Ependymoma

Ependymomas are gliomas that may occur intracranial, but mainly in the spinal cord. They arise from the cells of the ependyma, at thin epithelial membrane lining the ventricular system of the brain and the spinal cord.

    
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Different Types of Ependymomas

  • subependymoma (WHO grade I)
  • myxopapillary ependymoma (WHO grade I)
  • cellular ependymoma (WHO grade II)
  • papillary ependymoma (WHO grade II)
  • clear cell ependymoma (WHO grade III)
  • anaplastic ependymoma (WHO grade III)

 

Histology of Ependymoma

  • glial tumour with ependymal differentiation and characteristic rosette structure
  • very slowly growing grade-I-ependymoma
  • slowly growing, differenciated and regularily built grade-II-ependymoma
  • anaplastic ependymoma (grade III)

 

Epidemiology of Ependymoma

  • peak age in childhood around 5, in adulthood between 30 and 40
  • third most occuring brain tumour in children

 

Symptoms of Ependymoma

  • vary, depending on tumour localisation
  • possibly interference of flow of cerebrospinal fluid caused by localisation in ventricular sytem
  • increase of cerebral pressure, sometimes hydrocephalus
  • symptoms of cerebral pressure (e.g. vertigo, headache, emesis)
  • in infants mainly developmental delays, tendency to fall

 

Diagnosing Ependymoma

  • diagnosis with MRI of the entire subarachnoid space, maybe examination of the liquor
  • lesion with contact to ventricular system
  • varying enhancement of contrast agent
  • in 50% calcifications
  • main localisation: posterior fossa, lateral ventricles and third ventricle; in adulthood mainly spinal cord
  • higher risk for patients with Neurofibromatosis type II

 

Therapy of Ependymoma

  • surgery (if possible complete surgical removal) of brain tumour
  • radiotherapy after incomplete surgical removal or grade II and III
  • irradiation of subarachnoid space if dissemination in liquor
  • chemotherapy (in children and patients at increased risk within clinical trials)

 

Therapy of Recurrent Ependymoma

  • need for interdisciplinary therapeutic decision-making
  • renewed surgery
  • (second) irradiation
  • subsequently chemotherapy should be considered

 

Aftercare of Ependymoma

  • first check-up 6 weeks after therapy

 

Course of Ependymoma

  • ependymoma with WHO-grade I and II: slow, clearly defined growth
  • ependymoma with WHO-grade III: relatively fast, infiltrating growth
  • sometimes recurrences even after 15 years

 

Prognosis of Ependymoma

  • great differences depending on metastasis, localisation, remains of tumour and age
  • complete surgical removal of brain tumour leads to significantly better prognosis

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