Diffuse intrinsic pontine gliomas: Diagnostic approach and treatment strategies

M Burhan Janjua1, Vin Shen Ban2, Tarek Y El Ahmadieh2

  • 1Division of Pediatric Neurosurgery, Department of Neurosurgery, UT Southwestern Medical Center, Dallas, TX, United States; Department of Neurosurgery, Shriners Hospital for Children, Philadelphia, PA, United States; Department of Neurosurgery, University of Pennsylvania Hospital, United States.

Insights

Diffuse intrinsic pontine gliomas (DIPG) require molecular research beyond histopathology. Neuronavigation-assisted biopsy provides crucial tumor tissue for identifying genetic mutations and developing targeted therapies for this fatal brainstem glioma.

Area of Science:

  • Neuro-oncology
  • Molecular Pathology
  • Surgical Neurology

Background:

  • Diffuse intrinsic pontine gliomas (DIPG) are aggressive brainstem tumors with poor prognoses.
  • Current treatments, including radiation, offer limited long-term efficacy, with frequent relapses.
  • Histopathological diagnosis alone is insufficient for understanding DIPG molecular heterogeneity and identifying therapeutic targets.

Purpose of the Study:

  • To emphasize the critical need for molecular research in DIPG.
  • To discuss the importance of obtaining tumor tissue for advanced molecular studies.
  • To advocate for improved diagnostic and therapeutic strategies for DIPG.

Main Methods:

  • Review of current research on DIPG molecular subgroups and therapeutic targets.
  • Discussion of neuronavigation-assisted stereotactic biopsy for tumor tissue acquisition.
  • Exploration of techniques for identifying histone protein genetic mutations and methylation changes.
  • Overview of convection-enhanced delivery (CED) for therapeutic agent instillation.

Main Results:

  • Histopathological diagnosis is inadequate for DIPG molecular subtyping.
  • Neuronavigation-assisted stereotactic biopsy is a feasible technique for obtaining tumor tissue.
  • Molecular analysis can identify genetic/epigenetic mutations and biomarkers.
  • Convection-enhanced delivery shows promise for future drug delivery.

Conclusions:

  • Acquiring tumor tissue via stereotactic biopsy is essential for advancing DIPG research.
  • Molecular profiling is crucial for understanding DIPG oncogenesis and developing targeted therapies.
  • Further pre-clinical and clinical research is necessary to overcome the challenges posed by DIPG.

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