed.All patients received active therapy,which consisted of radiation therapy (RT) (n =2),RT and chemotherapy (n =6),or chemotherapy only (n =2).Four patients died of tumor progression after a median of 0.7 years ( range,0.5-3.7 years).Six patients survived for a median of 2.3 years (range,0.9-8 years).The 3-year progression-free and overall survival rates were 45％ ± 19％ and 69％ ± 19％ respectively,Both were better than patients who were more than 3 years old.Conclusions Children aged ＜3 years with DPG fare better than older patients with the same diagnosis.The current results would suggest that DPG in younger children may be a biologically distinct entity.