Young age may predict a better outcome for children with diffuse pontine glioma.
BACKGROUND
Because diffuse pontine glioma (DPG) is rare among young children, the outcome of affected patients is unknown.


METHODS
The authors reviewed clinical and radiologic characteristics of all children aged <3 years with DPG who were evaluated at their institution. Inclusion followed standard magnetic resonance imaging criteria for the diagnosis of DPG.


RESULTS
The median age at diagnosis in 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 with (n = 7) or without (n = 3) other neurologic deficits attributable to brainstem involvement. All patients had pons-based tumors involving >50% of this brainstem segment. Histologic confirmation was attempted in 2 patients who had atypical radiologic features at diagnosis. Four patients initially were observed only. All patients received 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 have 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.


CONCLUSIONS
Children aged <3 years with DPG potentially may fare better than older patients with the same diagnosis despite the use of similar therapy. The current results suggested that DPG in younger children may be distinct biologically from similar tumors in older age groups.
Broniscer
Alberto
A
Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA. alberto.broniscer@stjude.org
Laningham
Fred H
FH
Sanders
Robert P
RP
Kun
Larry E
LE
Ellison
David W
DW
Gajjar
Amar
A
D000367:Age Factors; D020295:Brain Stem Neoplasms / Q000175:diagnosis* / Q000628:therapy; D002675:Child, Preschool; D018572:Disease-Free Survival; D042241:Early Diagnosis; D005260:Female; D005910:Glioma / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome