Ependymoma Presenting as a -Rim-Enhancing Lesion in the Brainstem

Fatema Malbari, Guillermo Aldave, Sherri B. Birchansky, Arnold C. Paulino, Dolores H. Lopez-Terrada, Carrie A. Mohila, Sibo Zhao, Murali Chintagumpala

Research output: Contribution to journalArticlepeer-review

Abstract

Introduction: The posterior fossa is the most common intracranial location for pediatric ependymoma. While ependymoma usually arises from the ventricular lining of the fourth ventricle as a solid mass, it rarely originates from the brainstem. Grade II ependymomas also infrequently appear as a cavitary ring-enhancing lesion. Case Presentation: We describe a case of a 6-year-old boy with an ependymoma arising within the medulla with imaging features of a thick-walled rim-enhancing cavitary lesion. A stereotactic biopsy was obtained which confirmed a grade II ependymoma. The patient received focal proton beam radiation therapy and is doing well with no concerns for disease progression at 28 months after diagnosis. Conclusion: Posterior fossa ependymomas typically arise from ependymal cells within the fourth ventricle or foramina of Luschka. They rarely invade or arise within the brainstem parenchyma. Our case had atypical imaging findings in addition to the atypical tumor location. The lesion was described as a thick-walled rim-enhancing focal cystic necrotic lesion centered within the medulla with surrounding nonenhancing expansile infiltrative changes. Ring-enhancing lesions can be seen in patients with anaplastic ependymoma, but is not commonly reported in grade II ependymomas. In summary, this report highlights a unique case of a posterior fossa ependymoma in a pediatric patient arising in an atypical brainstem location as well as having unique imaging features.

Original languageEnglish (US)
Pages (from-to)455-459
Number of pages5
JournalPediatric Neurosurgery
Volume56
Issue number5
DOIs
StatePublished - Sep 1 2021

Keywords

  • Brainstem
  • Ependymoma
  • Pediatric patient
  • Proton Therapy
  • Biopsy
  • Ependymoma/diagnostic imaging
  • Fourth Ventricle/diagnostic imaging
  • Humans
  • Brain Stem/diagnostic imaging
  • Male
  • Child

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Surgery

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