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Pediatric spinal intramedullary anaplastic myxopapillary ependymoma: a case report

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Abstract

A 6-year-old girl presented with a 1-week history of progressive upper and lower extremity weakness and bilateral upper extremity dysesthesia. Imaging demonstrated a 4.7 × 1.2-cm enhancing intramedullary lesion in the cervical spine from level C2 to C5 with associated cystic components and syringomyelia. The patient underwent a C2–C5 laminoplasty, with gross total resection of the intramedullary lesion. Histological analysis showed small to medium-sized epithelioid cells, with predominantly a solid architecture focally infiltrating into the adjacent spinal cord tissue. Focal papillary differentiation was present along with peri-vascular pseudorosettes, mucin microcysts, and globules of dense collagen. Focal anaplasia was noted with mitosis (5/10 HPF), focal necrosis, and elevated Ki67 10–15%. These findings were consistent with a myxopapillary ependymoma with anaplastic features. CSF cytology was negative for tumor cells. MYCN amplification was not present. She was treated with targeted proton-beam radiation therapy. This is the fourth case of an intramedullary anaplastic myxopapillary ependymoma to date, and the first case in the cervical spine reported in the literature.

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Authors and Affiliations

  1. Department of Neurosurgery, Division of Pediatric Neurosurgery, Northwestern University Feinberg School of Medicine/Ann and Robert H. Lurie Children’s Hospital, 225 E. Chicago Avenue, Chicago, IL, 60611, USA

    S. Joy Trybula, Laila M. Mohammad, Sandi K. Lam & Tord D. Alden

  2. Department of Pathology and Laboratory Medicine, Director of Pediatric Neuropathology, Northwestern University Feinberg School of Medicine/Ann and Robert H. Lurie Children’s Hospital, Chicago, IL, USA

    Nitin R. Wadhwani

  3. Department of Pediatrics, Division of Hematology, Oncology, Neuro-Oncology and Stem Cell Transplantation, Northwestern University Feinberg School of Medicine/Ann and Robert H. Lurie Children’s Hospital, Chicago, IL, USA

    Alicia C. Lenzen

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  1. S. Joy Trybula
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Correspondence to Tord D. Alden.

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Trybula, S.J., Wadhwani, N.R., Mohammad, L.M. et al. Pediatric spinal intramedullary anaplastic myxopapillary ependymoma: a case report. Childs Nerv Syst 38, 223–227 (2022). https://doi.org/10.1007/s00381-021-05171-2

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