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Childhood Ependymoma Treatment (PDQ®)

Health Professional Version
Last Modified: 05/10/2012

Treatment of Recurrent Childhood Ependymoma

Treatment Options Under Clinical Evaluation
Current Clinical Trials

Recurrence is not uncommon in both benign and malignant childhood brain tumors and may develop many years after initial treatment.[1] For ependymoma, late recurrence beyond 10 to 15 years has been reported.[2,3] Disease generally recurs at the primary tumor site, even in children with malignant ependymomas.[4,5] Systemic relapse is extremely rare. At time of relapse, a complete evaluation for extent of recurrence is indicated for all patients. The need for surgical intervention must be individualized on the basis of the extent of the tumor, the length of time between initial treatment and the reappearance of the recurrent lesion, and the clinical picture. Patients with recurrent ependymomas who have not previously received radiation therapy and/or chemotherapy should be considered for treatment with these modalities.[6][Level of evidence: 3iiiB] In addition, patients may be candidates for focal retreatment with various radiation modalities, including stereotactic radiosurgery.[7][Level of evidence: 3iiiA]; [8,9][Level of evidence: 3iiiDi] Active agents include cyclophosphamide, cisplatin, carboplatin, lomustine, and etoposide. Regardless of treatment strategy, the prognosis for patients with recurrence is poor.[1] Entry into studies of novel therapeutic approaches should be considered.

Treatment Options Under Clinical Evaluation

Early phase therapeutic trials may be available for selected patients. These trials may be available via Children's Oncology Group phase I institutions, the Pediatric Brain Tumor Consortium, or other entities.

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood ependymoma 1. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site 2.

References

  1. Zacharoulis S, Ashley S, Moreno L, et al.: Treatment and outcome of children with relapsed ependymoma: a multi-institutional retrospective analysis. Childs Nerv Syst 26 (7): 905-11, 2010.  [PUBMED Abstract]

  2. 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]

  3. Vanuytsel LJ, Bessell EM, Ashley SE, et al.: Intracranial ependymoma: long-term results of a policy of surgery and radiotherapy. Int J Radiat Oncol Biol Phys 23 (2): 313-9, 1992.  [PUBMED Abstract]

  4. Goldwein JW, Corn BW, Finlay JL, et al.: Is craniospinal irradiation required to cure children with malignant (anaplastic) intracranial ependymomas? Cancer 67 (11): 2766-71, 1991.  [PUBMED Abstract]

  5. Merchant TE, Haida T, Wang MH, et al.: Anaplastic ependymoma: treatment of pediatric patients with or without craniospinal radiation therapy. J Neurosurg 86 (6): 943-9, 1997.  [PUBMED Abstract]

  6. Messahel B, Ashley S, Saran F, et al.: Relapsed intracranial ependymoma in children in the UK: patterns of relapse, survival and therapeutic outcome. Eur J Cancer 45 (10): 1815-23, 2009.  [PUBMED Abstract]

  7. Kano H, Yang HC, Kondziolka D, et al.: Stereotactic radiosurgery for pediatric recurrent intracranial ependymomas. J Neurosurg Pediatr 6 (5): 417-23, 2010.  [PUBMED Abstract]

  8. Merchant TE, Boop FA, Kun LE, et al.: A retrospective study of surgery and reirradiation for recurrent ependymoma. Int J Radiat Oncol Biol Phys 71 (1): 87-97, 2008.  [PUBMED Abstract]

  9. Kano H, Niranjan A, Kondziolka D, et al.: Outcome predictors for intracranial ependymoma radiosurgery. Neurosurgery 64 (2): 279-87; discussion 287-8, 2009.  [PUBMED Abstract]





Glossary Terms

Level of evidence 3iiiA
Nonconsecutive case series with total mortality as an endpoint. See Levels of Evidence for Adult and Pediatric Cancer Treatment Studies (PDQ®) for more information.
Level of evidence 3iiiB
Nonconsecutive case series with cause-specific mortality as an endpoint. See Levels of Evidence for Adult and Pediatric Cancer Treatment Studies (PDQ®) for more information.
Level of evidence 3iiiDi
Nonconsecutive case series with event-free survival as an endpoint. See Levels of Evidence for Adult and Pediatric Cancer Treatment Studies (PDQ®) for more information.
stereotactic radiosurgery (STAYR-ee-oh-TAK-tik RAY-dee-oh-SER-juh-ree)
Stereotactic radiosurgery (SRS) implies the delivery of a single, high dose of radiation using stereotactic techniques. Classically, for cranial SRS, a rigid neurosurgical frame is attached to the patient’s skull, and a stereotactic localizing device is attached to the frame to allow for improved targeting accuracy. Commercial SRS systems are available in which immobilization is achieved without a rigid neurosurgical frame. While there are innumerable commercial SRS and stereotactic radiation therapy systems, all of these systems utilize the same underlying principles: (1) patient immobilization, (2) targeting accuracy, (3) delivery of high doses of radiation, and (4) heterogeneous dose distribution with a steep dose gradient. Commercial SRS systems include Gamma Knife, which utilizes 201 radioactive cobalt sources positioned in a semispherical array, and Cyberknife, which utilizes robotics technology allowing the linear accelerator (LINAC) to track the patient position in real time during treatment. A standard LINAC can be equipped with cones attached to its head, allowing for circular collimation of the beam. The dose distribution from Gamma Knife, Cyberknife, or a standard LINAC equipped with cones is spherical, and thus treating nonspherically shaped targets requires the superposition of multiple spherical dose distributions. A LINAC equipped with multi-leaf collimators can also be used for SRS, which allows more conformal beam shaping. Also called radiation surgery, radiosurgery, and stereotaxic radiosurgery.

Table of Links

1http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=43456&tt=1&a
mp;format=2&cn=1
2http://www.cancer.gov/clinicaltrials