Ependymomas

Summary

  • Ependymoma accounts for 8-10% of all childhood CNS tumors
  • < 170 new pediatric cases per year in US
  • The mean age at diagnosis ranges from 51-71 months
  • 25 to 40% are diagnosed in children less than 3 years of age.
  • 5-OS 50-64%
  • 5-PFS 23-45%
  • median time to recurrence of 13-25 months
  • 20% of failures have isolated distant recurrence.

Etiology and Epidemiology

  • mean age at presentation for children is 5 years
  • mean age at presentation for adults is 3rd and 4th decades of life

Clinical manifestation

  • Infratentorial ependymoma may be suspected on the basis of posterior fossa symptomatology
  • Posterior fossa symptomatology
    • headache, vomiting, ataxia, neck stiffness or headtilt
    • CN 6, 7, 8 defects
    • hydrochephalus
    • long tract signs
Spinal Seeding Rate
overall 6.9%
infratentorial 9.7%
supratentorial 1.6%
anaplastic 8.4%
low-grade 4.5%
infratentorial and high-grade 15.7
supratentorial and low-grade 2.7%

Treatment

Surgery

  • maximum safe resection in all cases
  • Gross total resection is the most important prognostic factor in patient > 18 mo. of age

Chemotherapy

  • chemotherapy to defer RT in younger (< 3 years old) patients, otherwise no defined role

There are no data to suggest an obvious role for chemotherapy for patients with ependymoma, especially those greater than 3 years of age at the time of presentation. The poor outcome for children less than 3 years of age 11 has been attributed in part to the delay in the administration of radiation therapy, which suggests that the approach for this
important group of patients with ependymoma should be re-evaluated.

Radiation "Standard"

  • post-operative radiation therapy directed at the primary site for all patients.
  • Previously “Local” RT encompassing the entire posterior fossa to C-2 to 45 Gy
  • Currently CTV (pre-op gross and macroscopic residual disease) + 1.0 cm conformal therapy to > 55.8 Gy [1]
    • The group included very young children with a median age of 3.0 years (range: 1.1 to 22.9 years).
    • Both failures were encompassed by the prescription isodose.
    • The majority of patients received a total dose of 59.4 Gy.
Bibliography
1. Merchant TE, Zhu Y, Thompson SJ, et al: Preliminary results from a phase II trial of conformal
radiation therapy for pediatric patients with localized low-grade astrocytoma and ependymoma. Int J Radiat Oncol Biol Phys 52:325-332, 2002
Unless otherwise stated, the content of this page is licensed under Creative Commons Attribution-Share Alike 2.5 License.