Pediatric spinal intramedullary anaplastic myxopapillary ependymoma: a case report

S. Joy Trybula, Nitin Ramesh Wadhwani, Laila M. Mohammad, Sandi K. Lam, Alicia Lenzen, Tord D Alden*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
JournalChild's Nervous System
DOIs
StateAccepted/In press - 2021

Keywords

  • Anaplasia
  • Cervical spine
  • Intramedullary
  • Myxopapillary ependymoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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