395 results match your criteria Ependymoma* Neurology

Pathology-proven extradural ("distant") metastases of gliomas in adults in the Netherlands between 1971 and 2018: a systematic case series.

Neurooncol Pract 2021 Jun 22;8(3):317-324. Epub 2021 Jan 22.

Center of Expertise for Cancer Survivorship, Radboud University Medical Center, Nijmegen, the Netherlands.

Background: Diffuse gliomas are the most frequent primary tumors originating in the central nervous system parenchyma. Although the majority of these tumors are highly malignant, extradural metastases (EDM) are extremely rare. We aimed to perform a systematic review of patients with pathology-proven EDM of diffuse gliomas in the Netherlands. Read More

View Article and Full-Text PDF

Targeting fibroblast growth factor receptors to combat aggressive ependymoma.

Acta Neuropathol 2021 May 27. Epub 2021 May 27.

Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria.

Ependymomas (EPN) are central nervous system tumors comprising both aggressive and more benign molecular subtypes. However, therapy of the high-risk subtypes posterior fossa group A (PF-A) and supratentorial RELA-fusion positive (ST-RELA) is limited to gross total resection and radiotherapy, as effective systemic treatment concepts are still lacking. We have recently described fibroblast growth factor receptors 1 and 3 (FGFR1/FGFR3) as oncogenic drivers of EPN. Read More

View Article and Full-Text PDF

Multisegmental versus monosegmental intramedullary spinal cord ependymomas: perioperative neurological functions and surgical outcomes.

Neurosurg Rev 2021 May 14. Epub 2021 May 14.

Department of Neurosurgery, Peking University Third Hospital, Peking University, Beijing, China.

Multiple factors, such as tumor size, lateralization, tumor location, accompanying syringomyelia, and regional spinal cord atrophy, may affect the resectability and clinical prognosis of intramedullary spinal cord ependymomas. However, whether long-segmental involvement of the spinal cord may impair functional outcomes remains unclear. This study was aimed to compare perioperative neurological functions and long-term surgical outcomes between multisegmental ependymomas and their monosegmental counterparts. Read More

View Article and Full-Text PDF

A coordinated approach for the assessment of molecular subgroups in pediatric ependymomas using low-cost methods.

J Mol Med (Berl) 2021 Apr 26. Epub 2021 Apr 26.

Department of Paediatrics, Ribeirão Preto Medical School, 3900 Bandeirantes Avenue, Ribeirão Preto, SP, 14049-900, Brazil.

Although ependymoma (EPN) molecular subgroups have been well established by integrated high-throughput platforms, low- and middle-income countries still need low-cost techniques to promptly classify these molecular subtypes. Here, we applied low-cost methods to classify EPNs from a Brazilian cohort with 60 pediatric EPN patients. Fusion transcripts (C11orf95-RELA, YAP1-MAMLD1, and YAP1-FAM118B) were investigated in supratentorial EPN (ST-EPNs) samples through RT-PCR/Sanger sequencing and immunohistochemistry (IHC) for p65/L1CAM. Read More

View Article and Full-Text PDF

Cross-species genomics reveals oncogenic dependencies in ZFTA/C11orf95 fusion-positive supratentorial ependymomas.

Cancer Discov 2021 Apr 20. Epub 2021 Apr 20.

Department of Neuropathology, Charite Universitatsmedizin Berlin, corporate member of Freie Universitat Berlin, Humboldt-Universitat zu Berlin, and Berlin Institute of Health.

Molecular groups of supratentorial ependymomas comprise tumors with ZFTA-RELA or YAP1-involving fusions and fusion-negative subependymoma. However, occasionally supratentorial ependymomas cannot be readily assigned to any of these groups due to lack of detection of a typical fusion and/or ambiguous DNA methylation-based classification. An unbiased approach with a cohort of unprecedented size revealed distinct methylation clusters composed of tumors with ependymal but also various other histological features containing alternative translocations that shared ZFTA as a partner gene. Read More

View Article and Full-Text PDF

Bevacizumab as a surgery-sparing agent for spinal ependymoma in patients with neurofibromatosis type II: Systematic review and case.

J Clin Neurosci 2021 Apr 1;86:79-84. Epub 2021 Feb 1.

Department of Neurological Surgery, University of Virginia Health System, Charlottesville, VA, USA.

Neurofibromatosis type 2 (NF2) is a rare, hereditary tumor syndrome, often requiring repeated surgeries for multiple lesions with significant cumulative morbidity. As such, non-operative management should be considered when possible for this patient population. The aim of this study is to provide a systematic review of the literature regarding this treatment strategy. Read More

View Article and Full-Text PDF

Mediastinal Anaplastic Ependymoma

J Chest Surg 2021 06 21;54(3):232-234. Epub 2021 Jan 21.

Department of Neurology, Dr. Soetomo Hospital, Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia.

Ependymomas arise from ependymal cells and can grow at any site in the central nervous system (CNS), as well as in some locations outside of the CNS. The latter is rare, contributing to the frequent misdiagnoses of such cases. Herein, we present the case of a 54-year-old man with a history of lower limb weakness and numbness. Read More

View Article and Full-Text PDF

Surgical and Radiologic Prognostic Factors in Intramedullary Spinal Cord Lesions.

World Neurosurg 2021 Jun 19;150:e550-e560. Epub 2021 Mar 19.

Department of Neurosurgery and Gamma Knife Radiosurgery, I.R.C.C.S. San Raffaele Hospital, Milan, Italy. Electronic address:

Objective: The present study aimed to perform a comprehensive data analysis of 47 consecutive patients treated in 8 years and to observe how clinical, radiologic, and surgical factors affect early and long-term outcomes, recurrence rate, and survival.

Methods: Clinical, radiologic, and surgical data were collected retrospectively from the review of a prospectively collected database. The neurologic disability was evaluated according to the modified Rankin Scale (mRS). Read More

View Article and Full-Text PDF

ZFTA-translocations constitute ependymoma chromatin remodeling and transcription factors.

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

View Article and Full-Text PDF

ZFTA-RELA Dictates Oncogenic Transcriptional Programs to Drive Aggressive Supratentorial Ependymoma.

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

View Article and Full-Text PDF

Outcomes after first relapse of childhood intracranial ependymoma.

Pediatr Blood Cancer 2021 Feb 9:e28930. Epub 2021 Feb 9.

Dana-Farber/Boston Children's Cancer and Blood Disorder Center, Boston, Massachusetts, USA.

Background: Ependymoma is the third most common malignant CNS tumor in children. Despite multimodal therapy, prognosis of relapsed ependymoma remains poor. Approaches to therapy for relapsed ependymoma are varied. Read More

View Article and Full-Text PDF
February 2021

A Paravermal Trans-Cerebellar Approach to the Posterior Fossa Tumor Causes Hypertrophic Olivary Degeneration by Dentate Nucleus Injury.

Cancers (Basel) 2021 Jan 12;13(2). Epub 2021 Jan 12.

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

In brain tumor surgery, injury to cerebellar connectivity pathways can induce a neurodegenerative disease called hypertrophic olivary degeneration (HOD), along with a disabling clinical syndrome. In children, cerebellar mutism syndrome (CMS) is another consequence of damage to cerebello-thalamo-cortical networks. The goal of this study was to compare paravermal trans-cerebellar to other more midline or lateral operative approaches in their risk of causing HOD on MR-imaging and CMS. Read More

View Article and Full-Text PDF
January 2021

Canadian Pediatric Neuro-Oncology Standards of Practice.

Front Oncol 2020 22;10:593192. Epub 2020 Dec 22.

Division of Pediatric Hematology/Oncology, IWK Health Centre, Halifax, NS, Canada.

Primary CNS tumors are the leading cause of cancer-related death in pediatrics. It is essential to understand treatment trends to interpret national survival data. In Canada, children with CNS tumors are treated at one of 16 tertiary care centers. Read More

View Article and Full-Text PDF
December 2020

Disease course of Neurofibromatosis Type 2; a 30-year follow-up study of 353 patients seen at a single institution.

Neuro Oncol 2020 Dec 18. Epub 2020 Dec 18.

Manchester Centre for Genomic Medicine, Manchester Academic Health Science Centre, Division of Evolution and Genomic Medicine, University of Manchester, St Mary's Hospital, Manchester Universities NHS Foundation Trust, Manchester, UK.

Background: Limited data exists on the disease course of Neurofibromatosis Type 2 (NF2) to guide clinical trial design.

Methods: A prospective database of patients meeting NF2 diagnostic criteria, reviewed between 1990-2020, was evaluated. Follow-up to first vestibular schwannoma (VS) intervention and death was assessed by univariate analysis and stratified by age at onset, era referred and inheritance type. Read More

View Article and Full-Text PDF
December 2020

Older age is a protective factor for academic achievements irrespective of treatment modalities for posterior fossa brain tumours in children.

PLoS One 2020 16;15(12):e0243998. Epub 2020 Dec 16.

Department of Radiology, Charles University, 2nd Faculty of Medicine and University Hospital Motol, Prague, Czech Republic.

The treatment of children with posterior fossa brain tumours (PFBT) impacts their long term functional and imaging outcomes. This study aimed to evaluate academic achievement correlated with long-term sequelae after different PFBT treatment modalities. The study cohort consisted of 110 survivors (median age at diagnosis 10. Read More

View Article and Full-Text PDF
February 2021

Integrated Proteogenomic Characterization across Major Histological Types of Pediatric Brain Cancer.

Cell 2020 12 25;183(7):1962-1985.e31. Epub 2020 Nov 25.

Department of Pathology, University of Michigan, Ann Arbor, MI 48109, USA.

We report a comprehensive proteogenomics analysis, including whole-genome sequencing, RNA sequencing, and proteomics and phosphoproteomics profiling, of 218 tumors across 7 histological types of childhood brain cancer: low-grade glioma (n = 93), ependymoma (32), high-grade glioma (25), medulloblastoma (22), ganglioglioma (18), craniopharyngioma (16), and atypical teratoid rhabdoid tumor (12). Proteomics data identify common biological themes that span histological boundaries, suggesting that treatments used for one histological type may be applied effectively to other tumors sharing similar proteomics features. Immune landscape characterization reveals diverse tumor microenvironments across and within diagnoses. Read More

View Article and Full-Text PDF
December 2020

Tumors in the cauda equina: A SEER analysis of tumor types and predictors of outcome.

J Clin Neurosci 2020 Nov 14;81:227-233. Epub 2020 Oct 14.

Neurological Surgery, Kentucky Spinal Cord Research Center; University of Louisville School of Medicine, Louisville, KY, USA.

Caudaequinatumors are histologically diverse. International Classification of Diseases for Oncology (ICD-O3) confers dedicated site code (C72. 1) for cauda equina. Read More

View Article and Full-Text PDF
November 2020

Long-term outcomes of spinal ependymomas: an institutional experience of more than 60 cases.

J Neurooncol 2021 Jan 11;151(2):241-247. Epub 2020 Nov 11.

Department of Radiation Oncology, Northwestern Lou and Jean Malnati Brain Tumor Institute, Northwestern University Robert H. Lurie Comprehensive Cancer Center, 676 N. St Clair Street, Suite 1820, Chicago, IL, 60611, USA.

Purpose: Spinal ependymomas represent the most common primary intramedullary tumors for which optimal management remains undefined. When possible, gross total resection (GTR) is often the mainstay of treatment, with consideration of radiotherapy (RT) in cases of residual or recurrent tumor. The impact of extent of resection and radiotherapy remain understudied. Read More

View Article and Full-Text PDF
January 2021

HGF/MET Signaling in Malignant Brain Tumors.

Int J Mol Sci 2020 Oct 13;21(20). Epub 2020 Oct 13.

Department of Microbiology, Immunology & Cancer Biology, University of Virginia, Charlottesville, VA 22908, USA.

Hepatocyte growth factor (HGF) ligand and its receptor tyrosine kinase (RTK) mesenchymal-epithelial transition factor (MET) are important regulators of cellular processes such as proliferation, motility, angiogenesis, and tissue regeneration. In healthy adult somatic cells, this ligand and receptor pair is expressed at low levels and has little activity except when tissue injuries arise. In cancer cells, HGF/MET are often overexpressed, and this overexpression is found to correlate with tumorigenesis, metastasis, and poorer overall prognosis. Read More

View Article and Full-Text PDF
October 2020

Treatment trends and overall survival in patients with grade II/III ependymoma: The role of tumor grade and location.

Clin Neurol Neurosurg 2020 12 6;199:106282. Epub 2020 Oct 6.

Vivian L. Smith Department of Neurosurgery, The University of Texas Health Science Center at Houston, Houston, TX, USA; Memorial Hermann Hospital-Texas Medical Center, Houston, TX, USA; Center for Precision Health, School of Biomedical Informatics, The University of Texas Health Science Center at Houston, Houston, TX, USA. Electronic address:

Background: Treatment of ependymoma (EPN) is guided by associated tumor features, such as grade and location. However, the relationship between these features with treatments and overall survival in EPN patients remains uncharacterized. Here, we describe the change over time in treatment strategies and identify tumor characteristics that influence treatment and survival in EPN. Read More

View Article and Full-Text PDF
December 2020

Ependymoma, NOS and anaplastic ependymoma incidence and survival in the United States varies widely by patient and clinical characteristics, 2000-2016.

Neurooncol Pract 2020 Oct 9;7(5):549-558. Epub 2020 May 9.

Department of Population and Quantitative Health Sciences, Case Western Reserve University School of Medicine, Cleveland, Ohio.

Background: Ependymoma is a rare CNS tumor arising from the ependymal lining of the ventricular system. General differences in incidence and survival have been noted but not examined on a comprehensive scale for all ages and by histology. Despite the rarity of ependymomas, morbidity/mortality associated with an ependymoma diagnosis justifies closer examination. Read More

View Article and Full-Text PDF
October 2020

Deep Learning for Pediatric Posterior Fossa Tumor Detection and Classification: A Multi-Institutional Study.

AJNR Am J Neuroradiol 2020 09 13;41(9):1718-1725. Epub 2020 Aug 13.

Department of Radiology (W.B., J.S., M.P.L., K.W.Y.)

Background And Purpose: Posterior fossa tumors are the most common pediatric brain tumors. MR imaging is key to tumor detection, diagnosis, and therapy guidance. We sought to develop an MR imaging-based deep learning model for posterior fossa tumor detection and tumor pathology classification. Read More

View Article and Full-Text PDF
September 2020

Molecular Advances and Targeted Therapies for Pediatric Central Nervous System Tumors.

J Child Neurol 2021 01 18;36(1):5-29. Epub 2020 Aug 18.

Division of Child Neurology and Developmental Medicine, Department of Pediatrics, Goryeb Children's Hospital, 3368Atlantic Health System, Morristown, NJ, USA.

Central nervous system tumors are extremely rare in the pediatric population and molecularly heterogeneous. Growing scientific research and clinical practice experience are improving medical therapies to increase survival outcomes and quality of life and reduce side effects. The 2019 Neurobiology of Disease in Children Symposium, held in conjunction with the 48th annual meeting of the Child Neurology Society, aimed to (1) describe molecular advances in tumor classification, (2) better understand the evolution of targeted therapies, and (3) more clearly formulate a treatment plan for patients. Read More

View Article and Full-Text PDF
January 2021

Available Therapies for Patients with Neurofibromatosis-Related Nervous System Tumors.

Roy E Strowd

Curr Treat Options Oncol 2020 08 7;21(10):81. Epub 2020 Aug 7.

Department of Neurology, Internal Medicine, Section on Hematology and Oncology, and the Translational Sciences Institute, Wake Forest School of Medicine, 1 Medical Center Boulevard, Winston Salem, NC, 27157, USA.

Opinion Statement: Though the majority of nervous system tumors are sporadic, several clinically relevant genetic syndromes are associated with a predisposition to tumors of the central and peripheral nervous system including neurofibromatosis type 1 (NF1), type 2 (NF2), and schwannomatosis (SWN). These represent prototypical tumor suppressor syndromes where loss of a tumor suppressor gene-protein impairs the cell's ability to regulate cell proliferation. While clinical manifestations vary widely for each of these syndromes, tumors arising in the peripheral nerve sheath are a unifying feature. Read More

View Article and Full-Text PDF

Rare Primary Central Nervous System Tumors in Adults: An Overview.

Front Oncol 2020 26;10:996. Epub 2020 Jun 26.

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, Paris, France.

Overall, tumors of primary central nervous system (CNS) are quite common in adults with an incidence rate close to 30 new cases/100,000 inhabitants per year. Significant clinical and biological advances have been accomplished in the most common adult primary CNS tumors (i.e. Read More

View Article and Full-Text PDF

Single-Cell RNA-Seq Reveals Cellular Hierarchies and Impaired Developmental Trajectories in Pediatric Ependymoma.

Cancer Cell 2020 07;38(1):44-59.e9

Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA.

Ependymoma is a heterogeneous entity of central nervous system tumors with well-established molecular groups. Here, we apply single-cell RNA sequencing to analyze ependymomas across molecular groups and anatomic locations to investigate their intratumoral heterogeneity and developmental origins. Ependymomas are composed of a cellular hierarchy initiating from undifferentiated populations, which undergo impaired differentiation toward three lineages of neuronal-glial fate specification. Read More

View Article and Full-Text PDF

Automatic Machine Learning to Differentiate Pediatric Posterior Fossa Tumors on Routine MR Imaging.

AJNR Am J Neuroradiol 2020 07;41(7):1279-1285

From the School of Computer Science and Engineering (R.H., B.Z., C.Z.)

Background And Purpose: Differentiating the types of pediatric posterior fossa tumors on routine imaging may help in preoperative evaluation and guide surgical resection planning. However, qualitative radiologic MR imaging review has limited performance. This study aimed to compare different machine learning approaches to classify pediatric posterior fossa tumors on routine MR imaging. Read More

View Article and Full-Text PDF