Intracranial ependymoma: factors affecting outcome
- 1 March 2009
- journal article
- review article
- Published by Future Medicine Ltd in Future Oncology
- Vol. 5 (2), 207-216
- https://doi.org/10.2217/14796694.5.2.207
Abstract
Ependymomas account for 2–9% of all neuroepithelial tumors, amounting to 6–12% of all intracranial tumors in children and up to 30% of those in children younger than 3 years. Recent findings provide evidence that intracranial and spinal ependymomas share similar molecular profiles with the radial glia of their corresponding locations. The management of intracranial ependymoma is still not optimal. The 5-year progression-free survival for children with ependymoma ranges between 30 and 50% with a worse prognosis for patients with residual disease after surgery. The prognostic relevance of most factors are still being debated. Recent studies, in which the current WHO classification criteria were applied, reported the relationship between histological grade and outcome. Biomolecular studies have identified that gain of 1q25 and EGFR overexpression correlate to poor prognosis, whereas low expression of nucleolin correlated with a favorable outcome. Ependymomas have been considered a ‘surgical disease’, where completeness of excision can be reached in approximately half of the cases. At present the standard treatment is radiation therapy for all patients after gross-total or near-total resection. For high-risk patients, with residual tumor, an interesting, although experimental, approach could be chemotherapy followed by secondary surgery and postoperative conformal irradiation.Keywords
This publication has 61 references indexed in Scilit:
- Multifactorial analysis of predictors of outcome in pediatric intracranial ependymomaNeuro-Oncology, 2008
- Predicting Change in Academic Abilities After Conformal Radiation Therapy for Localized EpendymomaJournal of Clinical Oncology, 2008
- Ki67 index in intracranial ependymoma: a promising histopathological candidate biomarkerHistopathology, 2008
- Primary postoperative chemotherapy without radiotherapy for intracranial ependymoma in children: the UKCCSG/SIOP prospective studyThe Lancet Oncology, 2007
- A multicenter study of the prognosis and treatment of adult brain ependymal tumorsCancer, 2004
- The histologic grade is a main prognostic factor for patients with intracranial ependymomas treated in the microneurosurgical eraCancer, 2004
- Postoperative Chemotherapy in Children Less Than 4 Years of Age with Malignant Brain TumorsJournal of Pediatric Hematology/Oncology, 1998
- Ependymoma: Results, Prognostic Factors and Treatment RecommendationsInternational Journal of Radiation Oncology*Biology*Physics, 1998
- Adjuvant chemotherapy of childhood posterior fossa ependymoma: Cranio-spinal irradiation with or without adjuvant CCNU, vincristine, and prednisone: A Childrens Cancer Group studyMedical and Pediatric Oncology, 1996
- Postoperative Chemotherapy and Delayed Radiation in Infants and Very Young Children with Choroid Plexus CarcinomasPediatric Neurosurgery, 1995