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Oligodendroglial Tumors

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Molecular Pathology of Nervous System Tumors

Part of the book series: Molecular Pathology Library ((MPLB,volume 8))

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Abstract

Oligodendroglioma (ODG) is a subtype of glioma marked by unique clinical, pathological, and genetic characteristics. These features include: validated molecular biomarkers for both diagnosis and clinical treatment decisions, sensitivity to radiation and cytotoxic chemotherapy, and unique imaging and histologic features. Despite several favorable properties, these tumors demonstrate infiltrative growth similar to other gliomas and eventually progress to becoming a multi-agent resistant cancer resulting in death. Current efforts are focused on capitalizing the strong association of anaplastic oligodendroglioma (AO) with co-deletions of chromosomes 1p and 19q to develop more effective and better tolerated treatment strategies via discovery of new cancer-associated mutations. Specifically, recent developments in brain tumor genomics have highlighted the distinctiveness of 1p/19q co-deleted ODG such that these tumors display a proneural gene expression signature and are associated with a constellation of positive prognostic molecular features including methylation of the MGMT promoter and somatic mutations of the IDH genes. Clinically, level one evidence for the benefit of radiation and chemotherapy for newly diagnosed 1p/19q co-deleted AO has recently been reported, however, there is remaining controversy about the optimal timing and types of therapies to use to maximize survival benefit and minimize toxicity for many patients with ODG, particularly for low-grade oligodendrogliomas (LGO). Both the preclinical and clinical branches of translational research targeting ODG are very active in the hopes of applying technologies such as next generation sequencing (NGS) to allow rapid, cost-effective “deep-sequencing” of cancer genomes along the spatial and temporal axes of tumor development to the development of new therapies for ODG patients.

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Authors and Affiliations

  1. Department of Pathology & Laboratory Medicine, Vancouver General Hospital, 899 W12th Avenue, Vancouver, BC, Canada, V5Z1M9

    Stephen Yip M.D., Ph.D.

  2. The Johns Hopkins Comprehensive Neurofibromatosis Center, Baltimore, MD, 21231, USA

    Jaishri Blakeley M.D.

  3. Department of Neurology, The Johns Hopkins Hospital, CRB II, Suite 1M16, 1550 Orleans Street, Baltimore, MD, 21231, USA

    Jaishri Blakeley M.D.

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  1. Stephen Yip M.D., Ph.D.
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  2. Jaishri Blakeley M.D.
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Correspondence to Stephen Yip M.D., Ph.D. .

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Editors and Affiliations

  1. Associate Professor of Pediatrics and Otolaryngology, NYU Langone Medical Center, New York, New York, USA

    Matthias A. Karajannis

  2. Professor of Pathology and Neurosurgery, NYU Langone Medical Center, New York, New York, USA

    David Zagzag

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Yip, S., Blakeley, J. (2015). Oligodendroglial Tumors. In: Karajannis, M., Zagzag, D. (eds) Molecular Pathology of Nervous System Tumors. Molecular Pathology Library, vol 8. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1830-0_8

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