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Cancers (Basel) 2021 Apr 27;13(9). Epub 2021 Apr 27.

Children's Brain Tumour Research Centre, School of Medicine, University of Nottingham Biodiscovery Institute, Nottingham NG7 2RD, UK.

Paediatric ependymomas are aggressive, treatment-resistant tumours with a tendency towards relapse, consistent with a sub-population of therapy-resistant cancer stem cells. These cells are believed to derive from brain lipid binding protein (BLBP)-expressing radial glia, hence we proposed that BLBP may be a marker for ependymoma therapy resistance. BLBP protein expression correlated with reduced overall survival (OS) in patients from two trials (CNS9204, a chemotherapy-led infant trial-5 y OS 45% vs. Read More

Metabolites 2021 Apr 14;11(4). Epub 2021 Apr 14.

Children's Brain Tumour Research Centre, School of Medicine, Biodiscovery Institute, University of Nottingham, Nottingham NG7 2RD, UK.

The integration of untargeted metabolomics and transcriptomics from the same population of cells or tissue enhances the confidence in the identified metabolic pathways and understanding of the enzyme-metabolite relationship. Here, we optimised a simultaneous extraction method of metabolites/lipids and RNA from ependymoma cells (BXD-1425). Relative to established RNA (mirVana kit) or metabolite (sequential solvent addition and shaking) single extraction methods, four dual-extraction techniques were evaluated and compared (methanol:water:chloroform ratios): cryomill/mirVana (1:1:2); cryomill-wash/Econospin (5:1:2); rotation/phenol-chloroform (9:10:1); Sequential/mirVana (1:1:3). Read More

Asian Pac J Cancer Prev 2021 Apr 1;22(4):1129-1136. Epub 2021 Apr 1.

Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.

Background: To evaluate the role of diffusion MRI in differentiating pediatric posterior fossa tumors and determine the cut-off values of ADC ratio to distinguish medulloblastoma from other common tumors.

Methods: We retrospectively reviewed MRI of 90 patients (7.5-year median age) with pathologically proven posterior fossa tumors (24 medulloblastoma, 7 ependymoma, 4 anaplastic ependymoma, 13 pilocytic astrocytoma, 30 diffuse intrinsic pontine glioma (DIPG), 4 ATRT, 3 diffuse astrocytoma, 2 high grade astrocytoma, 2 glioblastoma, and 1 low grade glioma). Read More

Anal Chem 2021 May 26;93(18):6947-6954. Epub 2021 Apr 26.

Advanced Materials & Healthcare Technologies Division, School of Pharmacy, University of Nottingham, University Park, Nottingham NG7 2RD, U.K.

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 extraction surface analysis-tandem mass spectrometry (LESA-MS/MS) permitted the detection of 887 metabolites (163 chemical classes) from pediatric ependymoma tumor tissue microarrays (diameter: <1 mm; thickness: 4 μm). From these 163 classes, 60 classes were detected with both techniques, whilst LESA-MS/MS and 3D OrbiSIMS individually allowed the detection of another 83 and 20 unique metabolite classes, respectively. Read More

J Avian Med Surg 2021 Apr;35(1):75-79

FauneVET, 22 rue René Viviani 44200 Nantes, France.

A 14-year-old unsexed African grey parrot () was presented with a 12-hour history of neurological signs and vomiting. The external physical examination of the patient revealed lethargy, moderate hypothermia, a head tilt, and horizontal nystagmus. Whole-body radiographic imaging and blood biochemistry parameters were unremarkable, and a serological test to detect bornavirus was negative. Read More

Oncol Rev 2021 Feb 17;15(1):518. Epub 2021 Mar 17.

Neurosurgery Department, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Myxopapillary ependymoma are rare tumors and optimal therapeutic strategy is remained controversial. The main treatments for myxopapillary ependymoma tumors include surgery and radiotherapy. Hence, the present study aimed to review adjuvant treatment of myxopapillary ependymoma, focusing on spinal myxopapillary ependymoma. Read More

Clin Oncol (R Coll Radiol) 2021 Apr 2. Epub 2021 Apr 2.

Department of Particle Therapy, University Hospital Essen, West German Proton Therapy Centre Essen, West German Cancer Centre, Essen, Germany; Department of Particle Therapy, University Hospital Essen, West German Proton Therapy Centre Essen (WPE), West German Cancer Center (WTZ), German Cancer Consortium (DKTK), Essen, Germany.

Aims: Proton beam therapy (PBT) has increasingly been applied for the treatment of young children when radiotherapy is needed. The treatment requires intensive multimodality care and is logistically demanding. In this analysis, we evaluated our experiences in treating infants with tumours of the central nervous system with PBT. Read More

Diagnostics (Basel) 2021 Mar 9;11(3). Epub 2021 Mar 9.

Department of Pediatric Hemato-Oncology and Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Autophagy is a degradative process occurring in eukaryotic cells to maintain homeostasis and cell survival. After stressful conditions including nutrient deprivation, hypoxia or drugs administration, autophagy is induced to counteract pathways that could lead to cell death. In cancer, autophagy plays a paradoxical role, acting both as tumour suppressor-by cleaning cells from damaged organelles and inhibiting inflammation or, alternatively, by promoting genomic stability and tumour adaptive response-or as a pro-survival mechanism to protect cells from stresses such as chemotherapy. Read More

Environ Res 2021 Mar 31;197:111078. Epub 2021 Mar 31.

Department of Epidemiology, Fielding School of Public Health, University of California, 650 Charles E. Young Dr. S, Box 951772, Los Angeles, CA, 90095-1772, USA; Jonsson Comprehensive Cancer Center, University of California, Box 951781, Los Angeles, CA, 90095-1781, USA; College of Health and Public Service, University of North Texas, 1155 Union Circle #311340, Denton, TX, 76203-5017, USA. Electronic address:

Background: Pesticide exposures have been examined previously as risk factors for childhood brain cancers, but few studies were able to assess risk from specific agents.

Objective: To evaluate risks for childhood central nervous system tumors associated with residential proximity to agricultural pesticide applications.

Methods: Using the California Cancer Registry, we identified cancer cases less than 6 years of age and frequency matched them by year of birth to 20 cancer-free controls identified from birth certificates. Read More

Clin Ter 2021 Mar;172(2):99-103

Department of Radiology, Hanoi Medical University, Ha Noi, Vietnam.

Abstract: Choroid plexus papilloma (CPP), a low incidence central nervous system (CNS) tumor, typically develops as an intraventricular neoplasm arising from the epithelium of the choroid plexus. Infratentorial CPP is predominantly clustered in adults with roughly 70% in the fourth ventricle, while supratentorial CPP commonly found in the lateral ventricles, is the most frequent location in children. The clinical and imaging features of CPP are not typical and may induce the misdiagnosis as other types of primary brain tumors. Read More

Pediatr Neurosurg 2021 24;56(2):184-196. Epub 2021 Mar 24.

Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India.

Introduction: Spinal atypical teratoid/rhabdoid tumour (AT/RT) is exquisitely rare and constitutes 2% of all AT/RTs.

Case Presentation: A 6-year-old boy presented with low backache for the last 5 months. MRI of the spine showed a 1. Read More

Turk Neurosurg 2020 Sep 1. Epub 2020 Sep 1.

Erciyes University Faculty of Medicine.

Aim: Torticollis can originate from a wide variety of different disorders.

Objective: We aimed to investigate the underlying conditions in children with torticollis.

Material And Methods: Between May 2016 and December 2019, 24 patients (10 girls and 14 boys; mean age, 8 years) presenting with twisted neck, neck pain, weakness of extremities, imbalance, and gait disorder were evaluated retrospectively. Read More

Int J Surg Case Rep 2021 Apr 11;81:105747. Epub 2021 Mar 11.

Spine Center, Sanbo Brain Hospital, Capital Medical University, Beijing, People's Republic of China. Electronic address:

Introduction And Importance: Huge intramedullary tumor is a rare condition. Surgical management of such huge ependymoma is technically challenging. We reported one case of 300 mm long intramedullary spinal cord ependymoma from oblongata to T4, which was satisfactorily en bloc gross total resected and the cervical spine alignment was successfully maintained by laminoplasty. Read More

Cancer Discov 2021 Mar 19. Epub 2021 Mar 19.

Oncology/CRUK Cambridge Institute, University of Cambridge

ZFTA (C11orf95)-a gene of unknown function-partners with a variety of transcriptional co-activators in translocations that drive supratentorial ependymoma, a frequently lethal brain tumor. Understanding the function of ZFTA is key to developing therapies that inhibit these fusion proteins. Here, using a combination of transcriptomics, chromatin immunoprecipitation-sequencing, and proteomics, we interrogated a series of deletion-mutant genes to identify a tri-partite transformation mechanism of ZFTA-containing fusions, including: spontaneous nuclear translocation, extensive chromatin binding, and SWI/SNF, SAGA and NuA4/Tip60 HAT chromatin modifier complex recruitment. Read More

Cancer Discov 2021 Mar 19. Epub 2021 Mar 19.

Texas Children's Cancer Center.

Over 60% of supratentorial (ST) ependymomas harbor a ZFTA-RELA (ZRfus) gene fusion (formerly C11orf95-RELA). To study the biology of ZRfus, we developed an autochthonous mouse tumor model using in utero electroporation (IUE) of the embryonic mouse brain. Integrative epigenomic and transcriptomic mapping was performed on IUE driven ZRfus tumors by CUT&RUN, ChIP, ATAC, and RNA sequencing and compared to human ZRfus driven ependymoma. Read More

Am J Surg Pathol 2021 Jan 11. Epub 2021 Jan 11.

*Laboratory of Neural Reconstruction, Beijing Key Laboratory of Central Nervous System Injury §Department of Neuropathology, Beijing Neurosurgical Institute †Department of Neurosurgery, Beijing Tian Tan Hospital ‡Department of Pathology, Xuanwu Hospital, Capital Medical University, Beijing, China.

Adult intracranial ependymomas (EPNs) are extremely rare brain tumors. Currently, clinical and molecular factors that could inform individualized treatment strategies are still lacking for EPNs in this age group. The aim of this study was to investigate potential prognostic indicators and rational therapeutic management in a large cohort of adult intracranial EPNs. Read More

J Neurol Surg B Skull Base 2021 Feb 17;82(Suppl 1):S53-S54. Epub 2020 Sep 17.

Department of Neurosurgery, Changzheng Hospital, Naval Medical University, Neurosurgical Institute of Shanghai, Neurosurgical Institute of People's Liberation Army, Shanghai, China.

Ependymoma is one of the most common pediatric tumors in central nervous system, for which gross total resection has been the most favorable prognostic factor. 1 2 However, surgery of ependymomas located in brain stem is significantly challenging. This video demonstrates the microsurgical removal of an ependymoma originating from ependymal cells of the lateral recess of fourth ventricle via retrosigmoid approach in an 11-year-old female. Read More

J Neurosurg Pediatr 2021 Mar 12:1-6. Epub 2021 Mar 12.

Divisions of1Neurosurgery and.

Objective: Facial palsy can be caused by masses within the posterior fossa and is a known risk of surgery for tumor resection. Although well documented in the adult literature, postoperative facial weakness after posterior fossa tumor resection in pediatric patients has not been well studied. The objective of this work was to determine the incidence of postoperative facial palsy after tumor surgery, and to investigate clinical and radiographic risk factors. Read More

Pediatr Neurosurg 2021 9;56(2):146-151. Epub 2021 Mar 9.

Maxine Dunitz Neurosurgical Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA,

Introduction: Down syndrome (DS) is the most common multiple malformation syndrome in humans and is associated with an increased risk of childhood malignancy, particularly leukemia. Incidence of brain tumors in patients with DS is limited to sporadic cases. We report the first case of a RELA fusion-positive ependymoma in a 3-year-old boy with DS. Read More

Acta Neuropathol Commun 2021 03 8;9(1):36. Epub 2021 Mar 8.

Division of Brain Tumor Translational Research, National Cancer Center Research Institute, Chuo-ku, Tokyo, 104-0045, Japan.

Recurrent C11orf95-RELA fusions (RELA) are the hallmark of supratentorial ependymomas. The presence of RELA as the fusion partner indicates a close association of aberrant NF-κB activity with tumorigenesis. However, the oncogenic role of the C11orf95 has not been determined. Read More

AJNR Am J Neuroradiol 2021 Mar 4. Epub 2021 Mar 4.

Departments of Radiology (U.L., K.M., F.D.).

Background And Purpose: Primary posterior fossa tumors comprise a large group of neoplasias with variable aggressiveness and short and long-term outcomes. This study aimed to validate the clinical usefulness of a radiologic decision flow chart based on previously published neuroradiologic knowledge for the diagnosis of posterior fossa tumors in children.

Materials And Methods: A retrospective study was conducted (from January 2013 to October 2019) at 2 pediatric referral centers, Children's Hospital of Philadelphia, United States, and Great Ormond Street Hospital, United Kingdom. Read More

PLoS One 2021 1;16(3):e0247899. Epub 2021 Mar 1.

Doctoral Program, Department of Radiology, Hanoi Medical University, Hanoi, Vietnam.

Introduction: Intravoxel incoherent motion (IVIM) imaging concurrently measures diffusion and perfusion parameters and has potential applications for brain tumor classification. However, the effectiveness of IVIM for the differentiation between pilocytic astrocytoma and ependymoma has not been verified. The aim of this study was to determine the potential diagnostic role of IVIM for the distinction between ependymoma and pilocytic astrocytoma. Read More

J Neurosurg Pediatr 2021 Feb 26:1-10. Epub 2021 Feb 26.

1Department of Paediatric Neurosurgery, Alder Hey Children's NHS Foundation Trust, Liverpool.

Objective: Complications in pediatric neurooncology surgery are seldom and inconsistently reported. This study quantifies surgical morbidity after pediatric brain tumor surgery from the last decade in a single center, using existing morbidity and outcome measures.

Methods: The authors identified all pediatric patients undergoing surgery for an intracranial tumor in a single tertiary pediatric neurosurgery center between January 2008 and December 2018. Read More

J Neurooncol 2021 May 23;152(3):491-499. Epub 2021 Feb 23.

Department of Neurosurgery, Sorbonne University, AP-HP, La Pitié-Salpêtrière Hospital, 47-83, boulevard de L'Hôpital, 75651, Paris Cedex 13, France.

Purpose: Myxopapillary ependymoma (MPE) is the most frequent tumor affecting the medullary conus. The surgical therapeutic management is still debated and only few studies have focused on the postoperative clinical outcome of patients. This study aimed to demonstrate long-term postoperative outcome and to assess the predictive factors of recurrence as well as the clinical evolution of these patients. Read More

Cytopathology 2021 Feb 19. Epub 2021 Feb 19.

Department of Histopathology, Post Graduate Institute of Medical Education and Research, PGIMER, Chandigarh, India.

Objective: Intraoperative crush/squash smear or frozen section consultation is routinely performed at several centers and offers rapid onsite assessment of tumor type and provides invaluable information to the neurosurgeons. The WHO classification of central nervous system neoplasms underwent a paradigm shift in 2016 with the incorporation of molecular data with the morphological features, such that several new entities came to be distinctly defined. With this paper, we present our experience at intraoperative consultation of brain tumors arising in posterior fossa and aim to apprise the pathologists with the spectrum of cytomorphologic appearances that can occur during such consultation and highlight the diagnostic dilemmas and pitfalls encountered in this setting. Read More

Klin Padiatr 2021 May 17;233(3):127-134. Epub 2021 Feb 17.

Department of Pediatric Oncology and Hematology, Charité Universitätsmedizin Berlin, Berlin, Germany.

Background: MRI has shortcomings in differentiation between tumor tissue and post-therapeutic changes in pretreated brain tumor patients.

Patients: We assessed 22 static FET-PET/CT-scans of 17 pediatric patients (median age 12 years, range 2-16 years, ependymoma n=4, medulloblastoma n=4, low-grade glioma n=6, high-grade glioma n=3, germ cell tumor n=1, choroid plexus tumor n=1, median follow-up: 112 months) with multimodal treatment.

Method: FET-PET/CT-scans were analyzed visually by 3 independent nuclear medicine physicians. Read More

J Mol Neurosci 2021 Feb 17. Epub 2021 Feb 17.

Department of Neurosurgery (Divisions of Neuro-Oncology), MUSC Brain & Spine Tumor Program CSB 310, Medical University of South Carolina, Charleston, SC, 29425, USA.

The P gene maps precisely to human chromosome sub-band 22q12.1-12.3, a region where loss of heterozygosity has been reported in 30% of astrocytic tumors and associated with progression to anaplasia. Read More

Neuro Oncol 2021 Feb 13. Epub 2021 Feb 13.

Division of Haematology/Oncology, Hospital for Sick Children, Toronto, ON.

Background: Within PF-EPN-A, 1q gain is a marker of poor prognosis, however, it is unclear if within PF-EPN-A additional cytogenetic events exist which can refine risk stratification.

Methods: Five independent non-overlapping cohorts of PF-EPN-A were analyzed applying genome wide methylation arrays for chromosomal and clinical variables predictive of survival.

Results: Across all cohorts, 663 PF-EPN-A were identified. Read More

Brain Pathol 2021 Feb 12:e12943. Epub 2021 Feb 12.

Department of Human Pathology, Gunma University Graduate School of Medicine, Maebashi, Japan.

Recurrent fusion genes involving C11orf95, C11orf95-RELA, have been identified only in supratentorial ependymomas among primary CNS tumors. Here, we report hitherto histopathologically unclassifiable high-grade tumors, under the tentative label of "ependymoma-like tumors with mesenchymal differentiation (ELTMDs)," harboring C11orf95-NCOA1/2 or -RELA fusion. We examined the clinicopathological and molecular features in five cases of ELTMDs. Read More