• Journal of Korean Neurosurgical Society
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• v.30 no.sup2
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• pp.228-234
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• 2001

Objective : The goal of this study was to identify variables that were predictive of recurrence in primary intracranial ependymomas. Methods : We analyzed variables affecting recurrence in 30 patients with primary intracranial ependymomas. Age, location, CSF cytology, seeding on neuroimaging study, tumor grade, extent of surgery, use of chemotherapy, chemotherapy regimen, use of radiotherapy, and radiotherapy field were entered to test their impacts on recurrence. Results : Follow-up ranged from 2 to 110 months. Tumors were recurred at the primary tumor site only in 13 patients (43.3%). The overall average recurrence free period was 55 months, with overall recurrence free rates at 3 and 6 years of 61.0% and 20.9%, respectively. Extent of surgery was the strongest variable affecting recurrence. The median recurrence free period and 3-year recurrence free rate were 72 months and 78.4% for patients having complete excision and 33 months and 0% for those having incomplete excision(p=0.05). Other prognostic variables like age, location, tumor grade, use of chemotherapy, and use of radiotherapy did not affect recurrence(p=0.2848, 0.7899, 0.1714, 0.2157, 0.7076, respectively). Conclusions : Intracranial ependymomas have a propensity to recur after treatment, and recurrence at the primary site is still the main obstacle to cure. Among various variables, only extent of resection had the strongest impact on recurrence. Additional studies may still be needed to precisely define the prognostic variables on recurrence in intracranial ependymomas.

• Journal of Korean Neurosurgical Society
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• v.59 no.2
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• pp.83-90
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• 2016

Ependymomas occur in both the brain and spine. The prognosis of these tumors sometimes differs for different locations. The genetic landscape of ependymoma is very heterogeneous despite the similarity of histopathologic findings. In this review, we describe the genetic differences between spinal ependymomas and their intracranial counterparts to better understand their prognosis. From the literature review, many studies have reported that spinal cord ependymoma might be associated with NF2 mutation, NEFL overexpression, Merlin loss, and 9q gain. In myxopapillary ependymoma, NEFL and HOXB13 overexpression were reported to be associated. Prior studies have identified HIC-1 methylation, 4.1B deletion, and 4.1R loss as common features in intracranial ependymoma. Supratentorial ependymoma is usually characterized by NOTCH-1 mutation and p75 expression. TNC mutation, no hypermethylation of RASSF1A, and GFAP/NeuN expression may be diagnostic clues of posterior fossa ependymoma. Although MEN1, TP53, and PTEN mutations are rarely reported in ependymoma, they may be related to a poor prognosis, such as recurrence or metastasis. Spinal ependymoma has been found to be quite different from intracranial ependymoma in genetic studies, and the favorable prognosis in spinal ependymoma may be the result of the genetic differences. A more detailed understanding of these various genetic aberrations may enable the identification of more specific prognostic markers as well as the development of customized targeted therapies.