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Childhood Ependymoma Treatment (PDQ®)

Health Professional Version
Last Modified: 05/10/2012

Treatment Option Overview

Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary Levels of Evidence 1 for more information.)

Many of the improvements in survival in childhood cancer have been made as a result of clinical trials that have attempted to improve on the best available, accepted therapy. Clinical trials in pediatrics are designed to compare new therapy with therapy that is currently accepted as standard. This comparison may be done in a randomized study of two treatment arms or by evaluating a single new treatment and comparing the results with those previously obtained with existing therapy.

Because of the relative rarity of cancer in children, all patients with aggressive brain tumors should be considered for entry into a clinical trial. To determine and implement optimum treatment, treatment planning by a multidisciplinary team of cancer specialists who have experience treating childhood brain tumors is required. Radiation therapy of pediatric brain tumors is technically very demanding and should be carried out in centers that have experience in that area in order to ensure optimal results.

Treatment for childhood ependymoma has included surgery followed by standard fractionated radiation therapy. There is evidence to suggest that more extensive surgical resections are related to an improved rate of survival.[1-7] Chemotherapy has been shown to be active in patients with recurrent ependymoma.[8] One relatively small, prospective, randomized trial suggests that chemotherapy activity in newly diagnosed cases is limited,[9] and current treatment approaches do not include chemotherapy as a component of primary therapy for most children with newly diagnosed ependymomas that are completely resected. Children younger than 3 years are particularly susceptible to the adverse effect of radiation on brain development.[10][Level of evidence: 3iiiC] Debilitating effects on growth and neurologic development have frequently been observed, especially in younger children.[11-13] For this reason, conformal radiation approaches, such as 3-dimensional conformal radiation therapy, that minimize damage to normal brain tissue and charged-particle radiation therapy, such as proton beam therapy, are under evaluation for infants and children with ependymoma.[14,15] Long-term management of these patients is complex and requires a multidisciplinary approach.

There is evidence that surveillance neuroimaging in childhood ependymoma will identify tumors that have recurred when the patient is asymptomatic; however, it is unclear whether this detection will change the ultimate prognosis of the patient.[16]

References

  1. Pollack IF, Gerszten PC, Martinez AJ, et al.: Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37 (4): 655-66; discussion 666-7, 1995.  [PUBMED Abstract]

  2. Horn B, Heideman R, Geyer R, et al.: A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21 (3): 203-11, 1999 May-Jun.  [PUBMED Abstract]

  3. van Veelen-Vincent ML, Pierre-Kahn A, Kalifa C, et al.: Ependymoma in childhood: prognostic factors, extent of surgery, and adjuvant therapy. J Neurosurg 97 (4): 827-35, 2002.  [PUBMED Abstract]

  4. Abdel-Wahab M, Etuk B, Palermo J, et al.: Spinal cord gliomas: A multi-institutional retrospective analysis. Int J Radiat Oncol Biol Phys 64 (4): 1060-71, 2006.  [PUBMED Abstract]

  5. Kothbauer KF: Neurosurgical management of intramedullary spinal cord tumors in children. Pediatr Neurosurg 43 (3): 222-35, 2007.  [PUBMED Abstract]

  6. Zacharoulis S, Ji L, Pollack IF, et al.: Metastatic ependymoma: a multi-institutional retrospective analysis of prognostic factors. Pediatr Blood Cancer 50 (2): 231-5, 2008.  [PUBMED Abstract]

  7. Merchant TE, Li C, Xiong X, et al.: Conformal radiotherapy after surgery for paediatric ependymoma: a prospective study. Lancet Oncol 10 (3): 258-66, 2009.  [PUBMED Abstract]

  8. Goldwein JW, Glauser TA, Packer RJ, et al.: Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors. Cancer 66 (3): 557-63, 1990.  [PUBMED Abstract]

  9. Evans AE, Anderson JR, Lefkowitz-Boudreaux IB, et al.: Adjuvant chemotherapy of childhood posterior fossa ependymoma: cranio-spinal irradiation with or without adjuvant CCNU, vincristine, and prednisone: a Childrens Cancer Group study. Med Pediatr Oncol 27 (1): 8-14, 1996.  [PUBMED Abstract]

  10. von Hoff K, Kieffer V, Habrand JL, et al.: Impairment of intellectual functions after surgery and posterior fossa irradiation in children with ependymoma is related to age and neurologic complications. BMC Cancer 8: 15, 2008.  [PUBMED Abstract]

  11. Packer RJ, Sutton LN, Atkins TE, et al.: A prospective study of cognitive function in children receiving whole-brain radiotherapy and chemotherapy: 2-year results. J Neurosurg 70 (5): 707-13, 1989.  [PUBMED Abstract]

  12. Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80 (6): 1004-10, 1994.  [PUBMED Abstract]

  13. Packer RJ, Sutton LN, Goldwein JW, et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 74 (3): 433-40, 1991.  [PUBMED Abstract]

  14. Merchant TE, Mulhern RK, Krasin MJ, et al.: Preliminary results from a phase II trial of conformal radiation therapy and evaluation of radiation-related CNS effects for pediatric patients with localized ependymoma. J Clin Oncol 22 (15): 3156-62, 2004.  [PUBMED Abstract]

  15. MacDonald SM, Safai S, Trofimov A, et al.: Proton radiotherapy for childhood ependymoma: initial clinical outcomes and dose comparisons. Int J Radiat Oncol Biol Phys 71 (4): 979-86, 2008.  [PUBMED Abstract]

  16. Good CD, Wade AM, Hayward RD, et al.: Surveillance neuroimaging in childhood intracranial ependymoma: how effective, how often, and for how long? J Neurosurg 94 (1): 27-32, 2001.  [PUBMED Abstract]





Glossary Terms

3-dimensional conformal radiation therapy (3-dih-MEN-shuh-nul kun-FOR-mul RAY-dee-AY-shun THAYR-uh-pee)
3-dimensional conformal radiation therapy involves the use of computed tomography (CT) imaging in the planning of radiation therapy. The CT scan provides not only 3-dimensional imaging of the target and surrounding normal tissues, but also information about tissue density and tissue depth from the skin to the target. These parameters are critical in calculating the dose distribution. In addition to CT imaging, supplemental imaging modalities, such as magnetic resonance imaging or positron emission tomography, can be used to improve target delineation. With 3-dimensional conformal radiation therapy, conformal beams are used to shape the dose delivered to the target, and wedges or compensators can be used to optimize the dose distribution. Conformal beams are shaped either with a high-density material (e.g., Cerrobend) that allows beam contouring or with multi-leaf collimators, which are an array of high-density leaves (usually tungsten) situated in the head of the linear accelerator (LINAC) whose position is controlled via independent stepping motors that allow beam shaping. Wedges are high-density devices that are placed on the head of the LINAC to act as a tissue compensator and/or beam modifier. The effect of a wedge can be created by a moving jaw at the head of the LINAC. With 3-dimensional conformal radiation therapy, variable field weighting and/or use of different energies (higher energies are more penetrating) are additional tools that enable optimization of the dose distribution. Also called 3-dimensional radiation therapy and 3D-CRT.
charged-particle radiation therapy (… PAR-tih-kul RAY-dee-AY-shun THAYR-uh-pee)
Charged particles (such as protons and carbon) can be used to deliver therapeutic radiation. A proton is the charged nucleus of a hydrogen atom (hydrogen atom minus an electron). Standard radiation is delivered with a linear accelerator (LINAC) that delivers photon therapy (akin to high energy light), while protons and other charged particles are generated from a cyclotron. The difference between charged-particle and photon irradiation is that charged particles stop abruptly in the tissue (Bragg peak), so there is less exit dose through normal tissue. A disadvantage of charged-particle therapy is the greater neutron exposure compared with essentially none using photons, and thus the benefit of protons in reducing radiation-associated malignancies is not known and controversial. Proton therapy can be used to deliver intensity-modulated radiation therapy, stereotactic radiation therapy, or stereotactic radiosurgery.
Level of evidence 3iiiC
Nonconsecutive case series with carefully assessed quality of life as an endpoint. See Levels of Evidence for Adult and Pediatric Cancer Treatment Studies (PDQ®) for more information.

Table of Links

1http://www.cancer.gov/cancertopics/pdq/levels-evidence-adult-treatment/HealthPr
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