Diffuse Midline Glioma – Diffuse Intrinsic Pontine Glioma

  • Mohammad Hassan A. Noureldine
  • Nir Shimony
  • George I. JalloEmail author


Diffuse midline glioma, H3 K27M-mutant, formerly known as diffuse intrinsic pontine glioma (DIPG), is a malignant and infiltrative neoplasm of the pons. DIPG mainly affects the pediatric population and is associated with dismal prognosis, where less than 10% of sufferers survive beyond 2 years from diagnosis. The diagnosis was based upon the typical clinical presentation/progression and imaging features, and biopsy was discouraged at some point due to the associated surgical toxicity. The clinical interest and exponential growth in the number of molecular studies during the last decade have lead to the discovery of selective driver mutations. Consequently, biopsy regained popularity due to the importance of obtaining tissue for diagnostic and research purposes, and therapeutic trials are shifting from conventional strategies (radiation and chemotherapy) towards targeting unique molecular mechanisms. In this chapter, we present an overview of the current standards as well as the most important recent and clinically-relevant advances in the diagnosis and management of DIPG.


Diffuse intrinsic pontine glioma Diffuse midline glioma H3 K27M-mutant Brainstem Tumor Pons Diagnosis Cranial nerve palsies Magnetic resonance imaging Biopsy Molecular studies Management Radiation Chemotherapy Targeted therapy 



Apparent diffusion coefficient


Blood-brain barrier


Cluster of differentiation 44


Convection-enhanced delivery




Cranial nerve


Central nervous system


Cerebrospinal fluid


Computed tomography


Diffuse intrinsic pontine glioma


Deoxyribonucleic acid


Diffusion tensor imaging


Epidermal growth factor receptor


Epithelial membrane antigen


Enhancer of Zeste homologue 2


Fractional anisotropy


Fluid attenuated inversion recovery


Glial fibrillary acidic protein


Glial fibrillary acidic protein delta


Histone acetyltransferases


Histone deacetylases


Intracranial pressure


Lansky play scale


Magnetic resonance imaging


Magnetic resonance spectroscopy


Mammalian target of rapamycin


N-acetyl aspartate


Oligodendrocyte transcription factor 2


Platelet-derived growth factor receptor


Positron emission tomography


Primitive neuroectodermal tumor


Precursor-like cells


Polycomb repressive complex 2


Receptor tyrosine kinase




Single photon emission computed tomography


Susceptibility-weighted imaging




T2∗-weighted gradient echo


World Health Organization


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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Mohammad Hassan A. Noureldine
    • 1
  • Nir Shimony
    • 2
  • George I. Jallo
    • 1
    Email author
  1. 1.Department of Neurosurgery, Institute for Brain Protection Sciences, Johns Hopkins University School of Medicine, Johns Hopkins All Children’s HospitalSaint PetersburgUSA
  2. 2.Institute of Neuroscience, Geisinger Medical Center, Geisinger Commonwealth School of MedicineDanvilleUSA

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