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Updated: Aug 14, 2025
A Protocol for Rapid Post-mortem Cell Culture of Diffuse Intrinsic Pontine Glioma DIPG
Published on: March 7, 2017
Advances and Clinical Trials Update in the Treatment of Diffuse Intrinsic Pontine Gliomas
Cecilia Dalle Ore1, Christina Coleman2, Nalin Gupta1,3
1Department of Neurological Surgery, University of California San Francisco, San Francisco, California, USA.
Insights
Diffuse intrinsic pontine gliomas (DIPG) are distinct pediatric brain tumors. Biopsies reveal unique mutations, paving the way for targeted therapies and improved understanding of this aggressive disease.
Area of Science:
- Pediatric neuro-oncology
- Molecular pathology of brain tumors
- Cancer genetics
Background:
- Diffuse intrinsic pontine gliomas (DIPGs) are aggressive pediatric brain tumors.
- They represent a major cause of mortality in children with brain tumors, with a median survival of 8-11 months.
- DIPGs are biologically distinct from adult high-grade gliomas (HGGs).
Purpose of the Study:
- To highlight the distinct biological nature of DIPG compared to adult HGG.
- To emphasize the safety and diagnostic value of stereotactic biopsy in DIPG.
- To discuss the current limitations and future directions for personalized medicine in DIPG.
Main Methods:
- Review of emerging data on DIPG biology and treatment.
- Analysis of studies demonstrating the safety and efficacy of stereotactic biopsy.
- Examination of current therapeutic strategies and ongoing investigations.
Main Results:
- DIPG is characterized by specific mutations in histone genes (H3.3 and H3.1) not typically found in adult gliomas.
- Stereotactic biopsy is a safe procedure for DIPG, providing crucial diagnostic information.
- Identification of distinct driving mutations offers potential therapeutic targets.
Conclusions:
- DIPG is a unique entity requiring distinct therapeutic approaches.
- Biopsy-driven molecular profiling is essential for advancing personalized medicine in DIPG.
- Novel treatment modalities, including targeted therapies and immunotherapies, are under active investigation.
Background:
Diffuse intrinsic pontine gliomas (DIPGs) are high-grade gliomas (HGGs) that occur primarily in children, and represent a leading cause of death in pediatric patients with brain tumors with a median overall survival of only 8-11 months.
Summary:
While these lesions were previously thought to behave similarly to adult HGG, emerging data have demonstrated that DIPG is a biologically distinct entity from adult HGG frequently driven by mutations in the histone genes H3.3 and H3.1 not found in adult glioma. While biopsy of DIPG was historically felt to confer unacceptable risk of morbidity and mortality, multiple studies have demonstrated that stereotactic biopsy of DIPG is safe, allowing not only for improved understanding of DIPG but also forming the basis for protocols for personalized medicine in DIPG. However, current options for personalized medicine in DIPG are limited by the lack of efficacious targeted therapies for the mutations commonly found in DIPG. Multiple treatment modalities including targeted therapies, immunotherapy, convection-enhanced delivery, and focused ultrasound are in various stages of investigation.
Key Message:
Increasing frequency of biopsy for DIPG has identified distinct driving mutations that may serve as therapeutic targets. Novel treatment modalities are under investigation.
