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Updated: Jul 5, 2026
A Protocol for Rapid Post-mortem Cell Culture of Diffuse Intrinsic Pontine Glioma (DIPG)
Published on: March 7, 2017
Young age may predict a better outcome for children with diffuse pontine glioma
Alberto Broniscer1, Fred H Laningham, Robert P Sanders
1Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA. alberto.broniscer@stjude.org
Insights
Young children with diffuse pontine glioma (DPG) may have a better prognosis than older patients. This study suggests DPG in younger children might be biologically distinct, warranting further investigation.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Pediatric Neurosurgery
Background:
- Diffuse pontine glioma (DPG) is a rare brain tumor in young children, making its prognosis uncertain.
- Understanding outcomes for DPG in this age group is crucial for treatment planning.
Purpose of the Study:
- To investigate the clinical and radiologic characteristics of DPG in children under 3 years old.
- To determine the outcome and survival rates for this specific pediatric DPG population.
Main Methods:
- Retrospective review of 10 patients under 3 years old diagnosed with DPG.
- Analysis of clinical presentation, imaging findings, treatment modalities, and survival data.
Main Results:
- Median age at diagnosis was 2.2 years, with symptoms presenting 2.5 months prior.
- All patients had pons-based tumors; 7 presented with cranial nerve palsy.
- Three-year progression-free and overall survival rates were 45% and 69%, respectively, with a median follow-up of 2.3 years.
Conclusions:
- Children under 3 years with DPG may have a more favorable outcome compared to older patients.
- DPG in younger children might possess distinct biological characteristics influencing prognosis.
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.
