Updated: 12/26/2021

Ependymomas

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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
  • 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
  • Prognosis
    • Poor

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