Search Our Scientific Publications & Authors

Cancer 2019 Feb 15. Epub 2019 Feb 15.

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

Background: Posterior fossa ependymoma (PFE) comprises 2 groups, PF group A (PFA) and PF group B (PFB), with stark differences in outcome. However, to the authors' knowledge, the long-term outcomes of PFA ependymoma have not been described fully. The objective of the current study was to identify predictors of survival and neurocognitive outcome in a large consecutive cohort of subgrouped patients with PFE over 30 years. Read More

BMC Bioinformatics 2019 Jan 31;20(1):60. Epub 2019 Jan 31.

Department of Medicine, Division of Medical Genetics, University of California San Diego (UCSD), San Diego, USA.

Background: High-throughput technologies for analyzing chromosome conformation at a genome scale have revealed that chromatin is organized in topologically associated domains (TADs). While TADs are relatively stable across cell types, intra-TAD activities are cell type specific. Epigenetic profiling of different tissues and cell-types has identified a large number of non-coding epigenetic regulatory elements ('enhancers') that can be located far away from coding genes. Read More

Pediatr Neurosurg 2019 Jan 30:1-10. Epub 2019 Jan 30.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing,

Objective: The purpose of this study was to explore the clinical features and risk factors of outcomes in pediatric posterior cranial fossa ependymoma. We aim to provide evidence-based recommendations for the improvement of prognoses.

Patients And Methods: The clinical data, treatment modalities, approaches performed, recurrence rates and times, as well as the outcomes of 94 cases were analyzed retrospectively. Read More

Acta Neurochir (Wien) 2019 Jan 29. Epub 2019 Jan 29.

Department of Neurosurgery, Goethe University Hospital, Schleusenweg 2-16, 60528, Frankfurt am Main, Germany.

Background: Data about the influence of pregnancy on progression-free survival and overall survival of glioma patients are sparse and controversial. We aimed at providing further evidence on this relation.

Methods: The course of 18 glioma patients giving birth to 23 children after tumor surgery was reviewed and compared to the course of 18 nulliparous female patients matched for tumor diagnosis including molecular markers, extent of resection, and tumor location. Read More

J Pediatr Hematol Oncol 2018 Oct 23. Epub 2018 Oct 23.

Departments of Neurosciences.

Successful use of immune checkpoint inhibitors in a variety of cancers has generated interest in using this approach in pediatric brain tumors. We performed a retrospective review of 10 consecutive children (6 boys, 4 girls; ages, 2 to 17 y), with recurrent or refractory pediatric brain tumors (5 high-grade glioma, 1 low-grade glioma, pineoblastoma, medulloblastoma, ependymoma, and CNS embryonal tumor, NOS) treated at Rady Children's Hospital San Diego from 2015 to 2017 with the immune checkpoint inhibitor nivolumab (3 mg/kg every 2 wk). Eight of 10 patients received prior chemotherapy and 9 radiation therapy. Read More

Turk Neurosurg 2018 Jun 30. Epub 2018 Jun 30.

The First Hospital of Jilin University.

Aim: Objective: To identify ependymoma (EPN) driver genes and key pathways, and also to illuminate the connection between prognosis of EPN patients and expression levels of driver genes.

Material And Methods: Methods: The gene expression profiles of GSE50161, GSE66354, GSE74195, and GSE86574 were analyzed to figure out the differentially expressed genes (DEGs) between tissue of EPN and normal brain samples. To harvest the enrichment functions, pathways and hub genes, the Gene ontology (GO), Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis, and protein-protein interaction (PPI) network analysis were made. Read More

Childs Nerv Syst 2019 Jan 10. Epub 2019 Jan 10.

Departments of Pediatrics, Ribeirão Preto Medical of School, University of São Paulo, Ribeirão Preto, SP, Brazil.

Background: Ependymoma (EPN) is the third most common central nervous system tumor in childhood. Recent advances in the molecular classification of EPN revealed a supratentorial (ST) ependymoma subgroup characterized by C11orf95-RELA fusion.

Case Report: We describe a novel RELA-fusion composed by a chimeric transcript C11orf95-LOC-RELA in a supratentorial WHO grade II EPN occurring in a 4-year-old child. Read More

Neuro Oncol 2019 Jan 8. Epub 2019 Jan 8.

Department of Radiation Oncology, St. Jude Children's Research Hospital.

Background: Pediatric patients with brain tumors who are treated with radiation therapy (RT) are at risk for neurocognitive and psychosocial late effects. Research to date has primarily examined these outcomes at a group level and in isolation. Advanced statistical techniques allow for person-centered analyses, as well as examination of relationships between domain-specific trajectories. Read More

Oncotarget 2018 Nov 23;9(92):36530-36541. Epub 2018 Nov 23.

Children's Brain Tumour Research Centre, School of Medicine, University of Nottingham, Nottingham, UK.

Background: Epigenetic modifications have been shown to play an important role in the classification and pathogenesis of the pediatric brain tumor ependymoma, suggesting they are a potential therapeutic target.

Results: Agents targeting epigenetic modifications inhibited the growth and induced the death of ependymoma cells with variable efficiency. However, this was often not at clinically achievable doses. Read More

Childs Nerv Syst 2018 Dec 15. Epub 2018 Dec 15.

Children's Brain Tumour Research Centre, Academic Division of Child Health, Queen's Medical Centre, University of Nottingham, Nottingham, NG7 2RD, UK.

Purpose: This is the first UK multi-centre case-controlled study with follow-up in excess of 10 years to report the neurocognitive, academic and psychological outcomes of individuals diagnosed with a brain tumour in early childhood. Children enrolled into the UKCCSG CNS 9204 trial, diagnosed with intracranial ependymoma when aged ≤ 36 months old, who received a primary chemotherapy strategy to defer or avoid radiotherapy, were recruited.

Methods: Outcomes of those who relapsed and subsequently received radiotherapy (n = 13) were compared to those enrolled who did not relapse (n = 16), age-matched controls-diagnosed with solid non-central nervous system (SN-CNS; n = 15) tumours or low-grade posterior fossa pilocytic astrocytoma (PFPA; n = 15), and normative data. Read More

Childs Nerv Syst 2019 Feb 4;35(2):267-275. Epub 2018 Dec 4.

Department of Radiotherapy, The Maria Sklodowska-Curie Institute - Oncology Center, Gliwice Branch, Ul. Wybrzeże AK 15, 44-101, Gliwice, Poland.

Purpose: To assess the results and tolerance of radiosurgery/hypofractionated stereotactic radiotherapy performed after craniospinal irradiation for recurrent tumor.

Methods: Fourteen patients aged 3-46 years, diagnosed with medulloblastoma (10), anaplastic ependymoma (3), and primitive neuroectodermal tumor (1). All patients had craniospinal irradiation (CSI) with the total dose of 30. Read More

Acta Neuropathol Commun 2018 Dec 4;6(1):134. Epub 2018 Dec 4.

Division of Brain Tumor Translational Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.

Extensive molecular analyses of ependymal tumors have revealed that supratentorial and posterior fossa ependymomas have distinct molecular profiles and are likely to be different diseases. The presence of C11orf95-RELA fusion genes in a subset of supratentorial ependymomas (ST-EPN) indicated the existence of molecular subgroups. However, the pathogenesis of RELA fusion-negative ependymomas remains elusive. Read More

Surg Neurol Int 2018 23;9:211. Epub 2018 Oct 23.

Department of Anesthesiology, Advocate Illinois Masonic Medical Center, Chicago, Illinois, USA.

Background: Ependymomas represent 50-60% of all brain and central nervous system tumors. Previous lumbar spine surgery for resection of an ependymoma should not be considered a relative contraindication for the administration of epidural/subarachnoid anesthesia to patients in labor.

Case Description: A 34-year-old G1P0, who underwent resection of an L1-L3 intramedullary ependymoma 8 years previously, presented in active labor with residual left leg numbness and tingling. Read More

J Neurooncol 2019 Jan 20;141(2):449-457. Epub 2018 Nov 20.

Developmental and Stem Cell Biology Program, The Arthur and Sonia Labatt Brain Tumour Center, Division of Neurosurgery, Hospital for Sick children, Toronto, ON, Canada.

Background: DNA methylation inhibitors are logical therapeutic candidates for ependymomas originating in the posterior fossa of the brain. Our objective was to test the safety of infusing 5-Azacytidine (5-AZA), a DNA methylation inhibitor, directly into cerebrospinal fluid (CSF) spaces of the fourth ventricle or tumor resection cavity in children with recurrent ependymoma originating in the posterior fossa.

Materials And Methods: In patients with recurrent ependymoma whose disease originated in the posterior fossa, a maximal safe subtotal tumor resection was performed. Read More

Med J Armed Forces India 2018 Oct 5;74(4):407-409. Epub 2017 Oct 5.

Classified Specialist (ENT), Command Hospital (Central Command), Lucknow, India.

Neurofibromatosis is a neurocutaneous, tumor predisposing, inheritable disorder characterized by tumors of the brain and spine and the presence of skin lesions. The most important tumors associated with neurofibromatosis are vestibular nerve schwannomas, with others being meningiomas and ependymomas. The cord is also affected by tumors, ependymoma being the commonest, besides meningiomas and schwannomas. Read More

World Neurosurg 2019 Feb 16;122:e1300-e1304. Epub 2018 Nov 16.

Department of Radiology, Stanford University School of Medicine, Stanford, California, USA.

Background: Current standard of care for children with infratentorial ependymoma includes maximal safe resection and local radiation of 54-59 Gray. High-dose local radiation has been associated with declines in multiple cognitive domains. The anatomic and physiologic correlates of this cognitive decline remain undefined, and there have been no radiographic studies on the long-term effects of this treatment paradigm. Read More

BMJ 2018 Nov 16;363:k4844. Epub 2018 Nov 16.

University of Kent, Chatham Maritime, Kent, UK.

Brain Pathol 2019 01;29(1):75-84

Department of Pathology, University of California, San Francisco, CA.

Myxopapillary ependymomas (MPE) are considered benign (World Health Organization (WHO) grade I) neoplasms with favorable prognosis. However, malignant behavior occurs in a small subset. To our knowledge, only five anaplastic MPEs have been reported without consensus on diagnostic criteria. Read More

J Clin Neurosci 2019 Jan 6;59:310-312. Epub 2018 Nov 6.

Department of Pathology and Molecular Medicine/Neuropathology, McMaster University, Hamilton, Ontario, Canada. Electronic address:

The diagnosis and management of ependymoma may be challenging when there is a comorbidity of ependymoma and Parkinson's disease (PD). We report the first case to demonstrate unusual clinical and pathological features of an ependymoma associated with PD. A 77-year-old male with a history of PD had brain magnetic resonance imaging (MRI) that showed signal abnormalities in the right temporal lobe, most consistent with a low-grade glioma. Read More

Cureus 2018 Jul 18;10(7):e3000. Epub 2018 Jul 18.

Department of Pathology, Osceola Regional Medical Center, Orlando, USA.

Medulloblastoma is the most common type of aggressive pediatric primary brain malignancy. This case describes a 45-year-old Hispanic male with no significant past medical history who presented to the emergency department (ED) complaining of 15 days of 10/10 intractable headaches with one day of lightheadedness, confusion, and loss of balance. An urgent magnetic resonance imaging (MRI) of the brain revealed a 4. Read More

Clin Oncol (R Coll Radiol) 2019 Mar 29;31(3):191-198. Epub 2018 Oct 29.

Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network, Toronto, Canada; Department of Radiation Oncology, University of Toronto, Toronto, Canada.

Despite best available therapy, many children with cancer develop recurrence after multimodal treatment, including initial radiation therapy. Re-irradiation is defined as the use of a second course of radiation therapy with a retreatment volume that overlaps substantially with that of a previously delivered course of radiation therapy. Re-irradiation is an important part of salvage treatment for patients with recurrent ependymoma, diffuse intrinsic pontine glioma, medulloblastoma and germinoma. Read More

Brain Pathol 2018 Oct 16. Epub 2018 Oct 16.

Department of Neuropathology, Sainte-Anne Hospital, Paris, France.

Ependymoma with RELA fusion has been defined as a novel entity of the revised World Health Organization 2016 classification of tumors of the central nervous system (CNS), characterized by fusion transcripts of the RELA gene and consequent pathological activation of the NFkB pathway. These tumors represent the majority of supratentorial ependymomas in children. The validation of diagnostic tools to identify this clinically relevant ependymoma entity is essential. Read More

Front Pharmacol 2018 27;9:00950. Epub 2018 Sep 27.

Department of Pediatric Oncology, La Timone Children's Hospital, Assistance Publique Hôpitaux de Marseille, Marseille, France.

Metronomic chemotherapy (MC) is defined as the frequent administration of chemotherapy at doses below the maximal tolerated dose and with no prolonged drug-free break. MC has shown its efficacy in adult tumor types such as breast and ovarian cancer and has to some extent been studied in pediatrics. To assess the anti-tumor activity and toxicity of a four-drug metronomic regimen in relapsing/refractory pediatric brain tumors (BT) with progression-free survival (PFS) after two cycles as primary endpoint. Read More

Surg Neurol Int 2018 10;9:186. Epub 2018 Sep 10.

International Center for Neurosurgery, Henan Provincial People's Hospital, Zhengzhou, China.

Background: In this video abstract, we present an unedited microsurgical resection of a fourth ventricular ependymoma performed by a senior author (JH). Currently, the goal of a standard treatment of a fourth ventricular ependymoma is based on microsurgical resection followed by radiochemotherapy. Our aim is to demonstrate the efficiency and safety of our microsurgical technique in deep brain territories under the principle "simple, clean, and preserving the normal anatomy. Read More

Cancer 2018 Nov 26;124(21):4168-4180. Epub 2018 Sep 26.

Division of Neuro-Oncology, Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.

Despite significant improvements in pediatric brain tumor therapy and outcome, too many children still die of disease, and too many survivors experience significant sequelae as a result of conventional therapies. The molecular characterization of pediatric brain tumors has afforded tremendous insight into the basic biology and clinical management of these deadly childhood diseases. Genomic, epigenomic, and transcriptional profiling have facilitated the identification of significant heterogeneity among previously uniform disease entities. Read More

Brain Pathol 2018 Sep 23. Epub 2018 Sep 23.

Institute of Neuropathology, University of Bonn Medical Center, Bonn, Germany.

Ependymoma with YAP1-MAMLD1 fusion is a rare, recently described supratentorial neoplasm of childhood, with few cases published so far. We report on 15 pediatric patients with ependymomas carrying YAP1-MAMLD1 fusions, with their characteristic histopathology, immunophenotype and molecular/cytogenetic, radiological and clinical features. The YAP1-MAMLD1 fusion was documented by RT-PCR/Sanger sequencing, and tumor genomes were studied by molecular inversion probe (MIP) analysis. Read More

Neuroradiol J 2018 Dec 19;31(6):554-564. Epub 2018 Sep 19.

1 Department of Radiology and Biomedical Imaging, Yale School of Medicine, New Haven, USA.

Purpose: We applied voxelwise apparent diffusion coefficient (ADC) histogram analysis in addition to structural magnetic resonance imaging (MRI) findings and patients' age for differentiation of intraaxial posterior fossa tumors involving the fourth ventricle.

Participants And Methods: Pretreatment MRIs of 74 patients with intraaxial brain neoplasm involving the fourth ventricle, from January 1, 2004 to December 31, 2015, were reviewed. The tumor solid components were segmented and voxelwise ADC histogram variables were determined. Read More

World Neurosurg 2018 Dec 6;120:125-128. Epub 2018 Sep 6.

Division of Neurosurgery, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Tottori, Japan.

Background: Li-Fraumeni syndrome is a genetic disease that is caused by mutation of the tumor suppressor gene TP53. Patients with this syndrome may develop multiple malignant neoplasms including brain tumors. We herein report the first case of Li-Fraumeni syndrome in which development of supratentorial anaplastic ependymoma led to difficulty in terms of selecting the optimal postoperative therapeutic protocol. Read More

Clin Cancer Res 2018 Dec 31;24(24):6471-6482. Epub 2018 Aug 31.

Knight Cancer Institute, Oregon Health and Sciences University, Portland, Oregon.

Purpose: Gliomas, a genetically heterogeneous group of primary central nervous system tumors, continue to pose a significant clinical challenge. Discovery of chromosomal rearrangements involving kinase genes has enabled precision therapy, and improved outcomes in several malignancies.

Experimental Design: Positing that similar benefit could be accomplished for patients with brain cancer, we evaluated The Cancer Genome Atlas (TCGA) glioblastoma dataset. Read More

Mymensingh Med J 2018 Jul;27(3):665-668

Dr Kohinoor Jahan Shyamaly, Resident Phase-B, Department of General Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh.

Primitive neuroectodermal tumors are malignant tumor of pluripotent cells of neural crest. It has diverse clinical presentation and aggressive clinical behaviour. Clinical features may provide some clue but imaging studies such as MRI of brain; tissue histopathology, immune histochemistry and cytogenetic are essential to confirm the diagnosis. Read More

No Shinkei Geka 2018 Aug;46(8):707-711

Department of Neurosurgery, Faculty of Medicine, Yamagata University.

Introduction: The draining veins of the brain stem and cerebellum commonly drain into the petrosal vein and sigmoid sinus, and often drain into the marginal sinus in the caudal part of the posterior fossa. Here, we report a rare case of anaplastic ependymoma involving a bridging vein that drained directly into the occipital sinus.

Case Description: A 6-year-old boy was admitted to our hospital with a 1-month history of nausea, headache, and dizziness. Read More

J Appl Clin Med Phys 2018 Sep 19;19(5):632-639. Epub 2018 Aug 19.

Department of Radiotherapy, Regensburg University Medical Center, Regensburg, Germany.

Pediatric patients suffering from ependymoma are usually treated with cranial or craniospinal three-dimensional (3D) conformal radiotherapy (3DCRT). Intensity-modulated techniques spare dose to the surrounding tissue, but the risk for second malignancies may be increased due to the increase in low-dose volume. The aim of this study is to investigate if the flattening filter free (FFF) mode allows reducing the risk for second malignancies compared to the mode with flattening filter (FF) for intensity-modulated techniques and to 3DCRT. Read More

Pediatr Radiol 2018 12 17;48(13):1955-1963. Epub 2018 Aug 17.

Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, UK.

Central nervous system neoplasms are the most common solid tumours that develop in children, with the greatest proportion located in the infratentorium. The 2016 World Health Organization Central Nervous System tumour classification evolved from the 2007 edition with the integration of molecular and genetic profiling into the diagnosis, the addition of new entities and the removal of others. Radiology can assist with the subtyping of tumours from certain characteristics described below to provide prognostic information and guide further management. Read More

J Clin Oncol 2018 Oct 17;36(28):2854-2862. Epub 2018 Aug 17.

Ralph E. Vatner, Andrzej Niemierko, Madhusmita Misra, Elizabeth A. Weyman, Claire P. Goebel, David H. Ebb, Robin M. Jones, Mary S. Huang, Takara Stanley, Shannon M. MacDonald, Nancy J. Tarbell, and Torunn I. Yock, Massachusetts General Hospital, Boston, MA; Ralph E. Vatner, University of Cincinnati College of Medicine and Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Anita Mahajan, Mayo Clinic, Rochester, MN; and David R. Grosshans and Arnold C. Paulino, MD Anderson Cancer Center, Houston, TX.

Purpose: There are sparse data defining the dose response of radiation therapy (RT) to the hypothalamus and pituitary in pediatric and young adult patients with brain tumors. We examined the correlation between RT dose to these structures and development of endocrine dysfunction in this population.

Materials And Methods: Dosimetric and clinical data were collected from children and young adults (< 26 years of age) with brain tumors treated with proton RT on three prospective studies (2003 to 2016). Read More

J Neurooncol 2018 Nov 14;140(2):457-465. Epub 2018 Aug 14.

Pediatric Radiotherapy Unit (LG, EP), Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

Purpose: The aims of patients' radiological surveillance are to: ascertain relapse; apply second-line therapy; accrue patients in phase 1/2 protocols if second-line therapy is not standardized/curative; and assess/treat iatrogenic effects. To lessen the emotional and socioeconomic burdens for patients and families, we ideally need to establish whether scheduled radiological surveillance gives patients a better outcome than waiting for symptoms and signs to appear.

Methods: We analyzed a prospective series of 160 newly-diagnosed and treated pediatric/adolescent patients with intracranial ependymoma, comparing patients with recurrent disease identified on scheduled MRI (the RECPT group; 34 cases) with those showing signs/symptoms of recurrent disease (the SYMPPT group; 16 cases). Read More

Int J Radiat Oncol Biol Phys 2018 10 6;102(2):391-398. Epub 2018 Jun 6.

Harvard Medical School, Harvard University, Boston, Massachusetts; Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts.

Purpose: Radiation therapy is integral in treatment of pediatric brain tumors, but it is associated with negative long-term sequelae. Proton beam radiation therapy (PRT), which enables better focusing of radiation on tumors, may entail fewer sequelae. This prospective study examined cognitive and adaptive functioning in children and young adults treated with PRT. Read More

Surg Neurol Int 2018 24;9:144. Epub 2018 Jul 24.

Department of Neurosurgery, Clinical Neurosciences Center, University of Utah, Salt Lake City, Utah, USA.

Background: Ependymomas are rare neuroepithelial tumors thought to arise from radial glial precursor cells lining the walls of the ventricles and central canal of the brain and spinal cord, respectively. Histopathological classification, according to World Health Organization criteria, has only recently defined the RELA-fusion positive ependymoma. These tumors may account for 70% of supratentorial ependymomas in children and represent an aggressive entity distinct from other ependymomas. Read More

World Neurosurg 2018 Nov 9;119:e855-e863. Epub 2018 Aug 9.

Department of Neurosurgery, Medical Faculty, Heinrich Heine University, Düsseldorf, Düsseldorf, Germany.

Objective: To evaluate a new surgical tool combining suction and monopolar neurostimulation (stimulation sucker) for cerebellopontine angle (CPA) tumors. The usefulness for continuous (time) and dynamic (space) facial nerve mapping was studied.

Methods: Patients operated on with the stimulation sucker for a CPA tumor between April 2016 and May 2017 in a tertiary care center were identified. Read More

Sci Rep 2018 Aug 10;8(1):11992. Epub 2018 Aug 10.

Institute of Cancer and Genomic Sciences, University of Birmingham, Birmingham, UK.

Paediatric brain tumors are becoming well characterized due to large genomic and epigenomic studies. Metabolomics is a powerful analytical approach aiding in the characterization of tumors. This study shows that common cerebellar tumors have metabolite profiles sufficiently different to build accurate, robust diagnostic classifiers, and that the metabolite profiles can be used to assess differences in metabolism between the tumors. Read More

Clin Nucl Med 2018 Sep;43(9):e319-e321

A 49-year-old woman presented to the emergency room with subacute paraparesis associated with bilateral cervicobrachial neuralgia. Brain and spine MRI revealed a heterogeneous hypervascular pineal tumor and a multifocal thoracolumbar intradural-extramedullary infiltrate with signs of medullary compression. C7-T2 laminectomy was performed on the same day for medullary decompression, allowing for partial lesion excision. Read More

Clin Neuropathol 2018 Sep/Oct;37(5):221-227

Introduction: Edema is a significant cause of neuromorbidity in children and adults with brain tumors. Agents used to control this effect, such as corticosteroids, have their own associated morbidities. Sulfonylurea receptor 1 (SUR1) is a transmembrane protein that regulates the activity of ion channels in neurons, glia, and endothelial cells. Read More

Ann Transl Med 2018 Jun;6(12):248

Department of Biological Chemistry, Medical School, National and Kapodistrian University of Athens, Athens, Greece.

Brain tumors are regarded as the most prevalent solid neoplasms in children and the principal reason of death in this population. Even though surgical resection, radiotherapy and chemotherapy have improved outcome, a significant number of patients die in 6-12 months after diagnosis while those who survive, frequently experience side effects and relapses. Several studies suggest that many types of cancer including pediatric brain tumors are characterized by alterations in epigenetic profiles with deregulated chromatin remodeling and posttranslational covalent histone modifications playing a prominent role. Read More

Hum Pathol 2018 Dec 29;82:149-157. Epub 2018 Jul 29.

Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India. Electronic address:

Epithelial-to-mesenchymal transition (EMT) plays an important role in invasion and metastasis of various cancers including gliomas. EMT has also been linked to cancer stem cells and resistance to chemotherapy. An initial in-silico data mining in a published ependymoma (EPN) patient series (GSE21687) revealed up-regulation of EMT transcription factors in tumor samples. Read More

Pediatr Radiol 2018 10 30;48(11):1630-1641. Epub 2018 Jul 30.

Institute of Cancer and Genomic Sciences, University of Birmingham, Birmingham, UK.

Background: A tool for diagnosing childhood cerebellar tumours using magnetic resonance (MR) spectroscopy peak height measurement has been developed based on retrospective analysis of single-centre data.

Objective: To determine the diagnostic accuracy of the peak height measurement tool in a multicentre prospective study, and optimise it by adding new prospective data to the original dataset.

Materials And Methods: Magnetic resonance imaging (MRI) and single-voxel MR spectroscopy were performed on children with cerebellar tumours at three centres. Read More

Neuropathology 2018 Oct 26;38(5):557-560. Epub 2018 Jul 26.

Pathology Unit, Department of Diagnostics and Public Health, University and Hospital Trust of Verona, Verona, Italy.

Low-grade neuroepithelial tumors (LGNT) show a broad histopathological spectrum and may be difficult to classify using current World Health Organization (WHO) criteria. A 57-year-old man came to medical attention because of headaches. The patient medical history was otherwise unremarkable. Read More

World Neurosurg 2018 Oct 18;118:e906-e917. Epub 2018 Jul 18.

Seoul National University Biomedical Informatics, Division of Biomedical Informatics, Seoul National University College of Medicine, Seoul, The Republic of Korea. Electronic address:

Background: Although intracranial and spinal ependymomas are histopathologically similar, the molecular landscape is heterogeneous. An urgent need exists to identify differences in the genomic profiles to tailor treatment strategies. In the present study, we delineated differential gene expression patterns between intracranial and spinal ependymomas. Read More

Clin Neuroradiol 2018 Jul 19. Epub 2018 Jul 19.

Reference Center for Neuroradiology, Institute for Diagnostic and Interventional Neuroradiology, University Hospital of Würzburg, Josef-Schneider-Str. 11, 97080, Würzburg, Germany.

Purpose: Epigenetic profiling has recently identified clinically and molecularly distinct subgroups of ependymoma. The 2016 World Health Organization (WHO) classification recognized supratentorial ependymomas (ST-EPN) with REL-associated protein/p65 (RELA) fusion as a clinicopathological entity. These tumors represent 70% of pediatric ST-EPN characterized by recurrent C11orf95-RELA fusion transcripts, which lead to pathological activation of the nuclear factor 'kappa-light-chain-enhancer' of activated B-cells (NF-κB) signaling pathway. Read More

Acta Neuropathol 2018 Aug 17;136(2):227-237. Epub 2018 Jul 17.

Programme in Developmental and Stem Cell Biology, The Hospital for Sick Children, Toronto, ON, Canada.

Posterior fossa ependymoma comprise three distinct molecular variants, termed PF-EPN-A (PFA), PF-EPN-B (PFB), and PF-EPN-SE (subependymoma). Clinically, they are very disparate and PFB tumors are currently being considered for a trial of radiation avoidance. However, to move forward, unraveling the heterogeneity within PFB would be highly desirable. Read More