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Myxopapillary Ependymoma and Rare Tumors

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Tumors of the Spinal Canal

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Abstract

This chapter explores the details of five intradural extramedullary tumors of the spinal canal, including myxopapillary ependymoma (MPE), ganglioneuroma (GN), paraganglioma (PGL), malignant peripheral nerve sheath tumors (MPNST), and lipomas. While myxopapillary ependymoma, paraganglioma, and lipoma are commonly found in the intradural extramedullary space, ganglioneuroma and MPNST are extremely rare in this location and are more commonly found in other areas of the body. Intradural extramedullary tumors pose the risk of spinal cord compression and associated symptoms, such as pain, weakness, and motor and sensory dysfunction. These tumors are challenging to diagnose due to their rarity, non-specific symptoms, and radiologic similarity to other more common soft tissue tumors, with the exception of lipomas, which are more clearly identifiable and often occur concomitantly with occult spinal dysraphism. Definitive diagnosis of MPE, GN, PGL, and MPNST require histochemical and immunohistochemical assay and analysis for biomarkers, and even so, some are not correctly diagnosed until after they are resected and further analyzed. MPE, GN, PGL, and MPNST are all treated via surgical resection, while adjuvant chemotherapy and radiation for these tumors is still controversial and under investigation. Treatment for lipoma is also surgical resection. Prognosis is generally favorable for MPE, GN, PGL, and lipoma, but very poor for MPNST. This chapter outlines the clinical presentation, demographics, location, pathologic findings, treatment and prognosis, and differential diagnoses of the five tumors, and creates a comprehensive and systematic analysis of rare intradural extramedullary spinal cord tumors.

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Authors and Affiliations

  1. Department of Neurological Surgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA

    John Bruckbauer, James Harrop, Kevin Hines, Stephanie Perez, Victor Sabourin & Anthony Stefanelli

Authors
  1. John Bruckbauer
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  2. James Harrop
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  3. Kevin Hines
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  4. Stephanie Perez
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  5. Victor Sabourin
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  6. Anthony Stefanelli
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Corresponding author

Correspondence to Kevin Hines .

Editor information

Editors and Affiliations

  1. Department of Neurological Surgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA

    Simon Hanft

  2. Department of Neurosurgery, New York-Presbyterian/Columbia University, New York, NY, USA

    Paul C. McCormick

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Bruckbauer, J., Harrop, J., Hines, K., Perez, S., Sabourin, V., Stefanelli, A. (2021). Myxopapillary Ependymoma and Rare Tumors. In: Hanft, S., McCormick, P.C. (eds) Tumors of the Spinal Canal. Springer, Cham. https://doi.org/10.1007/978-3-030-55096-7_3

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