A Multi-Institutional Retrospective Study of Intracranial Ependymoma in Children

Horn, Biljana; Heideman, Richard L.; Geyer, Russel; Pollack, Ian F.; Packer, Roger J.; Goldwein, Joel W.; Tomita, Tadanori; Schomberg, Paula J.; Ater, Joann L.; Luchtman‐Jones, Lori; Rivlin, Kenneth; Lamborn, Kathleen R.; Prados, Michael D.; Bollen, Andrew W.; Berger, Mitchel S.; Dahl, Gary V.; McNeil, Elizabeth; Patterson, Kathleen; Shaw, Dennis; M, Kubalik; Russo, Carolyn

doi:10.1097/00043426-199905000-00008

Cited by 189 publications

(131 citation statements)

References 30 publications

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“…However, although complete resection is the most consistently reported favorable clinical prognostic factor (1,(16)(17)(18), this is not a universal finding and some studies fail to show this relationship for pediatric posterior fossa tumors (19,20). Furthermore, despite complete excision, local tumor recurrence can develop in up to 50% of cases, even following adjuvant radiotherapy (14,15,21).…”

Section: Introductionmentioning

confidence: 99%

Pediatric Ependymoma: Biological Perspectives

Kilday

Rahman

Dyer

et al. 2009

Molecular Cancer Research

168

204

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Pediatric ependymomas are enigmatic tumors that continue to present a clinical management challenge despite advances in neurosurgery, neuroimaging techniques, and radiation therapy. Difficulty in predicting tumor behavior from clinical and histological factors has shifted the focus to the molecular and cellular biology of ependymoma in order to identify new correlates of disease outcome and novel therapeutic targets. This article reviews our current understanding of pediatric ependymoma biology and includes a meta-analysis of all comparative genomic hybridization (CGH) studies done on primary ependymomas to date, examining more than 300 tumors. From this meta-analysis and a review of the literature, we show that ependymomas in children exhibit a different genomic profile to those in adults and reinforce the evidence that ependymomas from different locations within the central nervous system (CNS) are distinguishable at a genomic level. Potential biological markers of prognosis in pediatric ependymoma are assessed and the ependymoma cancer stem cell hypothesis is highlighted with respect to tumor resistance and recurrence. We also discuss the shifting paradigm for treatment modalities in ependymoma that target molecular alterations in tumor-initiating cell populations. (Mol Cancer Res 2009;7(6):765-86)

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Section: Introductionmentioning

confidence: 99%

Pediatric Ependymoma: Biological Perspectives

Kilday

Rahman

Dyer

et al. 2009

Molecular Cancer Research

168

204

View full text Add to dashboard Cite

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“…In children with intracranial ependymoma, event-free survival after 5 years is less than 50% (Bouffet et al, 1998;Robertson et al, 1998;Horn et al, 1999;Packer, 2000;Grill et al, 2001). Various factors have been reported to influence prognosis, though clinical research in this area has produced many conflicting results.…”

mentioning

confidence: 99%

“…Various factors have been reported to influence prognosis, though clinical research in this area has produced many conflicting results. However, gross surgical resection and the use of radiotherapy have been consistently associated with enhanced event-free and overall survival (Nazar et al, 1990;Sutton et al, 1990;Healey et al, 1991;Vanuytsel et al, 1992;Ferrante et al, 1994;Pollack et al, 1995;Bouffet et al, 1998;Robertson et al, 1998;Horn et al, 1999;Grill et al, 2001). Histological features of anaplasia, such as mitoses, microvascular proliferation and necrosis, serve as indicators of biological behaviour in other gliomas, including diffuse astrocytic tumours and oligodendrogliomas (Cohadon et al, 1985;Burger and Green, 1987;Ellison, 1998).…”

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confidence: 99%

Genetic abnormalities detected in ependymomas by comparative genomic hybridisation

et al. 2002

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Using comparative genomic hybridisation, we have analysed genetic imbalance in a series of 86 ependymomas from children and adults. Tumours were derived from intracranial and spinal sites, and classified histologically as classic, anaplastic or myxopapillary. Ependymomas showing a balanced profile were significantly (P50.0005) more frequent in children than adults. Profiles suggesting intermediate ploidy were common (44% of all tumours), and found more often (P50.0005) in tumours from adults and the spinal region. Loss of 22q was the most common specific abnormality, occurring in 50% of spinal (medullary) ependymomas and 26% of tumours overall. Genetic profiles combining loss of 22q with other specific abnormalities -gain of 1q, loss of 6q, loss of 10q/10, loss of 13, loss of 14q/14 -varied according to site and histology. In particular, we showed that classic ependymomas from within the cranium and spine have distinct genetic profiles. Classic and anaplastic ependymomas with gain of 1q tended to occur in the posterior fossa of children and to behave aggressively. Our extensive data on ependymomas demonstrate significant associations between genetic aberrations and clinicopathological variables, and represent a starting point for further biological and clinical studies.

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“…Histologic grading of these tumors does not predict clinical behavior as accurately as other gliomas, [1][2][3][4][5][6] although there are some studies that show a significant association between tumor grade and prognosis. [7][8][9][10] Thus far, the only consistent prognostic variables include extent of surgical resection, with tumor location and patient's age also having some significance.…”

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confidence: 99%

9p21 and 13q14 dosages in ependymomas. A clinicopathologic study of 101 cases

et al. 2004

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Ependymomas are glial neoplasms whose clinical behavior is difficult to predict based on histology alone. Recently, a comparative genomic hybridization study identified frequent chromosome 9p and 13q losses in anaplastic ependymomas, suggesting that p16 and RB alterations may be involved in tumor progression. In order to test this hypothesis further, 101 myxopapillary, conventional, and anaplastic ependymomas (51 spinal and 50 intracranial tumors) were tested for RB and p16 deletions using fluorescence in situ hybridization. Clinical follow-up, ranging from 2 to 198 months (median 46 months), was obtained in 90 cases (91%). RB and p16 deletions were seen in 22 of 92 (24%) and 22 of 89 (25%) informative cases, respectively. Polysomies were more frequent in the grade I and II spinal tumors, consistent with prior reports of increased aneuploidy in such cases. No significant genetic associations were seen with tumor grade, recurrence, or death, suggesting that 9p and 13q deletions do not play a prominent role in the malignant progression of ependymomas, as has been implicated in other glioma subtypes.

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A Multi-Institutional Retrospective Study of Intracranial Ependymoma in Children

Abstract: Adverse outcome in childhood intracranial ependymoma is related to age (3 years or younger), histology (grade 3), and degree of surgical resection (less than GTR). New approaches, particularly for local tumor control in younger patients, are needed to improve survival.

Cited by 189 publications

References 30 publications

Pediatric Ependymoma: Biological Perspectives

Pediatric Ependymoma: Biological Perspectives

Genetic abnormalities detected in ependymomas by comparative genomic hybridisation

9p21 and 13q14 dosages in ependymomas. A clinicopathologic study of 101 cases

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