Treatment of Recurrent Childhood Ependymoma
Recurrence is not uncommon for all grades of ependymoma and may develop many years after initial treatment. Late recurrence beyond 10 to 15 years has been reported.[2,3] Disease generally recurs at the primary tumor site,[4,5] although concomitant neuraxis dissemination may also be seen. Systemic relapse is extremely rare. At time of relapse, a complete evaluation for extent of recurrence is indicated for all patients.Surgery
The need for further surgical intervention is individualized based on the following:
- Extent of the tumor.
- Length of time between initial treatment and the reappearance of the recurrent lesion.
- Clinical picture.
In some cases, surgically accessible lesions may be treated alternatively by radiation therapy.Radiation Therapy and/or Chemotherapy
Patients with recurrent ependymomas who have not previously received radiation therapy and/or chemotherapy should be considered for treatment with the following modalities:[Level of evidence: 3iiiB]
- Focal retreatment with various radiation modalities, including stereotactic radiosurgery.[7,8][Level of evidence: 3iiiA]; [9,10][Level of evidence: 3iiiDi]
- Active anticancer agents, including cyclophosphamide, cisplatin, carboplatin, lomustine, and etoposide.
Regardless of treatment strategy, the prognosis for patients with recurrence is poor. Entry into studies of novel therapeutic approaches should be considered.Treatment Options Under Clinical Evaluation for Recurrent Childhood Ependymoma
Early-phase therapeutic trials may be available for selected patients. These trials may be available via Children's Oncology Group, the Pediatric Brain Tumor Consortium, or other entities. Information about ongoing clinical trials is available from the NCI Web site.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. 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.References
- 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]
- 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]
- 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]
- 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]
- 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]
- 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]
- 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]
- Bouffet E, Hawkins CE, Ballourah W, et al.: Survival benefit for pediatric patients with recurrent ependymoma treated with reirradiation. Int J Radiat Oncol Biol Phys 83 (5): 1541-8, 2012. [PUBMED Abstract]
- 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]
- 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]