8,551 results match your criteria: "Ependymoma"

Utility of OLIG2 immunostaining in pediatric brain tumors with embryonal morphology.

J Neuropathol Exp Neurol

December 2024

Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, CA, United States.

This study evaluates the diagnostic utility of OLIG2 immunohistochemistry for distinguishing between pediatric high-grade gliomas (pHGG) and embryonal tumors (ETs) of the CNS. Utilizing a retrospective pediatric cohort (1990-2021) of 56 CNS tumors, classified initially as primitive neuroectodermal tumors or CNS ET, we reclassified the cases based on WHO CNS5 criteria after comprehensive review and additional molecular testing that included next-generation sequencing and DNA methylation profiling. Our results indicate that OLIG2 immunopositivity was negative or minimal in a significant subset of pHGG cases (6 out of 11).

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: Differentiating pediatric posterior fossa (PF) tumors such as medulloblastoma (MB), ependymoma (EP), and pilocytic astrocytoma (PA) remains relevant, because of important treatment and prognostic implications. Diffusion kurtosis imaging (DKI) has not yet been investigated for discrimination of pediatric PF tumors. Estimating diffusion values from whole-tumor-based (VOI) segmentations may improve diffusion measurement repeatability compared to conventional region-of-interest (ROI) approaches.

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Pediatric intramedullary spinal cord tumors: A national cancer database analysis of demographics, patterns of care, and survival.

Clin Neurol Neurosurg

September 2024

Department of Neurosurgery, Mount Sinai Health System, New York, NY, United States. Electronic address:

Objective: Query the National Cancer Database (NCDB) to delineate epidemiologic frequency, care patterns, and survival outcomes of pediatric intramedullary spinal cord tumors (IMSCTs).

Methods: IMSCTs included ependymoma, astrocytoma, and hemangioblastoma. We examined data from the NCDB spanning 2004-2018, focusing on IMSCT in children aged 0-21 years.

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Purpose: This retrospective systematic literature review aimed to summarize available data regarding epidemiology, etiology, presentation, investigations, differentials, treatment, prevention, monitoring, complications, and prognosis for radiation-induced cavernous malformations (RICMs) in pediatric patients.

Methodology: Review conducted per PRISMA guidelines. Google Scholar, PubMed, Trip Medical Database, and Cochrane Library searched utilizing a keyphrase, articles filtered per inclusion/exclusion criteria, duplicates excluded.

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Objectives: Diagnosing brain tumors is critical due to their complex nature. This review explores the potential of hybridization for diagnosing brain neoplasms, examining their attributes and applications in neurology and oncology.

Methods: The review surveys literature and cross-references findings with the OMIM database, examining 513 records.

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Introduction: Radiation-induced gliomas (RIGs) were reported in the literature in general. In most of the reported cases and the reviewed articles, patients have a history of primary intracranial tumors like craniopharyngioma, medulloblastoma, and ependymoma, and the commonly resulting secondary tumors are meningiomas and sarcomas, mainly not gliomas. .

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Objective: We aim to report the epidemiology, surgical outcomes, and survival rates of pediatric patients with posterior fossa tumors in a large single-center case series.

Methods: A retrospective analysis was conducted on pediatric patients who underwent surgical treatment for posterior fossa tumors between January 2011 and January 2019.

Results: A total of 135 pediatric patients, with an average age of 7.

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Ependymal and choroid plexus tumours arise in anatomically related regions. Their intraoperative differential diagnosis is large and depends on factors such as age, tumour site and clinical presentation. Squash cytology can provide valuable information in this context.

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TULIPs decorate the three-dimensional genome of PFA ependymoma.

Cell

September 2024

Arnie Charbonneau Cancer Institute, Cumming School of Medicine, University of Calgary, Calgary, AB T2N 4N1, Canada; Alberta Children's Hospital Research Institute, Cumming School of Medicine, University of Calgary, Calgary, AB T2N 4N1, Canada; Department of Biochemistry and Molecular Biology, Cumming School of Medicine, University of Calgary, Calgary, AB T2N 4N1, Canada; Department of Pediatrics, Baylor College of Medicine, Houston, TX 77030, USA; Cancer and Hematology Center, Texas Children's Hospital, Houston, TX 77030, USA; Dan L Duncan Comprehensive Cancer Center, Baylor College of Medicine, Houston, TX 77030, USA. Electronic address:

Posterior fossa group A (PFA) ependymoma is a lethal brain cancer diagnosed in infants and young children. The lack of driver events in the PFA linear genome led us to search its 3D genome for characteristic features. Here, we reconstructed 3D genomes from diverse childhood tumor types and uncovered a global topology in PFA that is highly reminiscent of stem and progenitor cells in a variety of human tissues.

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Article Synopsis
  • * Analyzed data from 43 patients showed that IOUS effectively confirmed tumor locations, aided in minimizing neural injury, and helped identify hidden lesions, leading to better surgical outcomes.
  • * The findings suggest that IOUS is a beneficial tool in spinal surgeries, as it improves the accuracy of tumor removal and reduces the risk of complications.
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Background: Patients affected by Von Hippel-Lindau (VHL) are prone to develop central nervous system neoplasms such as hemangioblastomas (HBs). Myxopapillary ependymoma (MPE) is not commonly associated with VHL disease.

Case Description: We present the first case of a VHL patient affected by simultaneous silent cauda equina MPE and a symptomatic conus medullaris HB.

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Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy can present with or without systemic disease. It is a benign histioproliferative disorder characterized by generalized lymphadenopathy, weakness, anemia, and rarely extranodal involvement. While RDD most commonly affects lymph nodes, extranodal involvement of multiple organs has been reported, including the central nervous system (CNS).

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The molecular biology of NF2/Merlin on tumorigenesis and development.

FASEB J

July 2024

College of Life Science and Medicine, Zhejiang Provincial Key Laboratory of Silkworm Bioreactor and Biomedicine, Zhejiang Sci-Tech University, Hangzhou, China.

Article Synopsis
  • The NF2 gene, which encodes the tumor suppressor protein Merlin, plays a crucial role in understanding tumor formation and various cellular processes.
  • The review highlights Merlin's structural features, functional diversity, and its interaction with key signaling pathways that regulate cellular growth and differentiation, linking NF2 mutations to tumors like schwannomas and meningiomas.
  • It also addresses the severe impact of NF2 deficiency on embryonic development and discusses potential treatments targeting genetic abnormalities, stressing the need for further research to improve outcomes for those affected by NF2 mutations.
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Article Synopsis
  • The study investigates the imaging characteristics of supratentorial ependymomas (STE), rare tumors primarily found in children, using CT and MRI scans from 25 patients.
  • Key findings suggest that STEs often occur near ventricles and are associated with cystic components and specific imaging signs like the periwinkle sign.
  • The research also highlights significant links between these imaging features and the presence of a particular genetic alteration (ZFTA fusion), aiming to improve diagnosis and treatment outcomes.
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Predicting response to chemotherapy in brain tumor patients based on MRI features.

Clin Neurol Neurosurg

September 2024

Department of Neurosurgery, Section of Surgery, Aga Khan University Hospital, Karachi, Pakistan. Electronic address:

Chemotherapy in brain tumors is tailored based on tumor type, grade, and molecular markers, which are crucial for predicting responses and survival outcomes. This review summarizes the role of chemotherapy in gliomas, glioneuronal and neuronal tumors, ependymomas, choroid plexus tumors, medulloblastomas, and meningiomas, discussing standard treatment protocols and recent developments in targeted therapies.Furthermore, the studies reporting the integration of MRI-based radiomics and deep learning models for predicting treatment outcomes are reviewed.

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Article Synopsis
  • * Researchers analyzed data from 5,211 patients diagnosed with malignant and borderline malignant tumors between 2000 and 2019, focusing on demographic and clinical characteristics, as well as survival statistics.
  • * Findings revealed that ependymoma was the most common type of tumor, with incidence rates varying by age, sex, and ethnicity, and highlighted that males had a higher incidence than females, particularly among Caucasians.
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Article Synopsis
  • * The disease is linked to mutations in the NF2 gene, which disrupts the function of the Merlin protein, a crucial tumor suppressor that influences cell behavior, leading to tumor growth.
  • * The review highlights potential therapeutic options, including molecular therapies that target signaling pathways or proteins involved in tumorigenesis, aiming to improve treatment approaches for NF2 syndrome.
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Surgery for spinal cord tumors poses a significant challenge due to the inherent risk of neurological deterioration. Despite being performed at numerous centers, there is an ongoing debate regarding the efficacy of pre- and intraoperative neurophysiological investigations in detecting and preventing neurological lesions. This study begins by providing a comprehensive review of the neurophysiological techniques commonly employed in this context.

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Background And Purpose: Hemangioblastoma is a rare vascular tumor that occurs within the central nervous system in children. Differentiating hemangioblastoma from other posterior fossa tumors can be challenging on imaging, and preoperative diagnosis can change the neurosurgical approach. We hypothesize that a "lightbulb sign" on the arterial spin-labeling (ASL) sequence (diffuse homogeneous intense hyperperfusion within the solid component of the tumor) will provide additional imaging finding to differentiate hemangioblastoma from other posterior fossa tumors.

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Background And Aims: Ependymomas exhibit heterogeneity across age, location, histology, molecular nature and survival suggestive of an epigenetic component in its pathogenesis. The CNS WHO classification (2021) classifies ependymomas based on DNA methylation profiles. Studies suggest that molecular sub-types remain stable throughout the course of disease.

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Brigatinib in -Related Schwannomatosis with Progressive Tumors.

N Engl J Med

June 2024

From Massachusetts General Hospital and Harvard Medical School (S.R.P., V.L.M.) and the Dana-Farber Cancer Institute (G.F., L.T.) - all in Boston; the NYU Grossman School of Medicine (K.H.Y.) and the Children's Tumor Foundation (A.B.) - both in New York; the University of California, Los Angeles, Los Angeles (P.L.N.); the University of Miami Miller School of Medicine, Sylvester Comprehensive Cancer Center, Miami (C.T.D.); the Mayo Clinic, Rochester, MN (D.B.-V.); and Johns Hopkins University, Baltimore (J.O.B.).

Article Synopsis
  • Background: -related schwannomatosis (formerly neurofibromatosis type 2) is a progressive tumor syndrome characterized by various tumors including vestibular and nonvestibular schwannomas, meningiomas, and ependymomas, with no approved treatments available.
  • Methods: A phase 2 trial was conducted involving patients aged 12 and older with -SWN, treating them daily with 180 mg of oral brigatinib, and evaluating tumor response and safety through a central review committee.
  • Results: Out of 40 patients, radiographic response rates were 10% for target tumors and 23% for all tumors, with improvements notably in meningiomas
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Purpose: Pediatric spinal cord gliomas (PSGs) are rare in children and few reports detail their imaging features. We tested the association of tumoral grade with imaging features and proposed a novel approach to categorize post-contrast enhancement patterns in PSGs.

Methods: This single-center, retrospective study included patients <21 years of age with preoperative spinal MRI and confirmed pathological diagnosis of PSG from 2000-2022.

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Core-shell particles composed of polycaprolactone/polyvinyl alcohol (PCL/PVA) with pH sensitive properties were successfully fabricated by co-axial electrospraying in which PVA and PCL formed the shell and core layers respectively. The core-shell structure was confirmed by FTIR, DSC and SEM analysis. No chemical interaction between PVA and PCL core-shell were observed in the FTIR analysis.

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Article Synopsis
  • NF2-related schwannomatosis is a genetic disorder marked by the development of multiple tumors, including schwannomas and meningiomas, with evolving diagnostic criteria since the late 1980s.
  • The criteria have undergone several revisions, with notable updates in 2011 enhancing sensitivity for patients lacking typical tumors, and significant changes made by the Manchester group in 2019, including the introduction of molecular criteria.
  • The 2022 updates by an international committee not only refined diagnostic criteria but also redefined "schwannomatosis" as an overarching term for conditions leading to schwannomas and classified them based on genetic causes.
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