Analysis of clinical efficacy of treatment and characteristics of diffuse pontine glioma in children less than 3 years old

Abstract

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. Key words: Child; Pontine neoplasms; Glioma