Evolution of neurological impairment in pediatric infratentorial ependymoma patients
E Brannon Morris1, Chenghong Li, Raja B Khan
1Division of Neurology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA. Brannon.morris@stjude.org
Insights
Neurological deficits in children with infratentorial ependymoma typically peak post-surgery and improve with treatment. Most long-term effects are mild, except for hearing loss.
Area of Science:
- Pediatric neuro-oncology
- Radiation oncology
- Neurology
Background:
- Infratentorial ependymoma is a common pediatric brain tumor.
- Treatment involves maximal surgical resection and radiotherapy.
- Tumor location risks significant neurological impairment due to brainstem invasion.
Purpose of the Study:
- To assess the incidence, progression, and persistence of neurological impairment in children with infratentorial ependymoma.
- To evaluate outcomes after maximal safe surgery and radiation therapy (CRT/IMRT).
Main Methods:
- 96 children with non-metastatic infratentorial ependymoma were studied post-surgery.
- Prospective follow-up included comprehensive neurological examinations.
- Late adverse neurological events were graded using CTCAE v3.0.
Main Results:
- Common initial deficits included limb dysmetria, cranial nerve palsies, paresis, dysphagia, and ataxia.
- Gait dysfunction and dysphagia were often severe but improved over time.
- Few severe late effects (Grade 3/4/5) were observed at 60 months, except for hearing loss.
Conclusions:
- Neurological deficits were generally maximal post-operatively and improved or stabilized during and after treatment.
- Most residual neurological deficits in survivors are mild and minimally impact daily life, with hearing loss being a notable exception.
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.
Related Concept Videos
01:22Cerebral Edema ll: Pathophysiology
01:29Increased Intracranial Pressure ll: Pathophysiology
01:19Encephalitis l: Introduction
01:26Brain Abscess l: Introduction