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Diffuse Midline Glioma – Diffuse Intrinsic Pontine Glioma

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Book cover Brainstem Tumors

Abstract

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.

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Abbreviations

ADC:

Apparent diffusion coefficient

BBB:

Blood-brain barrier

CD44:

Cluster of differentiation 44

CED:

Convection-enhanced delivery

Cho/Cr:

Choline/creatine

CN:

Cranial nerve

CNS:

Central nervous system

CSF:

Cerebrospinal fluid

CT:

Computed tomography

DIPG:

Diffuse intrinsic pontine glioma

DNA:

Deoxyribonucleic acid

DTI:

Diffusion tensor imaging

EGFR:

Epidermal growth factor receptor

EMA:

Epithelial membrane antigen

EZH2:

Enhancer of Zeste homologue 2

FA:

Fractional anisotropy

FLAIR:

Fluid attenuated inversion recovery

GFAP:

Glial fibrillary acidic protein

GFAPδ:

Glial fibrillary acidic protein delta

HATs:

Histone acetyltransferases

HDACs:

Histone deacetylases

ICP:

Intracranial pressure

LPS:

Lansky play scale

MRI:

Magnetic resonance imaging

MRS:

Magnetic resonance spectroscopy

mTOR:

Mammalian target of rapamycin

NAA:

N-acetyl aspartate

Olig2:

Oligodendrocyte transcription factor 2

PDGFR:

Platelet-derived growth factor receptor

PET:

Positron emission tomography

PNET:

Primitive neuroectodermal tumor

PPCs:

Precursor-like cells

PRC2:

Polycomb repressive complex 2

RTK:

Receptor tyrosine kinase

SAHA:

Vorinostat

SPECT:

Single photon emission computed tomography

SWI:

Susceptibility-weighted imaging

TMZ:

Temozolomide

T2∗-GRE:

T2∗-weighted gradient echo

WHO:

World Health Organization

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  1. Department of Neurosurgery, Institute for Brain Protection Sciences, Johns Hopkins University School of Medicine, Johns Hopkins All Children’s Hospital, Saint Petersburg, FL, USA

    Mohammad Hassan A. Noureldine & George I. Jallo

  2. Institute of Neuroscience, Geisinger Medical Center, Geisinger Commonwealth School of Medicine, Danville, PA, USA

    Nir Shimony

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  1. Department of Neurosurgery, Institute for Brain Protection Sciences, Johns Hopkins University School of Medicine, Johns Hopkins All Children’s Hospital, Saint Petersburg, FL, USA

    George I. Jallo

  2. Department of Neurosurgery, Institute for Brain Protection Sciences, Johns Hopkins University School of Medicine, Johns Hopkins All Children’s Hospital, Saint Petersburg, FL, USA

    Mohammad Hassan A. Noureldine

  3. Institute of Neuroscience, Geisinger Medical Center, Geisinger Commonwealth School of Medicine, Danville, PA, USA

    Nir Shimony

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Noureldine, M.H.A., Shimony, N., Jallo, G.I. (2020). Diffuse Midline Glioma – Diffuse Intrinsic Pontine Glioma. In: Jallo, G., Noureldine, M., Shimony, N. (eds) Brainstem Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-38774-7_8

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