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.

Evaluating the efficacy and safety of single-agent etoposide intra-CSF chemotherapy in children and young people with relapsed/refractory central nervous system tumours (2023)
Journal Article
Butler, A., Meijer, L., Liu, J., Chohan, M., Jalloh, I., Macarthur, D., …Dandapani, M. (2023). Evaluating the efficacy and safety of single-agent etoposide intra-CSF chemotherapy in children and young people with relapsed/refractory central nervous system tumours. Child's Nervous System, 39, 1537-1544. https://doi.org/10.1007/s00381-023-05872-w

Purpose The aim of the project was to evaluate intra-CSF etoposide administration in a palliative setting for children and young people with relapsed/refractory central nervous system (CNS) tumours, with the primary endpoints being overall survival... Read More about Evaluating the efficacy and safety of single-agent etoposide intra-CSF chemotherapy in children and young people with relapsed/refractory central nervous system tumours.

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, 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.

Characterisation of Expression the Arginine Pathway Enzymes in Childhood Brain Tumours to Determine Susceptibility to Therapeutic Arginine Depletion (2022)
Journal Article
Bishop, E., Dimitrova, M., Froggatt, A., Estevez-Cebrero, M., Storer, L. C. D., Mussai, F., …Dandapani, M. (2022). Characterisation of Expression the Arginine Pathway Enzymes in Childhood Brain Tumours to Determine Susceptibility to Therapeutic Arginine Depletion. BioMed Research International, 2022, Article 9008685. https://doi.org/10.1155/2022/9008685

Despite significant improvements in treatment and survival in paediatric cancers, outcomes for children with brain tumours remain poor. Novel therapeutic approaches are needed to improve survival and quality of survival. Extracellular arginine depend... Read More about Characterisation of Expression the Arginine Pathway Enzymes in Childhood Brain Tumours to Determine Susceptibility to Therapeutic Arginine Depletion.

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.

Recurrent ACVR1 mutations in posterior fossa ependymoma (2022)
Journal Article
Pratt, D., Lucas, C. G., Selvam, P. P., Abdullaev, Z., Ketchum, C., Quezado, M., …Aldape, K. (2022). Recurrent ACVR1 mutations in posterior fossa ependymoma. Acta Neuropathologica, 144, 373-376. https://doi.org/10.1007/s00401-022-02435-2

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).