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High-Grade Tumors of the Brainstem (Except DIPG)

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Brainstem Tumors

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Abstract

High-grade tumors of the brainstem in adults and children, excluding diffuse intrinsic pontine glioma (DIPG), are rare. In adults, these tumors are almost exclusively malignant gliomas, while in children approximately 60% are high-grade gliomas and 40% are embryonal tumors. In almost all cases, the most common presenting symptoms are cranial neuropathies, hemiparesis, and ataxia, typically of short duration. Imaging characteristics are similar to the corresponding histologic counterparts arising in other locations of the brain; however, diagnosis can only be made by histologic confirmation following biopsy. In the overwhelming majority of cases, at least surgical biopsy or partial resection can be performed, and this intervention is the first step in the treatment of these tumors. Unfortunately, these tumors are in general rapidly progressive and relatively refractory to multimodality therapy with surgery, irradiation, and chemotherapy. Radiation may provide a degree of tumor stabilization in some cases, but the efficacy of chemotherapy remains limited, and the overall prognosis is dismal, with most patients succumbing to their disease within a year. Ultimately, it is hoped that the unique genetic and molecular alterations of this diverse group of tumors in both adults and children will afford the opportunity for the development of targeted therapeutics for improved outcomes.

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Abbreviations

ATRT:

Atypical teratoid rhabdoid tumor

CCNU:

Lomustine

CNS-PNET:

Central nervous system primitive neuroectodermal tumor

DIPG:

Diffuse intrinsic pontine glioma

ETMR:

Embryonal tumor with multilayered rosettes

FISH:

Fluorescence in situ hybridization

GBM:

Glioblastoma

HDC:

High-dose chemotherapy

IDH1:

Isocitrate dehydrogenase-1

IMRT:

Intensity-modulated radiation therapy

MGMT:

O6-methylguanine–DNA methyltransferase

MRI:

Magnetic resonance imaging

OS:

Overall survival

PCV:

Vincristine

PFS:

Progression-free survival

PTEN:

Phosphatase and tensin homolog

WHO:

World Health Organization

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Author information

Authors and Affiliations

  1. University of Cincinnati Brain Tumor Center, Cincinnati, OH, USA

    Soma Sengupta

  2. University of Cincinnati Medical Center, Cincinnati, OH, USA

    Daniel Pomeranz Krummel

  3. Department of Radiology and Imaging Sciences, Emory University School of Medicine, Atlanta, GA, USA

    Brent D. Weinberg

  4. Department of Pediatrics, Emory University School of Medicine, Aflac Cancer & Blood Disorders Center, and Children’s Healthcare of Atlanta, Atlanta, GA, USA

    Tobey J. MacDonald

Authors
  1. Soma Sengupta
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  2. Daniel Pomeranz Krummel
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  3. Brent D. Weinberg
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  4. Tobey J. MacDonald
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Editor information

Editors and Affiliations

  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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Sengupta, S., Krummel, D.P., Weinberg, B.D., MacDonald, T.J. (2020). High-Grade Tumors of the Brainstem (Except DIPG). In: Jallo, G., Noureldine, M., Shimony, N. (eds) Brainstem Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-38774-7_7

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  • DOI: https://doi.org/10.1007/978-3-030-38774-7_7

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  • Publisher Name: Springer, Cham

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  • Online ISBN: 978-3-030-38774-7

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