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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.