Epenclymomas 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. Epenclymomas 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.

Intracranial ependymoma: factors affecting outcome / Maura, Massimino; Buttarelli, Francesca Romana; Antonelli, Manila; Lorenza, Gandola; Piergiorgio, Modena; Giangaspero, Felice. - In: FUTURE ONCOLOGY. - ISSN 1479-6694. - 5:2(2009), pp. 207-216. [10.2217/14796694.5.2.207]

Intracranial ependymoma: factors affecting outcome

BUTTARELLI, Francesca Romana;ANTONELLI, MANILA;GIANGASPERO, FELICE
2009

Abstract

Epenclymomas 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. Epenclymomas 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.
2009
chemotherapy; ependymoma; gliomas; grading; prognosis; radiotherapy; surgery
01 Pubblicazione su rivista::01a Articolo in rivista
Intracranial ependymoma: factors affecting outcome / Maura, Massimino; Buttarelli, Francesca Romana; Antonelli, Manila; Lorenza, Gandola; Piergiorgio, Modena; Giangaspero, Felice. - In: FUTURE ONCOLOGY. - ISSN 1479-6694. - 5:2(2009), pp. 207-216. [10.2217/14796694.5.2.207]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/365331
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