Evolution of neurological impairment in pediatric infratentorial ependymoma patients
Background Infratentorial ependymoma is a common central nervous system tumor of childhood and in patients >1 year of age is treated with maximally feasible surgical resection and radiotherapy. Because of this tumor typically arises within the 4th ventricle and can invade the brainstem, patients are at risk for significant neurological impairment. Purpose To characterize the incidence, evolution, and persistence of neurologic impairment in children with infratentorial ependymoma following maximal safe surgery and conformal or intensity-modulated radiation therapy (CRT/IMRT). Patients and Methods After surgical resection, 96 children with non-metastatic infratentorial ependymoma were enrolled on a phase II study of image-guided radiation therapy and were prospectively followed with interval comprehensive neurological examinations. Late adverse neurological severity was graded according to the National Cancer Institute Common Terminology Criteria for Adverse Events (CTCAE), version 3.0. Results The most common deficits detected at baseline examination were limb dysmetria, cranial nerve VI/VII palsy, limb paresis, dysphagia, and truncal ataxia/hypotonia. When present, gait dysfunction and dysphagia were often severe. Oculomotor dysfunction, facial paresis, dysphagia, and gait impairment improved over time. With the exception of hearing loss, in the survivor cohort, very few severe late effects (CTCAE Grade 3/4/5) were present at 60 months survival. Conclusion In general, neurological deficits were maximal in the post-operative period and either remained stable or improved during radiation and the post-treatment evaluation period. With the exception of hearing, the majority of chronic residual neurological deficits in this at-risk population are mild and only minimally intrude upon daily life.
KeywordsEpendymoma Children Conformal radiation Neurological impairment
Financial Support: This work was supported by the Cancer Center Support (CORE) Grant P30 CA 21765 from the National Institutes of Health and by the American Lebanese Syrian Associated Charities (ALSAC).
- 1.CBTRUS 1/22/08. Selected primary (malignant and non-malignant) brain and central nervous system tumor age-specific incidence rates by age at diagnosis, CBTRUS 1998–2002. http://wwwcbtrusorg/2005-2006/tables/2006table12pdf Accessed 22 Jan 2008
- 2.Gurney JGSM, Bunin GR (1999) CNS and miscellaneous intracranial and intraspinal neoplasms. National Cancer Institute, SEER Program, Bethesda, MD. NIH Pub. No. 99-4649 ReportGoogle Scholar
- 3.Bouffet E, Perilongo G, Canete A et al (1998) Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation. Med Pediatr Oncol 30(6):319–329. doi: 10.1002/(SICI)1096-911X(199806)30:6<319::AID-MPO1>3.0.CO;2-H (discussion 329–331)PubMedCrossRefGoogle Scholar
- 4.Phase A II trial of conformal rt for pediatric patients with localized ependymoma, chemotherapy prior to 2nd surgery for incompletely resected ependymoma and observations for completely resected, differentiated, supratentorial ependymoma. https://members.childrensoncologygroup.org/prot/ProtInfo.asp?ProtocolNum=ACNS0121&Disease=CNS. Children’s Oncology Group Accessed
- 5.Merchant TE, Mulhern RK, Krasin MJ et al (2004) 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):3156–3162. doi: 10.1200/JCO.2004.11.142 PubMedCrossRefGoogle Scholar
- 6.Littell RCMG, Stroup WW, Wolfinger RD (1996) SAS system for mixed models. SAS Institute Inc, Cary, NCGoogle Scholar
- 14.Bamford FN, Jones PM, Pearson D et al (1976) Residual disabilities in children treated for intracranial space-occupying lesions. Cancer 37(2 (Suppl)):1149–1151. doi: 10.1002/1097-0142(197602)37:2+<1149::AID-CNCR2820370825>3.0.CO;2-C PubMedCrossRefGoogle Scholar