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Rare Childhood Tumors: Desmoplastic Infantile Ganglioglioma and Pleomorphic Xanthoastrocytoma

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Oncology of CNS Tumors

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Abstract

Desmoplastic infantile ganglioglioma (DIG) and pleomorphic xanthoastrocytoma (PXA) are both rare brain tumors but also important to recognize. They typically respond well to surgery alone without a need for adjuvant therapy. Particularly for PXA, the identification of the BRAF V600E mutation has aided in diagnosis and has potential benefit for therapy as well. This chapter will review the presentation, diagnosis, treatment, and prognosis of these entities.

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Acknowledgments

I would like to thank Dr. Murat Gokden, UAMS Department of Pathology and Chief of Neuropathology, for the pathology images and captions. I would like to thank Dr. Charles Glasier, UAMS Department of Radiology and ACH Chief of Neuroradiology, for the radiographs.

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Authors and Affiliations

  1. Division of Neurosurgery, Arkansas Children’s Hospital, Little Rock, AR, USA

    Gregory W. Albert

  2. Department of Neurosurgery, University of Arkansas for Medical Sciences, Little Rock, AR, USA

    Gregory W. Albert

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  1. Gregory W. Albert
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Correspondence to Gregory W. Albert .

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Editors and Affiliations

  1. Department of Neurosurgery, University of Munich Department of Neurosurgery, Munich, Germany

    Jörg-Christian Tonn

  2. Dana-Farber Cancer Institute Center, Harvard University, Boston, USA

    David A. Reardon

  3. SickKids Hospital, University of Toronto, Toronto, ON, Canada

    James T. Rutka

  4. Department of Neurosurgery, University of Hamburg, Hamburg, Germany

    Manfred Westphal

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Albert, G.W. (2019). Rare Childhood Tumors: Desmoplastic Infantile Ganglioglioma and Pleomorphic Xanthoastrocytoma. In: Tonn, JC., Reardon, D., Rutka, J., Westphal, M. (eds) Oncology of CNS Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-04152-6_26

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  • DOI: https://doi.org/10.1007/978-3-030-04152-6_26

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