title: Intracranial ependymoma: factors affecting outcome.
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.
authors.affiliation: Division of Pediatric Oncology, Istituto Nazionale dei Tumori, Milan, Italy.
authors.forename: Maura
authors.initials: M
authors.lastname: Massimino
authors.forename: Francesca R
authors.initials: FR
authors.lastname: Buttarelli
authors.forename: Manila
authors.initials: M
authors.lastname: Antonelli
authors.forename: Lorenza
authors.initials: L
authors.lastname: Gandola
authors.forename: Piergiorgio
authors.initials: P
authors.lastname: Modena
authors.forename: Felice
authors.initials: F
authors.lastname: Giangaspero
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000032:analysis; D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D002648:Child; D004806:Ependymoma / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D006801:Humans; D011379:Prognosis
chemical_list: D015415:Biomarkers