Alterations of protein 4.1 family members in ependymomas: a study of 84 cases

Rajaram, Veena; Gutmann, David H.; Prasad, Srinivas; Mansur, David B.; Perry, Arie

doi:10.1038/modpathol.3800390

Cited by 44 publications

(32 citation statements)

References 27 publications

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“…In addition, NF2 mutations are seen in patients with Neurofibromatosis type 2, a cancer predisposition syndrome regularly associated with the occurrence of spinal ependymomas. Disparate genetic alterations in other Protein 4.1 members have also been reported between spinal and intracranial ependymomas, such as 4.1B deletion and 4.1R loss of expression (78,82,84,85).…”

Section: Ependymoma Heterogeneity and Tumor Locationmentioning

confidence: 99%

“…Deletion of chromosome 6q23 has been associated with a worse event-free survival in a mixed age ependymoma cohort, warranting further analysis of this locus for prognostic gene markers (78). 6q24-26 also seems a region of frequent genomic loss in ependymomas (63,156).…”

Section: Immunohistochemical and Genomic Markersmentioning

confidence: 99%

“…Mixed age, adult and pediatric (<16 y). 32,35,36,58,59,76,78,86,. However, only a few of these candidates have been analyzed in sufficient numbers of childhood ependymoma to allow consideration as prognostic markers in this age group.…”

Section: Immunohistochemical and Genomic Markersmentioning

confidence: 99%

“…These include the overexpression of genes LDHB and STAM (77), the downregulation and deletion of members of the Protein 4.1 superfamily (78), and the reduced methylation of CDKN2A, CDKN2B, and p14ARF in posterior fossa ependymomas (79).…”

Section: Molecular Distinctions Between Pediatric and Adult Ependymomasmentioning

confidence: 99%

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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: Ependymoma Heterogeneity and Tumor Locationmentioning

confidence: 99%

Section: Immunohistochemical and Genomic Markersmentioning

confidence: 99%

Section: Immunohistochemical and Genomic Markersmentioning

confidence: 99%

Section: Molecular Distinctions Between Pediatric and Adult Ependymomasmentioning

confidence: 99%

See 2 more Smart Citations

Pediatric Ependymoma: Biological Perspectives

Kilday

Rahman

Dyer

et al. 2009

Molecular Cancer Research

168

204

View full text Add to dashboard Cite

show abstract

“…Among ependymomas, NF2 mutations are primarily encountered in spinal lesions. In contrast, it has been assumed that NF2 alterations do not significantly vary with tumor grade [1,2,9,13,18,20,21,25].…”

Section: Introductionmentioning

confidence: 99%