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Multi-omic approach identifies hypoxic tumor-associated myeloid cells that drive immunobiology of high-risk pediatric ependymoma. (2023)
Journal Article
Griesinger, A. M., Riemondy, K., Eswaran, N., Donson, A. M., Willard, N., Prince, E. W., …Ritzmann, T. A. (2023). Multi-omic approach identifies hypoxic tumor-associated myeloid cells that drive immunobiology of high-risk pediatric ependymoma. iScience, 26(9), Article 107585. https://doi.org/10.1016/j.isci.2023.107585

Ependymoma (EPN) is a devastating childhood brain tumor. Single-cell analyses have illustrated the cellular heterogeneity of EPN tumors, identifying multiple neoplastic cell states including a mesenchymal-differentiated subpopulation which characteri... Read More about Multi-omic approach identifies hypoxic tumor-associated myeloid cells that drive immunobiology of high-risk pediatric ependymoma..

Pediatric-type high-grade neuroepithelial tumors with CIC gene fusion share a common DNA methylation signature (2023)
Journal Article
Sievers, P., Sill, M., Schrimpf, D., Abdullaev, Z., Donson, A. M., Lake, J. A., …Jones, D. T. W. (in press). Pediatric-type high-grade neuroepithelial tumors with CIC gene fusion share a common DNA methylation signature. npj Precision Oncology, 7, Article 30. https://doi.org/10.1038/s41698-023-00372-1

Pediatric neoplasms in the central nervous system (CNS) show extensive clinical and molecular heterogeneity and are fundamentally different from those occurring in adults. Molecular genetic testing contributes to accurate diagnosis and enables an opt... Read More about Pediatric-type high-grade neuroepithelial tumors with CIC gene fusion share a common DNA methylation signature.

Optimising biomarkers for accurate ependymoma diagnosis, prognostication and stratification within International Clinical Trials: A BIOMECA study (2023)
Journal Article
Chapman, R. J., Ghasemi, D. R., Andreiuolo, F., Zschernack, V., Tauziede Espariat, A., Buttarelli, F. R., …Biomarkers of Ependymoma in Childhood and Adolescence (BIOMECA) Consortium. (2023). Optimising biomarkers for accurate ependymoma diagnosis, prognostication and stratification within International Clinical Trials: A BIOMECA study. Neuro-Oncology, 25(10), 1871–1882. https://doi.org/10.1093/neuonc/noad055

Background Accurate identification of brain tumour molecular subgroups is increasingly important. We aimed to establish the most accurate and reproducible ependymoma subgroup biomarker detection techniques, across 147 cases from International Society... Read More about Optimising biomarkers for accurate ependymoma diagnosis, prognostication and stratification within International Clinical Trials: A BIOMECA study.

Spatial transcriptomic analysis delineates epithelial and mesenchymal subpopulations and transition stages in childhood ependymoma (2022)
Journal Article
Fu, R., Norris, G. A., Willard, N., Griesinger, A. M., Riemondy, K. A., Amani, V., …Donson, A. M. (2023). Spatial transcriptomic analysis delineates epithelial and mesenchymal subpopulations and transition stages in childhood ependymoma. Neuro-Oncology, 25(4), 786-798. https://doi.org/10.1093/neuonc/noac219

Background The diverse cellular constituents of childhood brain tumor ependymoma, recently revealed by single cell RNA-sequencing, may underly therapeutic resistance. Here we use spatial transcriptomics to further advance our understanding of the tum... Read More about Spatial transcriptomic analysis delineates epithelial and mesenchymal subpopulations and transition stages in childhood ependymoma.

Impact of Tumour Epstein–Barr Virus Status on Clinical Outcome in Patients with Classical Hodgkin Lymphoma (cHL): A Review of the Literature and Analysis of a Clinical Trial Cohort of Children with cHL (2022)
Journal Article
Nohtani, M., Vrzalikova, K., Ibrahim, M., Powell, J. E., Fennell, É., Morgan, S., …Murray, P. G. (2022). Impact of Tumour Epstein–Barr Virus Status on Clinical Outcome in Patients with Classical Hodgkin Lymphoma (cHL): A Review of the Literature and Analysis of a Clinical Trial Cohort of Children with cHL. Cancers, 14(17), Article 4297. https://doi.org/10.3390/cancers14174297

In this study, we have re-evaluated how EBV status influences clinical outcome. To accomplish this, we performed a literature review of all studies that have reported the effect of EBV status on patient outcome and also explored the effect of EBV pos... Read More about Impact of Tumour Epstein–Barr Virus Status on Clinical Outcome in Patients with Classical Hodgkin Lymphoma (cHL): A Review of the Literature and Analysis of a Clinical Trial Cohort of Children with cHL.

Lipoprotein Deprivation Reveals a Cholesterol-Dependent Therapeutic Vulnerability in Diffuse Glioma Metabolism (2022)
Journal Article
Wood, J., Abdelrazig, S., Evseev, S., Ortori, C., Castellanos-Uribe, M., May, S. T., …Rahman, R. (2022). Lipoprotein Deprivation Reveals a Cholesterol-Dependent Therapeutic Vulnerability in Diffuse Glioma Metabolism. Cancers, 14(16), Article 3873. https://doi.org/10.3390/cancers14163873

Poor outcomes associated with diffuse high-grade gliomas occur in both adults and children, despite substantial progress made in the molecular characterisation of the disease. Targeting the metabolic requirements of cancer cells represents an alterna... Read More about Lipoprotein Deprivation Reveals a Cholesterol-Dependent Therapeutic Vulnerability in Diffuse Glioma Metabolism.

Toward Improved Diagnosis Accuracy and Treatment of Children, Adolescents, and Young Adults With Ependymoma: The International SIOP Ependymoma II Protocol (2022)
Journal Article
Leblond, P., Massimino, M., English, M., Ritzmann, T. A., Gandola, L., Calaminus, G., …Frappaz, D. (2022). Toward Improved Diagnosis Accuracy and Treatment of Children, Adolescents, and Young Adults With Ependymoma: The International SIOP Ependymoma II Protocol. Frontiers in Neurology, 13, Article 887544. https://doi.org/10.3389/fneur.2022.887544

Background: The clinical management of ependymoma in childhood and adolescence is complex and the clinicobiopathological correlates of outcome remain poorly understood. This international SIOP Ependymoma II (SIOP EPII) trial aims to improve the outco... Read More about Toward Improved Diagnosis Accuracy and Treatment of Children, Adolescents, and Young Adults With Ependymoma: The International SIOP Ependymoma II Protocol.

Phase II study of intravenous etoposide in patients with relapsed ependymoma (CNS 2001 04) (2022)
Journal Article
Apps, J. R., Maycock, S., Ellison, D. W., Jaspan, T., Ritzmann, T. A., Macarthur, D., …Picton, S. (2022). Phase II study of intravenous etoposide in patients with relapsed ependymoma (CNS 2001 04). Neuro-Oncology Advances, 4(1), Article vdac053. https://doi.org/10.1093/noajnl/vdac053

Background Relapsed ependymoma has a dismal prognosis, and the role of chemotherapy at relapse remains unclear. This study prospectively evaluated the efficacy of intensive intravenous (IV) etoposide in patients less than 21 years of age with relaps... Read More about Phase II study of intravenous etoposide in patients with relapsed ependymoma (CNS 2001 04).

Dynamic susceptibility-contrast magnetic resonance imaging with contrast agent leakage correction aids in predicting grade in pediatric brain tumours: a multicenter study (2022)
Journal Article
Withey, S. B., MacPherson, L., Oates, A., Powell, S., Novak, J., Abernethy, L., …Peet, A. C. (2022). Dynamic susceptibility-contrast magnetic resonance imaging with contrast agent leakage correction aids in predicting grade in pediatric brain tumours: a multicenter study. Pediatric Radiology, 52(6), 1134-1149. https://doi.org/10.1007/s00247-021-05266-7

Background Relative cerebral blood volume (rCBV) measured using dynamic susceptibility-contrast MRI can differentiate between low- and high-grade pediatric brain tumors. Multicenter studies are required for translation into clinical practice. Obj... Read More about Dynamic susceptibility-contrast magnetic resonance imaging with contrast agent leakage correction aids in predicting grade in pediatric brain tumours: a multicenter study.

SIOP Ependymoma I: Final results, long term follow-up and molecular analysis of the trial cohort: A BIOMECA Consortium Study (2022)
Journal Article
Ritzmann, T. A., Chapman, R. J., Kilday, J.-P., Thorp, N., Modena, P., Dineen, R. A., …Grundy, R. G. (2022). SIOP Ependymoma I: Final results, long term follow-up and molecular analysis of the trial cohort: A BIOMECA Consortium Study. Neuro-Oncology, 24(6), 936–948. https://doi.org/10.1093/neuonc/noac012

Background SIOP Ependymoma I was a non-randomised trial assessing event free and overall survival (EFS/OS) of non-metastatic intracranial ependymoma in children aged 3 to 21 years treated with a staged management strategy. A further aim was to asses... Read More about SIOP Ependymoma I: Final results, long term follow-up and molecular analysis of the trial cohort: A BIOMECA Consortium Study.

EPEN-04. SIOP EPENDYMOMA I: FINAL RESULTS, LONG TERM FOLLOW-UP AND MOLECULAR ANALYSIS OF THE TRIAL COHORT: A BIOMECA CONSORTIUM STUDY (2021)
Journal Article
Ritzmann, T. A., Chapman, R. J., Macarthur, D., Mallucci, C., Kilday, J.-P., Thorp, N., …Grundy, R. G. (2021). EPEN-04. SIOP EPENDYMOMA I: FINAL RESULTS, LONG TERM FOLLOW-UP AND MOLECULAR ANALYSIS OF THE TRIAL COHORT: A BIOMECA CONSORTIUM STUDY. Neuro-Oncology, 23(Supplement 1), i14. https://doi.org/10.1093/neuonc/noab090.054

Introduction Surgery and radiotherapy are established childhood ependymoma treatments. The efficacy of chemotherapy has been debated. We report final results of the SIOP Ependymoma I trial, with 12-year follow-up, in the context of a post-hoc analys... Read More about EPEN-04. SIOP EPENDYMOMA I: FINAL RESULTS, LONG TERM FOLLOW-UP AND MOLECULAR ANALYSIS OF THE TRIAL COHORT: A BIOMECA CONSORTIUM STUDY.

EPEN-07. SINGLE-CELL RNA SEQUENCING IDENTIFIES A UNIQUE MYELOID SUBPOPULATION ASSOCIATED WITH MESENCHYMAL TUMOR SUBPOPULATION IN POOR OUTCOME PEDIATRIC EPENDYMOMA (2021)
Journal Article
Griesinger, A., Riemondy, K., Donson, A., Willard, N., Prince, E., Harris, F., …Ritzmann, T. (2021). EPEN-07. SINGLE-CELL RNA SEQUENCING IDENTIFIES A UNIQUE MYELOID SUBPOPULATION ASSOCIATED WITH MESENCHYMAL TUMOR SUBPOPULATION IN POOR OUTCOME PEDIATRIC EPENDYMOMA. Neuro-Oncology, 23(Supplement 1), i14-i15. https://doi.org/10.1093/neuonc/noab090.057

We have previously shown immune gene phenotype variations between posterior fossa ependymoma subgroups. PFA1 tumors chronically secrete IL-6, which induces secretion of myeloid cell IL-8 and pushes the infiltrating myeloid cells to an immune suppress... Read More about EPEN-07. SINGLE-CELL RNA SEQUENCING IDENTIFIES A UNIQUE MYELOID SUBPOPULATION ASSOCIATED WITH MESENCHYMAL TUMOR SUBPOPULATION IN POOR OUTCOME PEDIATRIC EPENDYMOMA.

EPEN-08. THE TREM1 POSITIVE HYPOXIC MYELOID SUBPOPULATION IN POSTERIOR FOSSA EPENDYMOMA (2021)
Journal Article
Willard, N., Donson, A., Ritzmann, T., Grundy, R., Jackson, A., Hankinson, T., …Foreman, N. (2021). EPEN-08. THE TREM1 POSITIVE HYPOXIC MYELOID SUBPOPULATION IN POSTERIOR FOSSA EPENDYMOMA. Neuro-Oncology, 23(Supplement 1), i15. https://doi.org/10.1093/neuonc/noab090.058

We have previously shown the importance of immune factors in posterior fossa ependymoma (PF EPN). Recently, we found eight transcriptionally unique subpopulations of myeloid cells infiltrating PF EPN with one population particularly enriched in PFA1... Read More about EPEN-08. THE TREM1 POSITIVE HYPOXIC MYELOID SUBPOPULATION IN POSTERIOR FOSSA EPENDYMOMA.

EPEN-11. TUMOR DIFFERENTIATION IMPACTS THE BIOLOGY OF RECURRENCE IN CHILDHOOD POSTERIOR FOSSA EPENDYMOMA (2021)
Journal Article
Donson, A., Ritzmann, T., Willard, N., Griesinger, A., Amani, V., Harris, F., …Foreman, N. (2021). EPEN-11. TUMOR DIFFERENTIATION IMPACTS THE BIOLOGY OF RECURRENCE IN CHILDHOOD POSTERIOR FOSSA EPENDYMOMA. Neuro-Oncology, 23(Supplement 1), i15-i16. https://doi.org/10.1093/neuonc/noab090.061

Ependymoma (EPN) of childhood is curable in only 50% of cases, with recurrences in the remainder that are refractory to treatment. In recent years significant advances have been made in understanding the molecular and cellular biology of EPN. Recent... Read More about EPEN-11. TUMOR DIFFERENTIATION IMPACTS THE BIOLOGY OF RECURRENCE IN CHILDHOOD POSTERIOR FOSSA EPENDYMOMA.

Sequential Orbitrap Secondary Ion Mass Spectrometry and Liquid Extraction Surface Analysis-Tandem Mass Spectrometry-Based Metabolomics for Prediction of Brain Tumor Relapse from Sample-Limited Primary Tissue Archives (2021)
Journal Article
Meurs, J., Scurr, D. J., Lourdusamy, A., Storer, L. C., Grundy, R. G., Alexander, M. R., …Kim, D.-H. (2021). Sequential Orbitrap Secondary Ion Mass Spectrometry and Liquid Extraction Surface Analysis-Tandem Mass Spectrometry-Based Metabolomics for Prediction of Brain Tumor Relapse from Sample-Limited Primary Tissue Archives. Analytical Chemistry, 93(18), 6947-6954. https://doi.org/10.1021/acs.analchem.0c05087

We present here a novel surface mass spectrometry strategy to perform untargeted metabolite profiling of formalin-fixed paraffin-embedded pediatric ependymoma archives. Sequential Orbitrap secondary ion mass spectrometry (3D OrbiSIMS) and liquid extr... Read More about Sequential Orbitrap Secondary Ion Mass Spectrometry and Liquid Extraction Surface Analysis-Tandem Mass Spectrometry-Based Metabolomics for Prediction of Brain Tumor Relapse from Sample-Limited Primary Tissue Archives.

Assessment of Cannabidiol and ∆9-Tetrahydrocannabiol in Mouse Models of Medulloblastoma and Ependymoma (2021)
Journal Article
Andradas, C., Byrne, J., Kuchibhotla, M., Ancliffe, M., Jones, A. C., Carline, B., …Endersby, R. (2021). Assessment of Cannabidiol and ∆9-Tetrahydrocannabiol in Mouse Models of Medulloblastoma and Ependymoma. Cancers, 13, Article 330. https://doi.org/10.3390/cancers13020330

Children with medulloblastoma and ependymoma are treated with a multidisciplinary approach that incorporates surgery, radiotherapy, and chemotherapy; however, overall survival rates for patients with high-risk disease remain unsatisfactory. Data indi... Read More about Assessment of Cannabidiol and ∆9-Tetrahydrocannabiol in Mouse Models of Medulloblastoma and Ependymoma.

TBIO-14. Characterisation of the arginine pathway enzymes in paediatric brain tumours to determine susceptibility to therapeutic arginine depletion (2020)
Presentation / Conference Contribution
Bishop, E., Dimitrova, M., Storer, L., Grundy, R., & Dandapani, M. (2020). TBIO-14. Characterisation of the arginine pathway enzymes in paediatric brain tumours to determine susceptibility to therapeutic arginine depletion. Neuro-Oncology, 22(Issue Supplement_3), iii469. https://doi.org/10.1093/neuonc/noaa222.841

INTRODUCTION Extracellular arginine dependency (auxotrophy) is increasingly being recognised in several tumours. This is due to the inability of cancer cells to recycle or synthesise intracellular arginine through the urea cycle pathway compared to... Read More about TBIO-14. Characterisation of the arginine pathway enzymes in paediatric brain tumours to determine susceptibility to therapeutic arginine depletion.

Epen-22. Single-cell RNA sequencing identifies upregulation of IKZF1 in PFA2 myeloid subpopulation driving an anti-tumor phenotype (2020)
Presentation / Conference Contribution
Griesinger, A., Prince, E., Donson, A., Riemondy, K., Ritzman, T., Harris, F., …Foreman, N. (2020). Epen-22. Single-cell RNA sequencing identifies upregulation of IKZF1 in PFA2 myeloid subpopulation driving an anti-tumor phenotype. Neuro-Oncology, 22(Supplement 3), iii312. https://doi.org/10.1093/neuonc/noaa222.159

We have previously shown immune gene phenotype variations between posterior fossa ependymoma subgroups. PFA1 tumors chronically secrete IL-6, which pushes the infiltrating myeloid cells to an immune suppressive function. In contrast, PFA2 tumors have... Read More about Epen-22. Single-cell RNA sequencing identifies upregulation of IKZF1 in PFA2 myeloid subpopulation driving an anti-tumor phenotype.

Epen-23. A computational analysis of the tumour immune microenvironment in paediatric ependymoma (2020)
Presentation / Conference Contribution
Ritzmann, T., Lourdusamy, A., Jackson, A., Storer, L., Donson, A., Griesinger, A., …Grundy, R. (2020). Epen-23. A computational analysis of the tumour immune microenvironment in paediatric ependymoma. Neuro-Oncology, 22(Supplement 3), iii312. https://doi.org/10.1093/neuonc/noaa222.160

Ependymoma is the third commonest childhood brain tumour. Relapse is frequent, often fatal and current therapeutic strategies are inadequate. Previous ependymoma research describes an immunosuppressive environment with T-cell exhaustion, indicating a... Read More about Epen-23. A computational analysis of the tumour immune microenvironment in paediatric ependymoma.

Thioredoxin system protein expression is associated with poor clinical outcome in adult and paediatric gliomas and medulloblastomas (2020)
Journal Article
Martin, S., Yao, A., Storr, S. J., Al-Hadyan, K., Rahman, R., Smith, S., …Paine, S. (2020). Thioredoxin system protein expression is associated with poor clinical outcome in adult and paediatric gliomas and medulloblastomas. Molecular Neurobiology, 57(7), 2889–2901. https://doi.org/10.1007/s12035-020-01928-z

The thioredoxin (Trx) system is an important enzyme family that regulates cellular redox homeostasis. Protein expression of Trx system family members has been assessed in various cancers and linked to various clinicopathological variables, disease pr... Read More about Thioredoxin system protein expression is associated with poor clinical outcome in adult and paediatric gliomas and medulloblastomas.

Distinguishing between paediatric brain tumour types using multi-parametric magnetic resonance imaging and machine learning: A multi-site study (2020)
Journal Article
Grist, J. T., Withey, S., MacPherson, L., Oates, A., Powell, S., Novak, J., …Peet, A. C. (2020). Distinguishing between paediatric brain tumour types using multi-parametric magnetic resonance imaging and machine learning: A multi-site study. NeuroImage: Clinical, 25, Article 102172. https://doi.org/10.1016/j.nicl.2020.102172

The imaging and subsequent accurate diagnosis of paediatric brain tumours presents a radiological challenge, with magnetic resonance imaging playing a key role in providing tumour specific imaging information. Diffusion weighted and perfusion imaging... Read More about Distinguishing between paediatric brain tumour types using multi-parametric magnetic resonance imaging and machine learning: A multi-site study.

Pineoblastoma segregates into molecular sub-groups with distinct clinico-pathologic features: a Rare Brain Tumor Consortium registry study (2019)
Journal Article
Li, B. K., Vasiljevic, A., Dufour, C., Yao, F., Ho, B. L. B., Lu, M., …Jouvet, A. (2020). Pineoblastoma segregates into molecular sub-groups with distinct clinico-pathologic features: a Rare Brain Tumor Consortium registry study. Acta Neuropathologica, 139(2), 223–241. https://doi.org/10.1007/s00401-019-02111-y

Pineoblastomas (PBs) are rare, aggressive pediatric brain tumors of the pineal gland with modest overall survival despite intensive therapy. We sought to define the clinical and molecular spectra of PB to inform new treatment approaches for this orph... Read More about Pineoblastoma segregates into molecular sub-groups with distinct clinico-pathologic features: a Rare Brain Tumor Consortium registry study.

In search of effective therapies to overcome resistance to Temozolomide in brain tumours (2019)
Journal Article
Grundy, R., Bouzinab, K., Summers, H., Zhang, J., Stevens, M. F. G., Moody, C. J., …Bradshaw, T. D. (2019). In search of effective therapies to overcome resistance to Temozolomide in brain tumours. Cancer Drug Resistance, 2(4), 1018-1031. https://doi.org/10.20517/cdr.2019.64

Glioblastoma multiforme is the most common and lethal brain tumour-type. The current standard of care includes Temozolomide (TMZ) chemotherapy. However, inherent and acquired resistance to TMZ thwart successful treatment. The direct repair protein me... Read More about In search of effective therapies to overcome resistance to Temozolomide in brain tumours.

A C19MC-LIN28A-MYCN Oncogenic Circuit Driven by Hijacked Super-enhancers Is a Distinct Therapeutic Vulnerability in ETMRs: A Lethal Brain Tumor (2019)
Journal Article
Sin-Chan, P., Mumal, I., Suwal, T., Ho, B., Fan, X., Singh, I., …Huang, A. (2019). A C19MC-LIN28A-MYCN Oncogenic Circuit Driven by Hijacked Super-enhancers Is a Distinct Therapeutic Vulnerability in ETMRs: A Lethal Brain Tumor. Cancer Cell, 36(1), 51-67.e7. https://doi.org/10.1016/j.ccell.2019.06.002

© 2019 Elsevier Inc. Embryonal tumors with multilayered rosettes (ETMRs) are highly lethal infant brain cancers with characteristic amplification of Chr19q13.41 miRNA cluster (C19MC) and enrichment of pluripotency factor LIN28A. Here we investigated... Read More about A C19MC-LIN28A-MYCN Oncogenic Circuit Driven by Hijacked Super-enhancers Is a Distinct Therapeutic Vulnerability in ETMRs: A Lethal Brain Tumor.

Switching from linear to macrocyclic gadolinium‐based contrast agents halts the relative T 1 ‐Weighted signal increase in deep gray matter of children with brain tumors: A retrospective study (2019)
Journal Article
Rowe, S. K., Rodriguez, D., Cohen, E., Grundy, R., Morgan, P. S., Jaspan, T., & Dineen, R. A. (2019). Switching from linear to macrocyclic gadolinium‐based contrast agents halts the relative T 1 ‐Weighted signal increase in deep gray matter of children with brain tumors: A retrospective study. Journal of Magnetic Resonance Imaging, https://doi.org/10.1002/jmri.26831

Background Studies have shown signal intensity (SI) changes in the brains of children exposed to repeated doses of a gadolinium‐based contrast agent (GBCA). Hypothesis The trajectory of changes in relative dentate nucleus (DN) and globus pal... Read More about Switching from linear to macrocyclic gadolinium‐based contrast agents halts the relative T 1 ‐Weighted signal increase in deep gray matter of children with brain tumors: A retrospective study.

The European Society of Paediatric Oncology Ependymoma-II program Core-Plus model: Development and initial implementation of a cognitive test protocol for an international brain tumour trial (2019)
Journal Article
Thomas, S., Reynolds, D., Morrall, M., Limond, J., Chevignard, M., Calaminus, G., …Grundy, R. (2019). The European Society of Paediatric Oncology Ependymoma-II program Core-Plus model: Development and initial implementation of a cognitive test protocol for an international brain tumour trial. European Journal of Paediatric Neurology, 23(4), 560-570. https://doi.org/10.1016/j.ejpn.2019.05.009

© 2019 European Paediatric Neurology Society It is increasingly accepted that survival alone is an inadequate measure of the success of childhood brain tumour treatments. Consequently, there is growing emphasis on capturing quality of survival. Epend... Read More about The European Society of Paediatric Oncology Ependymoma-II program Core-Plus model: Development and initial implementation of a cognitive test protocol for an international brain tumour trial.

Outcomes of non-anaplastic stage III and ‘inoperable’ Wilms tumour treated in the UKW3 trial (2018)
Journal Article
Irtan, S., Messahel, B., Moroz, V., Taylor, R. E., Grundy, R., Kelsey, A., …Pritchard-Jones, K. (2019). Outcomes of non-anaplastic stage III and ‘inoperable’ Wilms tumour treated in the UKW3 trial. Radiotherapy and Oncology, 131, 1-7. https://doi.org/10.1016/j.radonc.2018.10.026

© 2018 Background and purpose: To describe the outcome of patients with stage III Wilms tumours (WT) treated in the UKW3 trial. Material and methods: Patients with a pathologically confirmed stage III non-anaplastic WT at nephrectomy (Group A) or wit... Read More about Outcomes of non-anaplastic stage III and ‘inoperable’ Wilms tumour treated in the UKW3 trial.

Chemical modulation of autophagy as an adjunct to chemotherapy in childhood and adolescent brain tumors (2018)
Journal Article
Servante, J., Estranero, J., Meijer, L., Layfield, R., & Grundy, R. (2018). Chemical modulation of autophagy as an adjunct to chemotherapy in childhood and adolescent brain tumors. Oncotarget, 9(81), 35266-35277. https://doi.org/10.18632/oncotarget.26186

Brain tumors are the leading cause of cancer-related death in children and are the most challenging childhood cancer in relation to diagnosis, treatment, and outcome. One potential novel strategy to improve outcomes in cancer involves the manipulati... Read More about Chemical modulation of autophagy as an adjunct to chemotherapy in childhood and adolescent brain tumors.

Tumour compartment transcriptomics demonstrates the activation of inflammatory and odontogenic programmes in human adamantinomatous craniopharyngioma and identifies the MAPK/ERK pathway as a novel therapeutic target (2018)
Journal Article
Apps, J. R., Carreno, G., Gonzalez-Meljem, J. M., Haston, S., Guiho, R., Cooper, J. E., …Martinez-Barbera, J. P. (2018). Tumour compartment transcriptomics demonstrates the activation of inflammatory and odontogenic programmes in human adamantinomatous craniopharyngioma and identifies the MAPK/ERK pathway as a novel therapeutic target. Acta Neuropathologica, 135(5), 757-777. https://doi.org/10.1007/s00401-018-1830-2

Adamantinomatous craniopharyngiomas (ACPs) are clinically challenging tumours, the majority of which have activating mutations in CTNNB1. They are histologically complex, showing cystic and solid components, the latter comprised of different morpholo... Read More about Tumour compartment transcriptomics demonstrates the activation of inflammatory and odontogenic programmes in human adamantinomatous craniopharyngioma and identifies the MAPK/ERK pathway as a novel therapeutic target.

Translating childhood brain tumour research into clinical practice: the experience of molecular classification and diagnostics (2018)
Journal Article
Ritzmann, T. A., & Grundy, R. G. (2018). Translating childhood brain tumour research into clinical practice: the experience of molecular classification and diagnostics. Paediatrics and Child Health, 28(4), https://doi.org/10.1016/j.paed.2018.01.006

Diagnosis and treatment of paediatric brain tumours has shown limited progress over the last half century. However, in the past 10 years the development of molecular techniques for investigating these tumours has expanded exponentially. The use of me... Read More about Translating childhood brain tumour research into clinical practice: the experience of molecular classification and diagnostics.

Integrated (epi)-Genomic Analyses Identify Subgroup-Specific Therapeutic Targets in CNS Rhabdoid Tumors (2016)
Journal Article
Torchia, J., Golbourn, B., Feng, S., Ching Ho, K., Sin-Chan, P., Vasiljevic, A., …Huang, A. (2016). Integrated (epi)-Genomic Analyses Identify Subgroup-Specific Therapeutic Targets in CNS Rhabdoid Tumors. Cancer Cell, 30(6), 891-908. https://doi.org/10.1016/j.ccell.2016.11.003

We recently reported that atypical teratoid rhabdoid tumors (ATRTs) comprise at least two transcriptional subtypes with different clinical outcomes; however, the mechanisms underlying therapeutic heterogeneity remained unclear. In this study, we anal... Read More about Integrated (epi)-Genomic Analyses Identify Subgroup-Specific Therapeutic Targets in CNS Rhabdoid Tumors.

Assessing ‘second-look’ tumour resectability in childhood posterior fossa ependymoma—a centralised review panel and staging tool for future studies (2016)
Journal Article
Millward, C. P., Malluci, C., Jaspan, T., Macarthur, D., Heyward, R., Cox, T., …Grundy, R. G. (2016). Assessing ‘second-look’ tumour resectability in childhood posterior fossa ependymoma—a centralised review panel and staging tool for future studies. Child's Nervous System, 32, 2189–2196. https://doi.org/10.1007/s00381-016-3225-9

Molecular subgroups of atypical teratoid rhabdoid tumours in children: an integrated genomic and clinicopathological analysis (2015)
Journal Article

Background Rhabdoid brain tumours, also called atypical teratoid rhabdoid tumours, are lethal childhood cancers with characteristic genetic alterations of SMARCB1/hSNF5. Lack of biological understanding of the substantial clinical heterogeneity of t... Read More about Molecular subgroups of atypical teratoid rhabdoid tumours in children: an integrated genomic and clinicopathological analysis.

Expression alterations define unique molecular characteristics of spinal ependymomas (2015)
Journal Article
Lourdusamy, A., Rahman, R., & Grundy, R. G. (2015). Expression alterations define unique molecular characteristics of spinal ependymomas. Oncotarget, 6(23), https://doi.org/10.18632/oncotarget.3715

Ependymomas are glial tumors that originate in either intracranial or spinal regions. Although tumors from different regions are histologically similar, they are biologically distinct. We therefore sought to identify molecular characteristics of spin... Read More about Expression alterations define unique molecular characteristics of spinal ependymomas.

Combined hereditary and somatic mutations of replication error repair genes result in rapid onset of ultra-hypermutated cancers (2015)
Journal Article

DNA replication?associated mutations are repaired by two components: polymerase proofreading and mismatch repair. The mutation consequences of disruption to both repair components in humans are not well studied. We sequenced cancer genomes from child... Read More about Combined hereditary and somatic mutations of replication error repair genes result in rapid onset of ultra-hypermutated cancers.

RHPS4 G-quadruplex ligand induces anti-proliferative effects in brain tumor cells (2014)
Journal Article
Lagah, S., Tan, I.-L., Radhakrishnan, P., Hirst, R. A., Ward, J. H., O'Callaghan, C., …Rahman, R. (2014). RHPS4 G-quadruplex ligand induces anti-proliferative effects in brain tumor cells. PLoS ONE, 9(1), Article e86187. https://doi.org/10.1371/journal.pone.0086187

Background Telomeric 3’ overhangs can fold into a four-stranded DNA structure termed G-quadruplex (G4), a formation which inhibits telomerase. As telomerase activation is crucial for telomere maintenance in most cancer cells, several classes of G4... Read More about RHPS4 G-quadruplex ligand induces anti-proliferative effects in brain tumor cells.

Metrics and textural features of MRI diffusion to improve classification of pediatric posterior fossa tumors (2013)
Journal Article
Rodriguez Gutierrez, D., Awwad, A., Meijer, L., Manita, M., Jaspan, T., Dineen, R. A., …Auer, D. P. (in press). Metrics and textural features of MRI diffusion to improve classification of pediatric posterior fossa tumors. American Journal of Neuroradiology, 35(5), https://doi.org/10.3174/ajnr.A3784

BACKGROUND AND PURPOSE: Qualitative radiologic MR imaging review affords limited differentiation among types of pediatric posterior fossa brain tumors and cannot detect histologic or molecular subtypes, which could help to stratify treatment. This st... Read More about Metrics and textural features of MRI diffusion to improve classification of pediatric posterior fossa tumors.

The role of the WNT/β-catenin pathway in central nervous system primitive neuroectodermal tumours (CNS PNETs) (2013)
Journal Article
Rogers, H., Ward, J., Miller, S., Lowe, J., Coyle, B., & Grundy, R. G. (2013). The role of the WNT/β-catenin pathway in central nervous system primitive neuroectodermal tumours (CNS PNETs). British Journal of Cancer, 108, https://doi.org/10.1038/bjc.2013.170

Background: Central nervous system primitive neuroectodermal tumours (CNS PNETs) are embryonal tumours occurring predominantly in children. Current lack of knowledge regarding their underlying biology hinders development of more effective treatments... Read More about The role of the WNT/β-catenin pathway in central nervous system primitive neuroectodermal tumours (CNS PNETs).

Serial MR diffusion to predict treatment response in high-grade pediatric brain tumors: a comparison of regional and voxel-based diffusion change metrics (2013)
Journal Article
Rodriguez Gutierrez, D., Manita, M., Jaspan, T., Dineen, R. A., Grundy, R. G., & Auer, D. P. (in press). Serial MR diffusion to predict treatment response in high-grade pediatric brain tumors: a comparison of regional and voxel-based diffusion change metrics. Neuro-Oncology, 15(8), https://doi.org/10.1093/neuonc/not034

Background Assessment of treatment response by measuring tumor size is known to be a late and potentially confounded response index. Serial diffusion MRI has shown potential for allowing earlier and possibly more reliable response assessment in a... Read More about Serial MR diffusion to predict treatment response in high-grade pediatric brain tumors: a comparison of regional and voxel-based diffusion change metrics.

WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma (2012)
Journal Article
Rogers, H., Sousa, S., Salto, C., Arenas, E., Coyle, B., & Grundy, R. G. (2012). WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma. British Journal of Cancer, 107, https://doi.org/10.1038/bjc.2012.377

Background: Medulloblastoma is the most common malignant childhood brain tumour. Aberrant activation of the WNT/β-catenin pathway occurs in approximately 25% of medulloblastomas. However, its role in medulloblastoma pathogenesis is not understood.... Read More about WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma.

Antiangiogenic therapy and mechanisms of tumor resistance in malignant glioma (2010)
Journal Article
Rahman, R., Smith, S., Rahman, C., & Grundy, R. (2010). Antiangiogenic therapy and mechanisms of tumor resistance in malignant glioma. Journal of Oncology, 2010, 1-16. https://doi.org/10.1155/2010/251231

Despite advances in surgery, radiation therapy, and chemotherapeutics, patients with malignant glioma have a dismal prognosis. The formations of aberrant tumour vasculature and glioma cell invasion are major obstacles for effective treatment. Angioge... Read More about Antiangiogenic therapy and mechanisms of tumor resistance in malignant glioma.

Pediatric Ependymoma: Biological Perspectives (2009)
Journal Article
Kilday, J., Rahman, R., Dyer, S., Ridley, L., Lowe, J., Coyle, B., & Grundy, R. (2009). Pediatric Ependymoma: Biological Perspectives. Molecular Cancer Research, 7(6), 765-786. https://doi.org/10.1158/1541-7786.mcr-08-0584

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 histologic... Read More about Pediatric Ependymoma: Biological Perspectives.

Cellular immortality in brain tumours: an integration of the cancer stem cell paradigm (2009)
Journal Article
Rahman, R., Heath, R., & Grundy, R. (2009). Cellular immortality in brain tumours: an integration of the cancer stem cell paradigm. BBA - Molecular Basis of Disease, 1792(4), 280-288. https://doi.org/10.1016/j.bbadis.2009.01.011

Brain tumours are a diverse group of neoplasms that continue to present a formidable challenge in our attempt to achieve curable intervention. Our conceptual framework of human brain cancer has been redrawn in the current decade. There is a gathering... Read More about Cellular immortality in brain tumours: an integration of the cancer stem cell paradigm.