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Brain Cancer Health Center

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Childhood Ependymoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Newly Diagnosed Childhood Ependymoma or Anaplastic Ependymoma

Standard treatment options for newly diagnosed ependymoma (WHO Grade II) or anaplastic ependymoma (WHO Grade III) include the following:

  1. Surgery.
  2. Adjuvant therapy.
    • Treatment options for no residual disease, no disseminated disease.
    • Treatment options for residual disease, no disseminated disease.
    • Treatment options for central nervous system (CNS) disseminated disease.
    • Treatment options for children younger than 3 years.

Typically, all patients undergo surgery to remove the tumor. Whether additional treatment is given depends on the extent of tumor resection and whether there is disseminated disease.

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Surgery should be performed in an attempt at maximal tumor reduction. Evidence suggests that more extensive surgical resection is related to an improved rate of survival.[1,2,3,4,5]; [6][Level of evidence: 3iDii] Magnetic resonance imaging (MRI) should be performed postoperatively to confirm the extent of resection. If not performed preoperatively, MRI of the entire neuraxis to evaluate disease dissemination and cerebrospinal fluid cytopathology should be performed.

Patients with residual tumor or disseminated disease should be considered at high risk for relapse and should be treated on protocols specifically designed for them. Those with no evidence of residual tumor still have an approximate 20% to 40% relapse risk in spite of postoperative radiation therapy.

Anecdotal experience suggests that surgery alone for completely resected supratentorial nonanaplastic tumors and intradural spinal cord ependymomas may, in select cases, be an appropriate approach to treatment.[7,8][Level of evidence: 3iiiDi]; [9,10][Level of evidence: 3iiiDiii]

Adjuvant Therapy

Treatment options for no residual disease, no disseminated disease

Radiation therapy

The traditional postsurgical treatment for these patients has been radiation therapy consisting of 54 Gy to 55.8 Gy to the tumor bed for children aged 3 years and older.[11] It is not necessary to treat the entire CNS (whole brain and spine) because these tumors usually recur initially at the local site.[12]; [13][Level of evidence: 3iiiA] When possible, patients should be treated in a center experienced with the delivery of highly conformal radiation therapy (including intensity-modulated radiation therapy or charged-particle radiation therapy) to pediatric patients with brain tumors.

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