362 results match your criteria Ependymoma* Neurology


European genetic ancestry associated with risk of childhood ependymoma.

Neuro Oncol 2020 Jun 2. Epub 2020 Jun 2.

Department of Epidemiology and Biostatistics, University of California San Francisco, San Francisco, California, USA.

Background: Ependymoma is a histologically defined central nervous system tumor most commonly occurring in childhood. Population-level incidence differences by race/ethnicity are observed, with individuals of European ancestry at highest risk. We aimed to determine whether extent of European genetic ancestry is associated with ependymoma risk in US populations. Read More

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http://dx.doi.org/10.1093/neuonc/noaa130DOI Listing

Abnormal spindle-like microcephaly-associated (ASPM) gene expression in posterior fossa brain tumors of childhood and adolescence.

Childs Nerv Syst 2020 Jun 26. Epub 2020 Jun 26.

Department of Pediatrics, Pediatric Oncology Institute-GRAACC, Federal University of São Paulo, São Paulo, SP, Brazil.

Purpose: In neurogenesis, ASPM (abnormal spindle-like microcephaly-associated) gene is expressed mainly in the ventricular zone of posterior fossa and is the major determinant in the cerebral cortex. Besides its role in embryonic development, ASPM overexpression promotes tumor growth, including central nervous system (CNS) tumors. This study aims to investigate ASPM expression levels in most frequent posterior fossa brain tumors of childhood and adolescence: medulloblastoma (MB), ependymoma (EPN), and astrocytoma (AS), correlating them with clinicopathological characteristics and tumor solid portion size. Read More

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http://dx.doi.org/10.1007/s00381-020-04740-1DOI Listing

First Onset Hypokalemic Periodic Paralysis Following Surgery for Myxopapillary Ependymoma, Case Report.

World Neurosurg 2020 Jun 22. Epub 2020 Jun 22.

George Emil Palade University of Medicine, Pharmacy, Science and Technology, Tîrgu MureȘ, Romania; Department of Neurology, Tîrgu MureȘ County Clinical Emergency Hospital, Tîrgu MureȘ, Romania.

Background: Hypokalemic periodic paralysis is a rare skeletal muscle channelopathy characterized by intermittent episodes of acute flaccid paralysis with associated hypokalaemia. We present here the case of a first-onset hypokalemic periodic paralysis triggered by a lumbar spinal surgery for tumor resection.

Case Description: A 37-years old male, without any known prior medical conditions who presented with a first-onset attack of hypokalemic paralysis 1. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.06.109DOI Listing

Understanding the Deadly Silence of Posterior Fossa A Ependymoma.

Mol Cell 2020 Jun;78(6):999-1001

Department of Neurology, Stanford University, Stanford, CA, USA. Electronic address:

In a breakthrough study in a recent issue of Cell, Michealraj et al. (2020) demonstrate that posterior fossa A ependymoma, a lethal pediatric brain tumor with a silent genome, is dependent upon metabolic changes associated with hypoxia that drive the tumor's characteristic epigenetic dysregulation. Read More

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http://dx.doi.org/10.1016/j.molcel.2020.05.020DOI Listing

Tailored therapy: Surgical and radiosurgical treatment for two distinct myxopapillary ependymomas in the same patient. A case report.

J Clin Neurosci 2020 Jul 20;77:237-239. Epub 2020 May 20.

Department of Neurosurgery, IRCCS Istituto Ortopedico Galeazzi, Milan, Italy.

Myxopapillary ependymomas are low grade neoplasms, which originate mostly from the medullary conus, cauda equina and the filum terminale. To date the principal treatment is surgical, total- or subtotal removal (GTR or STR), which can be associated with adjuvant radiotherapy. We report a patient with two tumor locations, one larger tumor at the L3 to S1 level and a smaller S2-S3 localized lesion. Read More

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http://dx.doi.org/10.1016/j.jocn.2020.05.004DOI Listing

Locoregional delivery of CAR T cells to the cerebrospinal fluid for treatment of metastatic medulloblastoma and ependymoma.

Nat Med 2020 May 27;26(5):720-731. Epub 2020 Apr 27.

The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, Ontario, Canada.

Recurrent medulloblastoma and ependymoma are universally lethal, with no approved targeted therapies and few candidates presently under clinical evaluation. Nearly all recurrent medulloblastomas and posterior fossa group A (PFA) ependymomas are located adjacent to and bathed by the cerebrospinal fluid, presenting an opportunity for locoregional therapy, bypassing the blood-brain barrier. We identify three cell-surface targets, EPHA2, HER2 and interleukin 13 receptor α2, expressed on medulloblastomas and ependymomas, but not expressed in the normal developing brain. Read More

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http://dx.doi.org/10.1038/s41591-020-0827-2DOI Listing
May 2020
27.363 Impact Factor

Mobile Myxopapillary Ependymoma with Associated Filum Terminale Cyst.

World Neurosurg 2020 Apr 25;139:337-342. Epub 2020 Apr 25.

Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA.

Background: Intradural ependymal cysts are benign, fluid-filled cysts usually situated along the ventral surface of the spinal cord. There are previous reports of 19 intradural cysts in the literature, including one cyst of the filum terminale. Here, we report for the first time the presence of a radiographically occult filum terminale cyst associated with a myxopapillary ependymoma. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.04.095DOI Listing

Leptomeningeal dissemination of low-grade neuroepithelial CNS tumors in adults: a 15-year experience.

Neurooncol Pract 2020 Jan 6;7(1):118-126. Epub 2019 Jul 6.

Department of Neurology, Yale School of Medicine, New Haven, CT, USA.

Background: Leptomeningeal dissemination (LD) in adults is an exceedingly rare complication of low-grade neuroepithelial CNS tumors (LGNs). We aimed to determine relative incidence, clinical presentation, and predictors of outcome.

Methods: We searched the quality control database of the Section of Neuro-Oncology, Yale Cancer Center, for patients with LGN (WHO grade I/II) seen between 2002 and 2017. Read More

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http://dx.doi.org/10.1093/nop/npz020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7104875PMC
January 2020

Parkinsonism caused by Intracranial ependymoma: A rare case report and literature review.

Aging Med (Milton) 2019 Dec 27;2(4):223-226. Epub 2019 Dec 27.

Department of Neurology Zhejiang Hospital Hangzhou China.

Background: Ependymomas, especially intracranial ependymomas, are rare neoplasms of the CNS. The clinical courses of patients with intracranial ependymomas can be quite variable. At present, data on parkinsonism caused by ependymomas are scarce. Read More

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http://dx.doi.org/10.1002/agm2.12093DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7003710PMC
December 2019

Genetics of Common Pediatric Brain Tumors.

Pediatr Neurol 2020 03 15;104:3-12. Epub 2019 Aug 15.

Division of Pediatric Hematology and Oncology, Department of Pediatrics, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas.

Central nervous system tumors are the most common solid tumors in pediatrics and represent the largest cause of childhood cancer-related mortality. Improvements have occurred in the management of these patients leading to better survival, but significant morbidity persists. With the era of next generation sequencing, considerable advances have occurred in the understanding of these tumors both biologically and clinically. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.08.004DOI Listing

Flipped Reposition Laminoplasty for Excision of Intradural Extramedullary Tumors in the Thoracolumbar Spine: A Case Series of 14 Patients.

Asian Spine J 2020 Jun 8;14(3):327-335. Epub 2020 Jan 8.

Central Institute of Orthopaedics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.

Study Design: A retrospective study was done to assess the outcome of the new technique of flipped reposition laminoplasty for excision of intradural extramedullary (IDEM) spinal tumors of the thoracolumbar region.

Purpose: To describe flipped reposition laminoplasty technique and evaluate its outcomes.

Overview Of Literature: Laminectomy has been the conventional approach for the surgical excision of IDEM spinal tumors, but it has potential postoperative complications. Read More

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http://dx.doi.org/10.31616/asj.2019.0034DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7280918PMC

Long-Term Surgical Resection Outcomes of Pediatric Myxopapillary Ependymoma: Experience of Two Centers and Brief Literature Review.

World Neurosurg 2020 Apr 30;136:e245-e261. Epub 2019 Dec 30.

Department of Neurosurgery Intensive Care Unit, University of Health Sciences, Bakırköy Research and Training Hospital for Neurology, Neurosurgery, and Psychiatry, Istanbul, Turkey.

Background: Myxopapillary ependymoma (MPE) is a rarely reported lesion of the spinal cord in the pediatric population. Based on their low mitotic index and slow-growth, MPEs are classified as low-grade glial tumors. However, MPEs have malignant characteristics, such as distant neural axis and extraneural axis dissemination. Read More

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http://dx.doi.org/10.1016/j.wneu.2019.12.128DOI Listing

Psychosis Remitted After Ependymoma Resection in a School-Aged Child.

J Neuropsychiatry Clin Neurosci 2019 Dec 4:appineuropsych19070147. Epub 2019 Dec 4.

The Departments of Psychiatry (Jules-Dole, Borden, Chambers, Salpekar), Neurosurgery (Uribe-Cardenas), Pathology (Bell, Raabe), and Neurology (Salpekar) and the Division of Oncology, Division of Pediatric Oncology, Sidney Kimmel Comprehensive Cancer Center (McReynolds, Raabe), Johns Hopkins University School of Medicine, Baltimore; the Department of Neurological Surgery, Cornell University, New York (Uribe-Cardenas); the Department of Neurologic Surgery, Mayo Clinic, Rochester Minn. (Ahn); and the Kennedy Krieger Institute, Baltimore (Salpekar).

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http://dx.doi.org/10.1176/appi.neuropsych.19070147DOI Listing
December 2019

Role of Diffusion Tensor Imaging Parameters in the Characterization and Differentiation of Infiltrating and Non-Infiltrating Spinal Cord Tumors : Preliminary Study.

Clin Neuroradiol 2019 Nov 21. Epub 2019 Nov 21.

2nd Department of Clinical Radiology, Medical University of Warsaw, ul. Zwirki i Wigury 61, 02-091, Warsaw, Poland.

Background And Purpose: Recent attempts to utilize diffusion tensor imaging (DTI) to identify the extent of microinfiltration of a tumor in the brain have been successful. It was therefore speculated that this technique could also be useful in the spinal cord. The aim of this study was to differentiate between infiltrating and noninfiltrating intramedullary spinal tumors using DTI-derived metrics. Read More

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http://dx.doi.org/10.1007/s00062-019-00851-8DOI Listing
November 2019

The TP53 p.R337H mutation is uncommon in a Brazilian cohort of pediatric patients diagnosed with ependymoma.

Neurol Sci 2020 Mar 14;41(3):691-694. Epub 2019 Nov 14.

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

Background: Ependymoma (EPN) is the third most common childhood cancer of the central nervous system. RELA fusion-positive EPN accounts for approximately 70% of all childhood supratentorial tumors and shows the worst prognosis among the supratentorial EPNs. TP53 mutation is infrequent in RELA fusions EPNs. Read More

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http://dx.doi.org/10.1007/s10072-019-04112-xDOI Listing

Actionable FGFR1 and BRAF mutations in adult circumscribed gliomas.

J Neurooncol 2019 Nov 31;145(2):241-245. Epub 2019 Oct 31.

Sorbonne Université, Inserm, CNRS, UMR S 1127, Institut du Cerveau Et de La Moelle épinière, ICM, AP-HP, Hôpitaux Universitaires La Pitié Salpêtrière - Charles Foix, Service de Neurologie 2-Mazarin, 75013, Paris, France.

Purpose: Circumscribed gliomas -pilocytic astrocytomas (PA), gangliogliomas (GG), ependymomas (EP)- are mostly low-grade tumours but may progress to anaplasia and sometimes surgery can be challenging due to deep anatomical localization. Because of the high frequency of MAPK-pathway alterations and availability of targeted therapies for FGFR1 and BRAF-mutated tumors, we investigated these mutational hotspots in a cohort of adult circumscribed gliomas.

Methods: Adult patients (>15 years) with diagnosis of PA, GG, EP and DNET were retrospectively identified from two institutions databases. Read More

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http://dx.doi.org/10.1007/s11060-019-03306-9DOI Listing
November 2019

Targeted fusion analysis can aid in the classification and treatment of pediatric glioma, ependymoma, and glioneuronal tumors.

Pediatr Blood Cancer 2020 01 8;67(1):e28028. Epub 2019 Oct 8.

Department of Pathology, Children's Hospital Colorado, University of Colorado, Aurora, Colorado.

Background: The use of next-generation sequencing for fusion identification is being increasingly applied and aids our understanding of tumor biology. Some fusions are responsive to approved targeted agents, while others have future potential for therapeutic targeting. Although some pediatric central nervous system tumors may be cured with surgery alone, many require adjuvant therapy associated with acute and long-term toxicities. Read More

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http://dx.doi.org/10.1002/pbc.28028DOI Listing
January 2020
1 Read

Association between patient-reported outcomes and objective disease indices in people with NF2.

Neurol Clin Pract 2019 Aug;9(4):322-329

Johns Hopkins School of Medicine (AS, BS, SL, JOB), the Johns Hopkins Comprehensive Neurofibromatosis Center, Baltimore, MD; Department of Biostatistics and Data Science (F-CH), Wake Forest School of Medicine, Winston-Salem, NC; Division of Biostatistics and Epidemiology (YL), Weill Cornell Medical College, New York; Department of Neurology (RES), Wake Forest School of Medicine, Winston-Salem, NC; and Department of Neurology (RES), Johns Hopkins School of Medicine, Baltimore, MD.

Objective: The association between patient-reported outcomes and currently used clinical trial endpoints, including total vestibular schwannoma (VS) volume and word recognition score (WRS) in neurofibromatosis type 2 (NF2), is not known.

Methods: A prospective observational study enrolling adult patients with NF2 was conducted at a single specialty center. Measures included: NF2 impact on quality of life (NFTI-QOL), short form (SF)-36; total VS volume, WRS; provider- and patient-reported disease severity (ProvSev, PatSev) measured with an institutionally derived multi-item (e. Read More

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http://dx.doi.org/10.1212/CPJ.0000000000000648DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6745746PMC
August 2019
1 Read

A systematic review and meta-analysis of outcomes in pediatric, recurrent ependymoma.

J Neurooncol 2019 Sep 9;144(3):445-452. Epub 2019 Sep 9.

Department of Neurology, University of California, San Francisco, Box 0434, 625 Nelson Rising Lane, San Francisco, CA, 94158, USA.

Purpose: The purpose of this study was to determine outcomes in recurrent pediatric ependymoma.

Methods: We performed a systematic review of PubMed, Embase, Web of Science and the Cochrane Library for studies reporting on survival outcomes for pediatric patients with recurrent ependymoma. We then performed a meta-analysis of all eligible results. Read More

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http://dx.doi.org/10.1007/s11060-019-03255-3DOI Listing
September 2019
1 Read

Brainstem Injury in Pediatric Patients Receiving Posterior Fossa Photon Radiation.

Int J Radiat Oncol Biol Phys 2019 12 28;105(5):1034-1042. Epub 2019 Aug 28.

Department of Radiation Oncology, Brigham and Women's Hospital, Dana-Farber Cancer Institute, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address:

Purpose: Brainstem necrosis is a rare, but dreaded complication of radiation therapy; however, data on the incidence of brainstem injury for tumors involving the posterior fossa in photon-treated patient cohorts are still needed.

Methods And Materials: Clinical characteristics and dosimetric parameters were recorded for 107 pediatric patients who received photon radiation for posterior fossa tumors without brainstem involvement from 2000 to 2016. Patients were excluded if they received a prescription dose <50. Read More

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http://dx.doi.org/10.1016/j.ijrobp.2019.08.039DOI Listing
December 2019
1 Read

Stroke-Like Migraine Attacks after Radiation Therapy Syndrome: Clinical and Imaging Characteristics.

J Clin Imaging Sci 2019 27;9. Epub 2019 Feb 27.

Division of Neuroradiology, Department of Radiology, The Ohio State University Medical Center Columbus, OH 43210 USA.

Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a rare, reversible phenomenon that occurs several years after radiotherapy in patients treated for intracranial neoplastic lesions. Patients typically present with symptoms of headache, seizures, and other focal neurologic deficits concerning for stroke or disease recurrence. In this report, we describe SMART syndrome in a 70-year-old male who developed a persistent right temporal headache, right-sided neck pain, and new-onset seizures 12 years after surgical resection of a temporal anaplastic ependymoma followed by irradiation. Read More

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http://dx.doi.org/10.25259/JCIS-9-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6702857PMC
February 2019
2 Reads

[Superficial siderosis of the central nervous system caused by myxopapillary ependymoma of conus medullaris and cauda equine: a case report and literature review].

Beijing Da Xue Xue Bao Yi Xue Ban 2019 Aug;51(4):769-774

Department of Neurology, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233004, Anhui, China.

Superficial siderosis of the central nervous system (SSCNS) is a rare disorder caused by hemosiderin deposits in the subpial layers of the brain and spinal cord due to prolonged or recurrent low-grade bleeding into the cerebrospinal fluid (CSF). Central nervous system tumor could be one of the sources of bleeding. Some problems exist at present regarding the diagnosis and treatment of SSCNS in China. Read More

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http://dx.doi.org/10.19723/j.issn.1671-167X.2019.04.031DOI Listing
August 2019
3 Reads

MYCN amplification drives an aggressive form of spinal ependymoma.

Acta Neuropathol 2019 12 14;138(6):1075-1089. Epub 2019 Aug 14.

Hopp-Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany.

Spinal ependymal tumors form a histologically and molecularly heterogeneous group of tumors with generally good prognosis. However, their treatment can be challenging if infiltration of the spinal cord or dissemination throughout the central nervous system (CNS) occurs and, in these cases, clinical outcome remains poor. Here, we describe a new and relatively rare subgroup of spinal ependymal tumors identified using DNA methylation profiling that is distinct from other molecular subgroups of ependymoma. Read More

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http://dx.doi.org/10.1007/s00401-019-02056-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6851394PMC
December 2019
4 Reads

Case-based review: ependymomas in adults.

Neurooncol Pract 2018 Aug 24;5(3):142-153. Epub 2018 Jul 24.

Department of Neuro-Oncology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.

Ependymomas are rare primary central nervous system (CNS) tumors in adults. They occur most commonly in the spinal cord, and have classically been graded histologically into World Health Organization (WHO) grades I, II, or III based on the level of anaplasia. Recent data are showing that genetic heterogeneity occurs within the same histological subgroup and that ependymomas arising from different CNS locations have different molecular signatures. Read More

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http://dx.doi.org/10.1093/nop/npy026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6656336PMC
August 2018
2 Reads

Cysts associated with intramedullary ependymomas of the spinal cord: clinical, MRI and oncological features.

J Neurooncol 2019 Sep 23;144(2):385-391. Epub 2019 Jul 23.

Department of Neurosurgery, Hospices Civils de Lyon, Hôpital Neurologique Pierre Wertheimer, 59, Bd Pinel, 69003, Lyon, France.

Purpose: Few published articles have explicitly focused on cysts associated with intramedullary (IM) ependymomas. The objective was to assess the clinical, MRI, and oncological results of patients operated for an IM ependymoma associated with a cystic portion.

Methods: During the study period, 23 IM tumors resected were cystic ependymomas. Read More

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http://dx.doi.org/10.1007/s11060-019-03241-9DOI Listing
September 2019
2 Reads

The medical necessity of advanced molecular testing in the diagnosis and treatment of brain tumor patients.

Neuro Oncol 2019 12;21(12):1498-1508

Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota.

Accurate pathologic diagnoses and molecularly informed treatment decisions for a wide variety of cancers depend on robust clinical molecular testing that uses genomic, epigenomic, and transcriptomic-based tools. Nowhere is this more essential than in the workup of brain tumors, as emphasized by the incorporation of molecular criteria into the 2016 World Health Organization classification of central nervous system tumors and the updated official guidelines of the National Comprehensive Cancer Network. Despite the medical necessity of molecular testing in brain tumors, access to and utilization of molecular diagnostics is still highly variable across institutions, and a lack of reimbursement for such testing remains a significant obstacle. Read More

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http://dx.doi.org/10.1093/neuonc/noz119DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6917404PMC
December 2019
8 Reads

Resection of Myxopapillary Ependymoma of the Filum Terminale: 2-Dimensional Operative Video.

Oper Neurosurg (Hagerstown) 2020 Feb;18(2):E40

Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.

We present the case of a 48-yr-old female who presented with persistent thigh pain and was found to have a heterogeneous mass caudal to the conus most consistent with a myxopapillary ependymoma. We performed L2-3 laminectomies for tumor resection. For this procedure, we used intraoperative ultrasound as well as neuromonitoring. Read More

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http://dx.doi.org/10.1093/ons/opz099DOI Listing
February 2020
7 Reads

Seizures in Pediatric Patients With Primary Brain Tumors.

Pediatr Neurol 2019 08 28;97:50-55. Epub 2019 Mar 28.

Division of Child Neurology, Department of Pediatrics, Montreal, Québec, Canada. Electronic address:

Background: Seizures are one of the most common symptoms of pediatric brain tumors. The purpose of this study was to define seizures related to primary central nervous system tumors and to identify risk factors predictive of seizure occurrence and recurrence.

Methods: We reviewed the records of children treated from January 1, 2004, to January 1, 2018 and collected data including age, gender, tumor location, histology, extent of initial resection, seizure characteristics, treatment modalities, recurrence, and seizure control. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.03.020DOI Listing
August 2019
3 Reads
1.504 Impact Factor

Trends in phenotype in the English paediatric neurofibromatosis type 2 cohort stratified by genetic severity.

Clin Genet 2019 08 29;96(2):151-162. Epub 2019 Apr 29.

Oxford NF2 Unit, Oxford University Hospitals NHS Trust, Oxford, UK.

Childhood onset neurofibromatosis type 2 can be severe and genotype dependent. We present a retrospective phenotypic analysis of all ascertained children in England Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/cge.13551
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http://dx.doi.org/10.1111/cge.13551DOI Listing
August 2019
51 Reads

The role of caveolin-1 in tumors of the brain - functional and clinical implications.

Cell Oncol (Dordr) 2019 Aug 16;42(4):423-447. Epub 2019 Apr 16.

Department of Physiology and Pharmacology, Loma Linda University School of Medicine, Loma Linda, CA, 92354, USA.

Background: Caveolin-1 (cav-1) is the major structural protein of caveolae, the flask-shaped invaginations of the plasma membrane mainly involved in cell signaling. Today, cav-1 is believed to play a role in a variety of disease processes including cancer, owing to the variations of its expression in association with tumor progression, invasive behavior, metastasis and therapy resistance. Since first detected in the brain, a number of studies has particularly focused on the role of cav-1 in the various steps of brain tumorigenesis. Read More

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http://link.springer.com/10.1007/s13402-019-00447-x
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http://dx.doi.org/10.1007/s13402-019-00447-xDOI Listing
August 2019
26 Reads

Molecular grouping and outcomes of young children with newly diagnosed ependymoma treated on the multi-institutional SJYC07 trial.

Neuro Oncol 2019 10;21(10):1319-1330

Department of Oncology, St Jude Children's Research Hospital, Memphis, Tennessee, USA.

Background: This report documents the clinical characteristics, molecular grouping, and outcome of young children with ependymoma treated prospectively on a clinical trial.

Methods: Fifty-four children (aged ≤3 y) with newly diagnosed ependymoma were treated on the St Jude Young Children 07 (SJYC07) trial with maximal safe surgical resection, 4 cycles of systemic chemotherapy, consolidation therapy using focal conformal radiation therapy (RT) (5-mm clinical target volume), and 6 months of oral maintenance chemotherapy. Molecular groups were determined by tumor DNA methylation using Infinium Methylation EPIC BeadChip and profiled on the German Cancer Research Center/Molecular Neuropathology 2. Read More

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https://academic.oup.com/neuro-oncology/advance-article/doi/
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http://dx.doi.org/10.1093/neuonc/noz069DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6784269PMC
October 2019
26 Reads

Evidence-Based Practice: Temozolomide Beyond Glioblastoma.

Curr Oncol Rep 2019 03 5;21(4):30. Epub 2019 Mar 5.

Department of Neurology, University of Michigan, 1500 E. Medical Center Dr., 1914 Taubman Center, Ann Arbor, MI, 48109, USA.

Purpose Of Review: Temozolomide is a first-line treatment for newly diagnosed glioblastoma. In this review, we will examine the use of temozolomide in other contexts for treating gliomas, including recurrent glioblastoma, glioblastoma in the elderly, diffuse low- and high-grade gliomas, non-diffuse gliomas, diffuse intrinsic pontine glioma (DIPG), ependymoma, pilocytic astrocytoma, and pleomorphic xanthoastrocytoma.

Recent Findings: Temozolomide improved survival in older patients with glioblastoma, anaplastic gliomas regardless of 1p/19q deletion status, and progressive ependymomas. Read More

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http://dx.doi.org/10.1007/s11912-019-0783-5DOI Listing
March 2019
11 Reads

Methylation array profiling of adult brain tumours: diagnostic outcomes in a large, single centre.

Acta Neuropathol Commun 2019 02 20;7(1):24. Epub 2019 Feb 20.

Division of Neuropathology, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square, London, WC1N 3BG, UK.

The introduction of the classification of brain tumours based on their DNA methylation profile has significantly changed the diagnostic approach for cases with ambiguous histology, non-informative or contradictory molecular profiles or for entities where methylation profiling provides useful information for patient risk stratification, for example in medulloblastoma and ependymoma. We present our experience that combines a conventional molecular diagnostic approach with the complementary use of a DNA methylation-based classification tool, for adult brain tumours originating from local as well as national referrals. We report the frequency of IDH mutations in a large cohort of nearly 1550 patients, EGFR amplifications in almost 1900 IDH-wildtype glioblastomas, and histone mutations in 70 adult gliomas. Read More

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https://actaneurocomms.biomedcentral.com/articles/10.1186/s4
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http://dx.doi.org/10.1186/s40478-019-0668-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6381711PMC
February 2019
32 Reads

Influence of pregnancy on glioma patients.

Acta Neurochir (Wien) 2019 03 29;161(3):535-543. 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

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http://dx.doi.org/10.1007/s00701-019-03823-6DOI Listing
March 2019
15 Reads
1.788 Impact Factor

A novel type of C11orf95-LOC-RELA fusion in a grade II supratentorial ependymoma: report of a case with literature review.

Childs Nerv Syst 2019 04 10;35(4):689-694. 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

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http://dx.doi.org/10.1007/s00381-018-04028-5DOI Listing
April 2019
10 Reads

High frequency of H3 K27M mutations in adult midline gliomas.

J Cancer Res Clin Oncol 2019 Apr 4;145(4):839-850. Epub 2019 Jan 4.

Department of Neuropathology, Institute of Pathology and Neuropathology, University Hospital of Tuebingen, Eberhard Karls University of Tuebingen, 72076, Tuebingen, Germany.

Purpose: Diffuse midline gliomas, H3 K27M-mutant were introduced as a new grade IV entity in WHO classification of tumors 2016. These tumors occur often in pediatric patients and show an adverse prognosis with a median survival less than a year. Most of the studies on these tumors, previously known as pediatric diffuse intrinsic pontine glioma, are on pediatric patients and its significance in adult patients is likely underestimated. Read More

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http://link.springer.com/10.1007/s00432-018-02836-5
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http://dx.doi.org/10.1007/s00432-018-02836-5DOI Listing
April 2019
49 Reads

Identifying the deficiencies of current diagnostic criteria for neurofibromatosis 2 using databases of 2777 individuals with molecular testing.

Genet Med 2019 07 7;21(7):1525-1533. Epub 2018 Dec 7.

Department of Genomic Medicine, St Mary's Hospital, Manchester Academic Health Sciences Centre (MAHSC), Division of Evolution and Genomic Science, University of Manchester, Manchester, UK.

Purpose: We have evaluated deficiencies in existing diagnostic criteria for neurofibromatosis 2 (NF2).

Methods: Two large databases of individuals fulfilling NF2 criteria (n = 1361) and those tested for NF2 variants with criteria short of diagnosis (n = 1416) were interrogated. We assessed the proportions meeting each diagnostic criterion with constitutional or mosaic NF2 variants and the positive predictive value (PPV) with regard to definite diagnosis. Read More

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http://dx.doi.org/10.1038/s41436-018-0384-yDOI Listing
July 2019
20 Reads
7.329 Impact Factor

Unusual features of ependymoma in a patient with Parkinson's disease.

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

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http://dx.doi.org/10.1016/j.jocn.2018.10.124DOI Listing
January 2019
39 Reads

Surgical Management of Isolated Fourth Ventricular Hydrocephalus Associated with Injury to the Guillain-Mollaret Triangle.

World Neurosurg 2019 Feb 25;122:71-76. Epub 2018 Oct 25.

Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University, Montreal, Quebec, Canada. Electronic address:

Background: The occurrence of isolated fourth ventricle and injury to the Guillain-Mollaret triangle in the setting of posterior fossa ependymoma represents a new association. In this case report, we discuss the clinical, theoretical, and therapeutic aspects of this problem. We describe a lateral transcerebellar trajectory and shunt valve configuration for safe fourth ventricle shunting in a patient with prior posterior fossa surgery. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S18788750183238
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http://dx.doi.org/10.1016/j.wneu.2018.10.091DOI Listing
February 2019
20 Reads
2.417 Impact Factor

Pediatric Spinal Ependymomas.

Med Sci Monit 2018 Oct 5;24:7072-7089. Epub 2018 Oct 5.

Department of Neurosurgery, Bezmialem Vakif University, Istanbul, Turkey.

BACKGROUND The aim of this study was to assess the clinical and radiological outcomes of surgical treatment for primary spinal ependymoma in children. MATERIAL AND METHODS Medical records of 46 primary spinal ependymoma patients who underwent surgery in BRSHH hospital during a 12-year period from 2004 to 2015 were retrospectively reviewed. All pediatric patients (patient age <18 years) were selected as the core sample used for this study. Read More

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https://www.medscimonit.com/abstract/index/idArt/910447
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http://dx.doi.org/10.12659/MSM.910447DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6186154PMC
October 2018
12 Reads

Childhood supratentorial ependymomas with YAP1-MAMLD1 fusion: an entity with characteristic clinical, radiological, cytogenetic and histopathological features.

Brain Pathol 2019 03 11;29(2):205-216. Epub 2018 Nov 11.

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

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http://dx.doi.org/10.1111/bpa.12659DOI Listing
March 2019
54 Reads
3.840 Impact Factor

Pediatric Case of Li-Fraumeni Syndrome Complicated with Supratentorial Anaplastic Ependymoma.

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

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https://linkinghub.elsevier.com/retrieve/pii/S18788750183198
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http://dx.doi.org/10.1016/j.wneu.2018.08.203DOI Listing
December 2018
25 Reads

Rare but Recurrent ROS1 Fusions Resulting From Chromosome 6q22 Microdeletions are Targetable Oncogenes in Glioma.

Clin Cancer Res 2018 12 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

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http://dx.doi.org/10.1158/1078-0432.CCR-18-1052DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6295214PMC
December 2018
20 Reads
8.722 Impact Factor

Differential diagnosis of posterior fossa tumours in children: new insights.

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

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http://link.springer.com/10.1007/s00247-018-4224-7
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http://dx.doi.org/10.1007/s00247-018-4224-7DOI Listing
December 2018
30 Reads

Fourth ventricle ependymoma mimicking benign paroxysmal positional vertigo.

Neurology 2018 08;91(7):327-328

From the Balance Disorders and Ataxia Service (L.P., D.J.S.), The Royal Victorian Eye and Ear Hospital; Florey Institute of Neuroscience and Mental Health (L.P., D.J.S.); Dizzy Day Clinics (L.P., K.M.); Department of Neurosurgery (K.J.D.), Royal Melbourne Hospital; Department of Surgery (K.J.D.), University of Melbourne; Medical Imaging Department (N.T.), St Vincents Hospital; and Cerebellar Ataxia Clinic (D.J.S.), Neuroscience Department, Alfred Health, Melbourne, Australia.

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http://dx.doi.org/10.1212/WNL.0000000000005992DOI Listing
August 2018
28 Reads

DNA methylation-based classification of ependymomas in adulthood: implications for diagnosis and treatment.

Neuro Oncol 2018 11;20(12):1616-1624

Department of Neurology, University Hospital and University of Zurich, Zurich, Switzerland.

Background: Ependymal tumors are glial tumors that commonly manifest in children and young adults. Their classification has remained entirely morphological until recently, and surgery and radiotherapy are the main treatment options, especially in adults. Here we sought to correlate DNA methylation profiles with clinical and pathological characteristics in the prospective cohort of the German Glioma Network. Read More

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http://dx.doi.org/10.1093/neuonc/noy118DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6231197PMC
November 2018
35 Reads

Heterogeneity within the PF-EPN-B ependymoma subgroup.

Acta Neuropathol 2018 08 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

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http://link.springer.com/10.1007/s00401-018-1888-x
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http://dx.doi.org/10.1007/s00401-018-1888-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6373486PMC
August 2018
47 Reads
10.762 Impact Factor

Severe Nightly Thoracic Pain Presenting during Pregnancy: A Case Report.

Case Rep Neurol 2018 May-Aug;10(2):135-139. Epub 2018 Jun 7.

Department of Neurology, Leiden University/Hagaziekenhuis, Leiden / The Hague, the Netherlands.

We present the case of a woman who developed severe nightly thoracic pain during pregnancy without neurological deficits upon examination. Spontaneously after childbirth, the pain was markedly reduced. Further investigation showed that her pain was caused by an ependymoma in the cervicothoracic spinal cord. Read More

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http://dx.doi.org/10.1159/000488756DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6031941PMC
June 2018
29 Reads

Outcomes in children with central nervous system tumors disseminated at presentation: a large single-center experience.

Childs Nerv Syst 2018 11 24;34(11):2259-2267. Epub 2018 Jun 24.

Department of Neurosurgery, Great Ormond Street Hospital for Children NHS Trust, London, WC1N 3JH, UK.

Purpose: Children with disseminated central nervous system (CNS) tumors have worse outcomes than those with solitary disease, but outcomes of disease dissemination at initial presentation have not been systematically studied and compared across tumor groups to date. We evaluated the impact of tumor dissemination at presentation on management and clinical outcomes in a cohort of consecutively treated children in a single neurosurgical unit over a 14-year period.

Methods: Method used was a retrospective review of data on children presenting to Great Ormond Street Hospital, London, UK, with medulloblastoma, primitive neuroectodermal tumor, atypical teratoid rhabdoid tumor, pilocytic astrocytoma, and ependymoma between 2003 and 2016 inclusive. Read More

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http://dx.doi.org/10.1007/s00381-018-3871-1DOI Listing
November 2018
19 Reads

Subependymomas - Characteristics of a "Leave me Alone" Lesion.

Rofo 2018 10 18;190(10):955-966. Epub 2018 Jun 18.

Goethe-University, Institute for Neuroradiology, Frankfurt, Germany.

Purpose: Intracranial subependymomas are rare, mostly asymptomatic tumours, which are often found incidentally and therefore did not receive much attention in previous literature. By being classified as benign grade I in the WHO classification of tumours of the central nervous system, they are given a special status compared to the other ependymal tumours. Tumor recurrences are a rarity, spinal "drop metastases" do not occur. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/a-0576-1028
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http://dx.doi.org/10.1055/a-0576-1028DOI Listing
October 2018
48 Reads