4.7 Review

The biology of ependymomas and emerging novel therapies

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A phase II study of dose-dense temozolomide and lapatinib for recurrent low-grade and anaplastic supratentorial, infratentorial, and spinal cord ependymoma

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Summary: A prospective phase II clinical trial of dose-dense temozolomide (TMZ) and lapatinib showed clinical activity with objective responses and prolonged disease control associated with disease-related symptom improvements in adult patients with recurrent ependymoma, making it a potential salvage regimen option.

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Local and systemic therapy of recurrent ependymoma in children and adolescents: short- and long-term results of the E-HIT-REZ 2005 study

Jonas E. Adolph et al.

Summary: In recurrent ependymomas in children and adolescents, the extent of resection is the most important predictor of survival. Focal re-irradiation is a useful approach for patients who cannot achieve complete resection, but does not provide additional benefit for those with complete or near-total resection. In some cases, disease stabilization for longer periods was achieved with TMZ.

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Ultra high-risk PFA ependymoma is characterized by loss of chromosome 6q

Lorena Baroni et al.

Summary: Through genome-wide methylation arrays analysis, a high-risk subgroup with 6q loss has been identified in PF-EPN-A, which can be reliably ascertained in routine clinical use. 6q loss remains the most significant independent predictor of survival in PF-EPN-A after treatment, but not in PF-EPN-B. Distant relapses are more common in 1q gain regardless of 6q loss.

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Characterization of molecular signatures of supratentorial ependymomas

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Molecular characterization of histopathological ependymoma variants

Julia E. Neumann et al.

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TET2 promotor methylation and TET2 protein expression in pediatric posterior fossa ependymoma

Daniela Pierscianek et al.

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Metabolic Regulation of the Epigenome Drives Lethal Infantile Ependymoma

Kulandaimanuvel Antony Michealraj et al.

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C11orf95-RELA reprograms 3D epigenome in supratentorial ependymoma

Jacqueline Jufen Zhu et al.

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Genetic predisposition to longer telomere length and risk of childhood, adolescent and adult-onset ependymoma

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Childhood cerebellar tumours mirror conserved fetal transcriptional programs

Maria C. Vladoiu et al.

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PFA ependymoma-associated protein EZHIP inhibits PRC2 activity through a H3 K27M-like mechanism

Siddhant U. Jain et al.

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A role for ABCB1 in prognosis, invasion and drug resistance in ependymoma

Durgagauri H. Sabnis et al.

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MYCN amplification drives an aggressive form of spinal ependymoma

David R. Ghasemi et al.

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Neurocognitive, academic and functional outcomes in survivors of infant ependymoma (UKCCSG CNS 9204)

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EANO guidelines for the diagnosis and treatment of ependymal tumors

Roberta Ruda et al.

NEURO-ONCOLOGY (2018)

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Molecular heterogeneity and CXorf67 alterations in posterior fossa group A (PFA) ependymomas

Kristian W. Pajtler et al.

ACTA NEUROPATHOLOGICA (2018)

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Heterogeneity within the PF-EPN-B ependymoma subgroup

Florence M. G. Cavalli et al.

ACTA NEUROPATHOLOGICA (2018)

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Patterns of relapse for children with localized intracranial ependymoma

Brian De et al.

JOURNAL OF NEURO-ONCOLOGY (2018)

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Prognostic relevance of miR-124-3p and its target TP53INP1 in pediatric ependymoma

Yulia Margolin-Miller et al.

GENES CHROMOSOMES & CANCER (2017)

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p16 Loss and E2F/cell cycle deregulation in infant posterior fossa ependymoma

Seth C. Lummus et al.

PEDIATRIC BLOOD & CANCER (2017)

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Spinal Myxopapillary Ependymomas Demonstrate a Warburg Phenotype

Stephen C. Mack et al.

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An in vivo screen identifies ependymoma oncogenes and tumor-suppressor genes

Kumarasamypet M. Mohankumar et al.

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Interleukin-6/STAT3 Pathway Signaling Drives an Inflammatory Phenotype in Group A Ependymoma

Andrea M. Griesinger et al.

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Telomerase inhibition abolishes the tumorigenicity of pediatric ependymoma tumor-initiating cells

Mark Barszczyk et al.

ACTA NEUROPATHOLOGICA (2014)

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Study of chromosome 9q gain, Notch pathway regulators and Tenascin-C in ependymomas

Rakesh Kumar Gupta et al.

JOURNAL OF NEURO-ONCOLOGY (2014)

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Epigenomic alterations define lethal CIMP-positive ependymomas of infancy

S. C. Mack et al.

NATURE (2014)

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C11orf95-RELA fusions drive oncogenic NF-κB signalling in ependymoma

Matthew Parker et al.

NATURE (2014)

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Axl/Gas6/NFκB signalling in schwannoma pathological proliferation, adhesion and survival

S. Ammoun et al.

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A prognostic gene expression signature in infratentorial ependymoma

Khalida Wani et al.

ACTA NEUROPATHOLOGICA (2012)

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Nestin Expression Identifies Ependymoma Patients with Poor Outcome

Till Milde et al.

BRAIN PATHOLOGY (2012)

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UBE4B promotes Hdm2-mediated degradation of the tumor suppressor p53

Hong Wu et al.

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Identification of MicroRNAs as Potential Prognostic Markers in Ependymoma

Fabricio F. Costa et al.

PLOS ONE (2011)

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Telomerase Inhibition as a Novel Therapy for Pediatric Ependymoma

Vincent C. H. Wong et al.

BRAIN PATHOLOGY (2010)

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EDG3 and SHC3 on chromosome 9q22 are co-amplified in human ependymomas

Lorenzo Magrassi et al.

CANCER LETTERS (2010)

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Molecular Staging of Intracranial Ependymoma in Children and Adults

Andrey Korshunov et al.

JOURNAL OF CLINICAL ONCOLOGY (2010)

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Tumor vessel biology in pediatric intracranial ependymoma Clinical article

Michiel Wagemakers et al.

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Cross-species genomics matches driver mutations and cell compartments to model ependymoma

Robert A. Johnson et al.

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Candidate Genes on Chromosome 9q33-34 Involved in the Progression of Childhood Ependymomas

Stephanie Puget et al.

JOURNAL OF CLINICAL ONCOLOGY (2009)

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Immune Gene and Cell Enrichment Is Associated with a Good Prognosis in Ependymoma

Andrew M. Donson et al.

JOURNAL OF IMMUNOLOGY (2009)

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Incidence patterns for ependymoma: a Surveillance, Epidemiology, and End Results study Clinical article

Courtney S. McGuire et al.

JOURNAL OF NEUROSURGERY (2009)

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Pediatric Ependymoma: Biological Perspectives

John-Paul Kilday et al.

MOLECULAR CANCER RESEARCH (2009)

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Telomere maintenance and dysfunction predict recurrence in paediatric ependymoma

U. Tabori et al.

BRITISH JOURNAL OF CANCER (2008)

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Merlin is apotent inhibitor of glioma growth

Ying-Ka Ingar Lau et al.

CANCER RESEARCH (2008)

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Prognostic value of tumor microinvasion and metalloproteinases expression in intracranial pediatric ependymomas

Matija Snuderl et al.

JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2008)

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Expression of cyclo-oxygenase-2 in ependymal tumors

Andres A. Roma et al.

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Radial glia cells are candidate stem cells of ependymoma

MD Taylor et al.

CANCER CELL (2005)

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Analysis of HIC-1 methylation and transcription in human ependymomas

A Waha et al.

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Article Clinical Neurology

CDKN2A, CDKN2B and p14(ARF) are frequently and differentially methylated in ependymal tumours

E Rousseau et al.

NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2003)

Article Pathology

Amplification and overexpression of mdm2 gene in ependymomas

SO Suzuki et al.

MODERN PATHOLOGY (2000)