426 results match your criteria Ependymoma* Neurology


Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours.

Nat Rev Neurol 2022 Jun 21. Epub 2022 Jun 21.

Center For Neuro-Oncology, Dana-Farber Cancer Institute and Department of Neurology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

A new edition of the WHO classification of tumours of the CNS was published in 2021. Although the previous edition of this classification was published just 5 years earlier, in 2016, rapid advances in our understanding of the molecular underpinnings of CNS tumours, including the diversity of clinically relevant molecular types and subtypes, necessitated a new classification system. Compared with the 2016 scheme, the new classification incorporates even more molecular alterations into the diagnosis of many tumours and reorganizes gliomas into adult-type diffuse gliomas, paediatric-type diffuse low-grade and high-grade gliomas, circumscribed astrocytic gliomas, and ependymal tumours. Read More

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Rare central nervous system tumors in adults: a population-based study of ependymomas, pilocytic astrocytomas, medulloblastomas, and intracranial germ cell tumors.

Neurooncol Adv 2022 Jan-Dec;4(1):vdac062. Epub 2022 Apr 22.

Department of Neurology/Brain Tumor Center, Erasmus MC Cancer Institute, Rotterdam, The Netherlands.

Background: Ependymomas, pilocytic astrocytomas, medulloblastomas, and intracranial germ cell tumors occur relative frequently in children, but are rare central nervous system (CNS) tumors in adults. In this population-based survey, we established incidence, treatment, and survival patterns for these tumors diagnosed in adult patients (≥18 years) over a 30-year period (1989-2018).

Methods: Data on 1384 ependymomas, 454 pilocytic astrocytomas, 205 medulloblastomas, and 112 intracranial germ cell tumors were obtained from the Netherlands Cancer Registry (NCR) on the basis of a histopathological diagnosis. Read More

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Activation of Hedgehog signaling by the oncogenic RELA fusion reveals a primary cilia-dependent vulnerability in Supratentorial Ependymoma.

Neuro Oncol 2022 May 31. Epub 2022 May 31.

Department of Pediatrics, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, SP, 14049-900, Brazil.

Background: Supratentorial RELA fusion (ST-RELA) ependymomas (EPNs) are resistant tumors without an approved chemotherapeutic treatment. Unfortunately, the molecular mechanisms that leads to chemoresistance traits of ST-RELA remains elusive. The aim of this study was to assess RELA fusion-dependent signaling modules, specifically the role of the Hedgehog (Hh) pathway as a novel targetable vulnerability in ST-RELA. Read More

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Multifocal and Multiphasic Demyelinating Lesions After Radiation for Ependymoma in a Pediatric Population.

J Child Neurol 2022 Jun 26;37(7):609-616. Epub 2022 May 26.

Department of Pediatrics, 5447Nicklaus Children's Hospital, Miami, FL, USA.

Radiation treatment is widely used to address unresectable intracranial tumors. Owing to the nature of therapy, healthy tissue and diseased regions will be affected. New insights have shown that not only does this impact brain parenchyma but it causes changes in fluid status, myelination, and the integrity of the blood-brain barrier. Read More

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Primary Spinal Tumors and Masses in Children.

Iran J Child Neurol 2022 14;16(2):129-135. Epub 2022 Mar 14.

Pediatric Neurology Research Center, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Objectives: Spinal cord tumors are rare in children, mostly presented with unspecific symptoms that might pose a problem due to their possible malignancy and further complications. However, there are limited data on spinal cord lesions in Iran. This study aimed to present a series of 37 cases of primary spinal tumors treated at the same institution and briefly review their pathology, symptoms, and site of occurrence. Read More

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Resection of Ependymomas Infiltrating the Fourth Ventricular Floor: Anatomosurgical and Stimulation Mapping Techniques.

Oper Neurosurg (Hagerstown) 2022 05;22(5):e189-e197

UCL Queen Square Institute of Neurology, University College London, London, UK.

Background: Despite the importance of complete, gross total resection (GTR) of fourth ventricular ependymomas, significant morbidity and/or subtotal resections are reported, particularly when the ventricular floor is infiltrated. Step-by-step technique descriptions are lacking in the literature.

Objective: To describe monitoring and stimulation mapping techniques and surgical nuances in the challenging subgroup of infiltrating fourth ventricular ependymomas by a highly illustrated, step-by-step description. Read More

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Superficial Siderosis Due to Spinal Myxopapillary Ependymoma Mimicking Idiopathic Intracranial Hypertension.

Ann Indian Acad Neurol 2022 Jan-Feb;25(1):156-157. Epub 2021 Oct 11.

Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.

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October 2021

Pediatric ependymoma: New perspectives on older trials.

Neuro Oncol 2022 Jun;24(6):949-950

Division of Pediatric Neurology, Department of Neurology, Seattle Children's Hospital, University of Washington, Seattle, Washington, USA.

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Radiomics-A new age of presurgical assessment to improve outcomes in pediatric neuro-oncology.

Neuro Oncol 2022 Jun;24(6):995-996

Michael Rice Cancer Centre, Women's and Children's Hospital, North Adelaide, South Australia, Australia.

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Next-generation Sequencing of Cerebrospinal Fluid for Clinical Molecular Diagnostics in Pediatric, Adolescent and Young Adult (AYA) Brain Tumor Patients.

Neuro Oncol 2022 Feb 11. Epub 2022 Feb 11.

Department of Pediatrics, Memorial Sloan Kettering Cancer Center.

Background: Safe sampling of CNS tumor tissue for diagnostic purposes may be difficult if not impossible, especially in pediatric patients, and an unmet need exists to develop less invasive diagnostic tests.

Methods: We report our clinical experience with minimally invasive molecular diagnostics using a clinically validated assay for sequencing of cerebrospinal fluid (CSF) cell-free DNA (cfDNA). All CSF samples were collected as part of clinical care, and results reported to both clinicians and patients/families. Read More

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February 2022

Sarcomatous Change of Cerebellopontine Angle Ependymoma Following Radiosurgery: A Case Report.

Cureus 2022 Jan 1;14(1):e20864. Epub 2022 Jan 1.

Neurological Surgery, Lenox Hill Hospital/Donald and Barbara Zucker School of Medicine at Hofstra, New York, USA.

Sarcomatous change in ependymal tumors is rare and has been poorly described. We report on a cerebellopontine angle lesion that demonstrated rapid progression two years after radiosurgery in a 73-year-old female patient. Histopathological diagnosis at clinical progression showed an ependymoma with sarcomatous change ("ependymosarcoma") that was believed to be due to radiation. Read More

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January 2022

Diffusion tensor imaging in unclear intramedullary tumor-suspected lesions allows separating tumors from inflammation.

Spinal Cord 2021 Dec 30. Epub 2021 Dec 30.

Department of Neuroradiology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Design: Prospective diagnostic study.

Objectives: Primary imaging-based diagnosis of spinal cord tumor-suspected lesions is often challenging. The identification of the definite entity is crucial for dedicated treatment and therefore reduction of morbidity. Read More

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December 2021

An Overview of Intracranial Ependymomas in Adults.

Cancers (Basel) 2021 Dec 5;13(23). Epub 2021 Dec 5.

Lyon University, Université Claude Bernard Lyon 1, 69007 Lyon, France.

Ependymomas are rare primary central nervous system tumors. They can form anywhere along the neuraxis, but in adults, these tumors predominantly occur in the spine and less frequently intracranially. Ependymal tumors represent a heterogenous group of gliomas, and the WHO 2016 classification is based essentially on a grading system, with ependymomas classified as grade I, II (classic), or III (anaplastic). Read More

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December 2021

Radiomic signatures of posterior fossa ependymoma: Molecular subgroups and risk profiles.

Neuro Oncol 2022 Jun;24(6):986-994

Department of Radiology, Lucile Packard Children's Hospital, Stanford, California, USA.

Background: The risk profile for posterior fossa ependymoma (EP) depends on surgical and molecular status [Group A (PFA) versus Group B (PFB)]. While subtotal tumor resection is known to confer worse prognosis, MRI-based EP risk-profiling is unexplored. We aimed to apply machine learning strategies to link MRI-based biomarkers of high-risk EP and also to distinguish PFA from PFB. Read More

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Neurofibromatoses.

Hematol Oncol Clin North Am 2022 02 27;36(1):253-267. Epub 2021 Oct 27.

Pappas Center for Neuro-Oncology and Family Center for Neurofibromatosis, Massachusetts General Hospital, Department of Neurology, 55 Fruit Street, Yawkey 9E, Boston, MA 02114, USA.

The neurofibromatoses are a group of genetic disorders that cause development of nervous system tumors as well as various other tumor and systemic manifestations. Neurofibromatosis type 1 is the most prevalent of these conditions and has the most variable phenotype and highest risk of malignant tumor formation. Neurofibromatosis type 2 has no associated malignant tumors but does carry significant morbidity, including deafness, facial weakness, and physical disability. Read More

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February 2022

Primary Spinal Cord Glioblastoma.

Cureus 2021 Oct 3;13(10):e18464. Epub 2021 Oct 3.

Department of Radioneurosurgery, Institute of Neurology and Neurosurgery, Ciudad de México, MEX.

We present the case of a 29-year-old patient whose pain began with the interscapular region, progressing to paresthesia and loss of muscle strength in the lower extremities. MRI of the spine was done, a lesion was found in T2 to T6, ependymoma was suspected and was taken to subtotal resection with laminectomy, the histopathological report, as well as the immunohistochemistry, was compatible with glioblastoma type not otherwise specified (NOS). He received adjuvant with radiotherapy and concomitant chemotherapy, but he progressed to the cervical and lumbar spine, the patient died 16 months after diagnosis. Read More

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October 2021

Intramedullary spinal cord tumors: A retrospective multicentric study.

J Craniovertebr Junction Spine 2021 Jul-Sep;12(3):269-278. Epub 2021 Sep 8.

Department of Neurosurgery, Tunisia Faculty of Medicine, National Institute of Neurology Tunis, University of Tunis El Manar, Tunis, Tunisia.

Context: Intramedullary tumors are neoformations taking part on the spinal cord, and they are a rare pathology. Due to the rarity of such lesions, clinical studies take years to ensure a decent feedback with a significant number of cases.

Design: Our study is retrospective and descriptive. Read More

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September 2021

Diagnostic, therapeutic, and prognostic implications of the 2021 World Health Organization classification of tumors of the central nervous system.

Cancer 2022 01 11;128(1):47-58. Epub 2021 Oct 11.

Cancer Center, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

The 2016 revised fourth edition of the World Health Organization (WHO) classification of central nervous system (CNS) tumors incorporated molecular features with histologic grading, revolutionizing how oncologists conceptualize primary brain and spinal cord tumors as well as providing new insights into their management and prognosis. The 2021 revised fifth edition of the WHO classification further integrates molecular alterations for CNS tumor categorization, updating current understanding of the pathophysiology of many of these disease entities. Here, the authors review changes in the new classification for the most common primary adult tumors-gliomas (including astrocytomas, oligodendrogliomas, and ependymomas) and meningiomas-highlighting the key genomic alterations for each group classification to help clinicians interpret them as they consider therapeutic options-including clinical trials and targeted therapies-and discuss the prognosis of these tumors with their patients. Read More

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January 2022

Gliomas in children and adolescents: investigation of molecular alterations with a potential prognostic and therapeutic impact.

J Cancer Res Clin Oncol 2022 Jan 9;148(1):107-119. Epub 2021 Oct 9.

Department of Pediatrics, Pediatric Oncology Institute-GRAACC/UNIFESP, Federal University of Sao Paulo, 743 Botucatu Street, 8th Floor - Genetics Laboratory, Vila Clementino, Sao Paulo, SP, 04023-062, Brazil.

Purpose: Gliomas represent the most frequent central nervous system (CNS) tumors in children and adolescents. However, therapeutic strategies for these patients, based on tumor molecular profile, are still limited compared to the wide range of treatment options for the adult population. We investigated molecular alterations, with a potential prognostic marker and therapeutic target in gliomas of childhood and adolescence using the next-generation sequencing (NGS) strategy. Read More

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January 2022

Treatment and outcome of intracranial ependymoma after first relapse in the 2nd AIEOP protocol.

Neuro Oncol 2022 03;24(3):467-479

Department of Neurology and Psychiatry, La Sapienza University, Rome, Italy.

Background: More than 40% of patients with intracranial ependymoma need a salvage treatment within 5 years after diagnosis, and no standard treatment is available as yet. We report the outcome after first relapse of 64 patients treated within the 2nd AIEOP protocol.

Methods: We considered relapse sites and treatments, that is, various combinations of complete/incomplete surgery, if followed by standard or hypofractionated radiotherapy (RT) ± chemotherapy (CT). Read More

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Molecular profiling of pediatric and adolescent ependymomas: identification of genetic variants using a next-generation sequencing panel.

J Neurooncol 2021 Oct 27;155(1):13-23. Epub 2021 Sep 27.

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

Purpose: Ependymoma (EPN) accounts for approximately 10% of all primary central nervous system (CNS) tumors in children and in most cases, chemotherapy is ineffective and treatment remains challenging. We investigated molecular alterations, with a potential prognostic marker and therapeutic target in EPNs of childhood and adolescence, using a next-generation sequencing (NGS) panel specific for pediatric neoplasms.

Methods: We selected 61 samples with initial diagnosis of EPN from patients treated at Pediatric Oncology Institute-GRAACC/UNIFESP. Read More

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October 2021

Nuclear IMPDH Filaments in Human Gliomas.

J Neuropathol Exp Neurol 2021 10;80(10):944-954

Center for Cancer Therapeutics and Neurosciences, Ottawa Hospital Research Institute and Department of Pathology and Laboratory Medicine, University of Ottawa, Ottawa, Ontario, Canada.

The analysis of nuclear morphology plays an important role in glioma diagnosis and grading. We previously described intranuclear rods (rods) labeled with the SDL.3D10 monoclonal antibody against class III beta-tubulin (TUBB3) in human ependymomas. Read More

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October 2021

Freiburg Neuropathology Case Conference : A 6-year-old Girl Presenting with Vomiting and Right-sided Facial Paresis.

Clin Neuroradiol 2021 09 1;31(3):885-892. Epub 2021 Sep 1.

Department of Neuroradiology, Medical Centre-University of Freiburg, Breisacherstraße 64, 79106, Freiburg, Germany.

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September 2021

Machine Assist for Pediatric Posterior Fossa Tumor Diagnosis: A Multinational Study.

Neurosurgery 2021 10;89(5):892-900

Department of Neurosurgery, Great Ormond Street Hospital, London, United Kingdom.

Background: Clinicians and machine classifiers reliably diagnose pilocytic astrocytoma (PA) on magnetic resonance imaging (MRI) but less accurately distinguish medulloblastoma (MB) from ependymoma (EP). One strategy is to first rule out the most identifiable diagnosis.

Objective: To hypothesize a sequential machine-learning classifier could improve diagnostic performance by mimicking a clinician's strategy of excluding PA before distinguishing MB from EP. Read More

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October 2021

Recurrent fusions in PLAGL1 define a distinct subset of pediatric-type supratentorial neuroepithelial tumors.

Acta Neuropathol 2021 11 5;142(5):827-839. Epub 2021 Aug 5.

Institute of Neuropathology, University of Giessen, Giessen, Germany.

Ependymomas encompass a heterogeneous group of central nervous system (CNS) neoplasms that occur along the entire neuroaxis. In recent years, extensive (epi-)genomic profiling efforts have identified several molecular groups of ependymoma that are characterized by distinct molecular alterations and/or patterns. Based on unsupervised visualization of a large cohort of genome-wide DNA methylation data, we identified a highly distinct group of pediatric-type tumors (n = 40) forming a cluster separate from all established CNS tumor types, of which a high proportion were histopathologically diagnosed as ependymoma. Read More

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November 2021

Chemotherapy for adult patients with spinal cord gliomas.

Neurooncol Pract 2021 Aug 8;8(4):475-484. Epub 2021 Mar 8.

Department of Neurology, Clinical Neuroscience Center, University Hospital and University of Zurich, Zurich, Switzerland.

Background: The incidence of spinal cord gliomas, particularly in adults is low, and the role of chemotherapy has remained unclear.

Methods: We performed a multicenter, retrospective study of 21 patients diagnosed with spinal cord glioma who received chemotherapy at any time during the disease course. Benefit from chemotherapy was estimated by magnetic resonance imaging. Read More

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Surgical techniques in the management of supratentorial pediatric brain tumors: 10 years' experience at a tertiary care center in the Middle East.

Surg Neurol Int 2021 7;12:269. Epub 2021 Jun 7.

Department of Surgery-Neurosurgery, American University of Beirut, United States.

Background: The goal of this retrospective study is to present the first epidemiological data on pediatric supratentorial central nervous system (CNS) tumors in Lebanon and to review the various surgical management strategies used.

Methods: We conducted a retrospective case series of all pediatric patients who presented with a supratentorial CNS tumor and underwent surgery at our institution between 2006 and 2016. We collected and analyzed demographic characteristics, tumor location, clinical manifestations, histopathology, and surgical management strategies and outcome, and discussed them after dividing the tumors as per location and in view of published literature. Read More

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Pediatric Neuro-Oncology.

Authors:
Fatema Malbari

Neurol Clin 2021 08 10;39(3):829-845. Epub 2021 Jun 10.

Department of Pediatrics, Division of Pediatric Neurology and Developmental Neurosciences, Texas Children's Hospital, Baylor College of Medicine, 6701 Fannin Street, Suite 1250, Houston, TX 77030, USA. Electronic address:

Central nervous system (CNS) tumors are the most common solid tumor in pediatrics and represent the largest cause of childhood cancer-related mortality. With advances in molecular characterization of tumors, considerable developments have occurred impacting diagnosis and management, and refined prognostication. Advances in management have led to better survival, but mortality remains high and significant morbidity persists. Read More

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Ependymoma Presenting as a -Rim-Enhancing Lesion in the Brainstem.

Pediatr Neurosurg 2021 18;56(5):455-459. Epub 2021 Jun 18.

Department of Pediatrics, Texas Children's Cancer Center, Baylor College of Medicine, Houston, Texas, USA.

Introduction: The posterior fossa is the most common intracranial location for pediatric ependymoma. While ependymoma usually arises from the ventricular lining of the fourth ventricle as a solid mass, it rarely originates from the brainstem. Grade II ependymomas also infrequently appear as a cavitary ring-enhancing lesion. Read More

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October 2021