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Snapshot
  • An 8-year-old boy presents to the pediatric emergency department with headache, nausea, and vomiting. His symptoms progressively developed over the course of days. Physical examination is notable for a left sixth nerve palsy and papilledema. MRI brain with and without contrast demonstrates a mass in the fourth ventricle that enhances with gadolinium.
Introduction
  • Overview
    • neoplasm arising from or adjacent to the ependymal cells that line the ventricular system
      • accounts for < 10% of central nervous system tumors
      • most commonly occur in the fourth ventricle or intramedullary spinal cord
  • Epidemiology
    • incidence
      • most common in early childhood
        • though can occur at any age
        • spinal ependymomas are more common in adults
  • Prognosis
    • poor
Presentation
  • Symptoms/physical exam
    • clinical manifestations depend on the location of the tumor
      • most occur in the fourth ventricle, leading to hydrocephalus
        • headache, nausea, and vomiting
        • papilledema
      • seizures or focal neurological deficits
Imaging
  • MRI brain with and without gadolinium 
    • indication
      • imaging study of choice
    • findings
      • enhancing mass in the ventricular system
        • may find enlarge ventricles due to hydrocephalus
Studies
  • Histopathology
    • indication
      • required to confirm the diagnosis and determine if the meningioma is benign or malignant
    • findings 
      • perivascular pseudorosettes 
      • basal ciliary bodies near cellular nuclei
Differential
  • Other malignant brain neoplasm
    • differentiating factors
      • based on histological findings
        • e.g., glioblastomas will have pleomorphic tumor cells surrounding a central area of necrosis
Treatment
  • Medical
    • chemotherapy
      • indication
        • in very young patients
        • bulky residual disease
        • recurrent or refractory tumors
    • radiation therapy
      • indication
        • adjuvant therapy with surgical resection in most cases
  • Surgical
    • resection
      • indication
        • mainstay of treatment
Complications
  • Seizures
  • Hydrocephalus
 

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