5,466 results match your criteria Primitive Neuroectodermal Tumors of the Central Nervous System


Role of Microbiota-Derived Extracellular Vesicles in Gut-Brain Communication.

Int J Mol Sci 2021 Apr 19;22(8). Epub 2021 Apr 19.

Department of Molecular and Cellular Pathology of Alcohol, Príncipe Felipe Research Center, 46012 Valencia, Spain.

Human intestinal microbiota comprise of a dynamic population of bacterial species and other microorganisms with the capacity to interact with the rest of the organism and strongly influence the host during homeostasis and disease. Commensal and pathogenic bacteria coexist in homeostasis with the intestinal epithelium and the gastrointestinal tract's immune system, or GALT (gut-associated lymphoid tissue), of the host. However, a disruption to this homeostasis or dysbiosis by different factors (e. Read More

View Article and Full-Text PDF

Clinical-MRI radiomics enables the prediction of preoperative cerebral spinal fluid dissemination in children with medulloblastoma.

World J Surg Oncol 2021 Apr 22;19(1):134. Epub 2021 Apr 22.

Department of Radiology, Xinhua Hospital affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.

Background: Medulloblastoma (MB) is the most common pediatric embryonal tumor. Accurate identification of cerebral spinal fluid (CSF) dissemination is important in prognosis prediction. Both MRI of the central nervous system (CNS) and CSF cytology will appear false positive and negative. Read More

View Article and Full-Text PDF

SWI/SNF deficient central nervous system neoplasms.

Authors:
Chunyu Cai

Semin Diagn Pathol 2021 May 15;38(3):167-174. Epub 2021 Mar 15.

Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, United States. Electronic address:

The SWItch/Sucrose Non-Fermentable (SWI/SNF) complexes are ubiquitous ATP dependent chromatin remodeling complexes that provide epigenetic regulation of gene expressions across the genome. Different combination of SWI/SNF subunits allow tissue specific regulation of critical cellular processes. The identification of SMARCB1 inactivation in pediatric malignant rhabdoid tumors provided the first example that the SWI/SNF complex may act as a tumor suppressor. Read More

View Article and Full-Text PDF

The transcriptional landscape of Shh medulloblastoma.

Nat Commun 2021 03 19;12(1):1749. Epub 2021 Mar 19.

Developmental & Stem Cell Biology Program, The Hospital for Sick Children, Toronto, ON, Canada.

Sonic hedgehog medulloblastoma encompasses a clinically and molecularly diverse group of cancers of the developing central nervous system. Here, we use unbiased sequencing of the transcriptome across a large cohort of 250 tumors to reveal differences among molecular subtypes of the disease, and demonstrate the previously unappreciated importance of non-coding RNA transcripts. We identify alterations within the cAMP dependent pathway (GNAS, PRKAR1A) which converge on GLI2 activity and show that 18% of tumors have a genetic event that directly targets the abundance and/or stability of MYCN. Read More

View Article and Full-Text PDF

Not all mouse blood-brain barriers are created equal.

Neuro Oncol 2021 05;23(5):705-706

Department of Neurological Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.

View Article and Full-Text PDF

Copper Dependent Modulation of α-Synuclein Phosphorylation in Differentiated SHSY5Y Neuroblastoma Cells.

Int J Mol Sci 2021 Feb 18;22(4). Epub 2021 Feb 18.

Department of Biological and Environmental Science and Technology, University of Salento, 73100 Lecce, Italy.

Copper (Cu) dyshomeostasis plays a pivotal role in several neuropathologies, such as Parkinson's disease (PD). Metal accumulation in the central nervous system (CNS) could result in loss-of-function of proteins involved in Cu metabolism and redox cycling, generating reactive oxygen species (ROS). Moreover, neurodegenerative disorders imply the presence of an excess of misfolded proteins known to lead to neuronal damage. Read More

View Article and Full-Text PDF
February 2021

Prevalence and risk factors for cisplatin-induced hearing loss in children, adolescents, and young adults: a multi-institutional North American cohort study.

Lancet Child Adolesc Health 2021 04 12;5(4):274-283. Epub 2021 Feb 12.

Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, CA, USA; Department of Pediatrics, University of Southern California, Los Angeles, CA, USA. Electronic address:

Background: Cisplatin is used to treat a wide range of childhood cancers and cisplatin-induced hearing loss (CIHL) is a common and debilitating toxicity. We aimed to address persistent knowledge gaps in CIHL by establishing benchmarks for the prevalence of and risk factors for CIHL.

Methods: In this multi-institutional cohort study, children (age 0-14 years), adolescents, and young adults (age 15-39 years) diagnosed with a cisplatin-treated tumour from paediatric cancer centres, who had available cisplatin dosing information, and primary audiology data for central review from consortia located in Canada and the USA were eligible for inclusion. Read More

View Article and Full-Text PDF

Molecular analysis of pediatric CNS-PNET revealed nosologic heterogeneity and potent diagnostic markers for CNS neuroblastoma with FOXR2-activation.

Acta Neuropathol Commun 2021 02 3;9(1):20. Epub 2021 Feb 3.

Clinical Cooperation Unit Neuropathology (G380), German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120, Heidelberg, Germany.

Primitive neuroectodermal tumors of the central nervous system (CNS-PNETs) are highly malignant neoplasms posing diagnostic challenge due to a lack of defining molecular markers. CNS neuroblastoma with forkhead box R2 (FOXR2) activation (CNS_NBL) emerged as a distinct pediatric brain tumor entity from a pool previously diagnosed as primitive neuroectodermal tumors of the central nervous system (CNS-PNETs). Current standard of identifying CNS_NBL relies on molecular analysis. Read More

View Article and Full-Text PDF
February 2021

Meningeal Relapse of Neuroblastoma-Utility of 68Ga-DOTATOC PET/CT for Detection.

Clin Nucl Med 2021 06;46(6):e339-e341

Pathology, Mahatma Gandhi Cancer Hospital and Research Institute, Visakhapatnam, Andhra Pradesh, India.

Abstract: In treatment of metastatic neuroblastoma, the central nervous system seems to act as a sanctuary leading to often unexpected relapse of disease. We present a case of neuroblastoma treated with chemotherapy and autologous stem cell transplantation only to relapse in central nervous system with meningeal metastases visualized on 68Ga-DOTATOC imaging. Read More

View Article and Full-Text PDF

Congenital primary primitive neuroectodermal tumor of the orbit in a newborn.

Eur J Ophthalmol 2021 Jan 26:1120672121991047. Epub 2021 Jan 26.

Department of Pediatrics, Jawaharlal Postgraduate Medical Education and Research, Puducherry, India.

Introduction: Primitive neuroectodermal tumors arise from the progenitor cells of the neural crest, in the central nervous system or other peripheral locations.

Case Presentation: We report a rare case of a congenital malignant tumor, diagnosed as a primary orbital primitive neuroectodermal tumor on histopathological examination.

Conclusion: Multidisciplinary management with adjuvant chemotherapy needed for the management of these cases. Read More

View Article and Full-Text PDF
January 2021

Induction of Brain Tumors by the Archetype Strain of Human Neurotropic JCPyV in a Transgenic Mouse Model.

Viruses 2021 01 22;13(2). Epub 2021 Jan 22.

Department of Neurosciences and Center for Neurovirology, Lewis Katz School of Medicine at Temple University, Philadelphia, PA 19140, USA.

JC Virus (JCPyV), a member of the family, is a human neurotropic virus with world-wide distribution. JCPyV is the established opportunistic infectious agent of progressive multifocal leukoencephalopathy, a fatal demyelinating disease, which results from the cytolytic infection of oligodendrocytes. Mutations in the regulatory region of JCPyV determine the different viral strains. Read More

View Article and Full-Text PDF
January 2021

Primary Primitive Neuroectodermal Tumor (PNET) of the Spine With t(11;22): Report of 3 Cases and Review of Literature.

J Pediatr Hematol Oncol 2021 Jan 20. Epub 2021 Jan 20.

Division of Pediatric Oncology, Children's Hospital of Orange County, Orange Division of Hematology/Oncology and Bone Marrow Transplantation, Children's Hospital of Los Angeles, Los Angeles, CA.

Intradural extramedullary peripheral primitive neuroectodermal tumor (pPNET) with t(11;22) is a rare clinical finding in the pediatric population with few published cases in the literature. The authors report 3 cases of intradural primary pPNET and discuss the clinical presentation, treatment, and survival of the patients. Clinicians should be vigilant in considering pPNET in the differential diagnosis of extradural masses. Read More

View Article and Full-Text PDF
January 2021

Glioblastoma with a primitive neuroectodermal component: two cases with implications for glioblastoma cell-of-origin.

Clin Imaging 2021 May 4;73:139-145. Epub 2020 Dec 4.

Department of Radiology, University of Florida College of Medicine, Gainesville, FL, United States. Electronic address:

Background: Glioblastoma (GBM) is the most common primary brain malignancy, but much remains unknown about the histogenesis of these tumors. In the great majority of cases, GBM is a purely glial tumor but in rare cases the classic-appearing high-grade glioma component is admixed with regions of small round blue cells with neuronal immunophenotype, and these tumors have been defined in the WHO 2016 Classification as "glioblastoma with a primitive neuronal component."

Methods: In this paper, we present two cases of GBM-PNC with highly divergent clinical courses, and review current theories for the GBM cell-of-origin. Read More

View Article and Full-Text PDF

Ovarian tissue cryopreservation and transplantation in patients with central nervous system tumours.

Hum Reprod 2021 04;36(5):1296-1309

Pôle de Gynécologie, Institut de Recherche Expérimentale et Clinique (IREC), Université Catholique de Louvain, Brussels, Belgium.

Study Question: Is there a possibility of reseeding cancer cells potentially present in frozen ovarian tissue from patients with central nervous system (CNS) tumours?

Summary Answer: Malignancy reseeding in cryopreserved ovarian tissue from 20 patients with CNS tumours was not detected by histology, immunohistochemistry (IHC), molecular biology or xenotransplantation.

What Is Known Already: Ovarian metastasis potential has been documented in patients with leukaemia, borderline ovarian tumours, advanced breast cancer and Ewing sarcoma. However, data on the safety of transplanting frozen-thawed ovarian tissue from cancer patients with CNS tumours are still lacking. Read More

View Article and Full-Text PDF

Delta-24-RGD, an Oncolytic Adenovirus, Increases Survival and Promotes Proinflammatory Immune Landscape Remodeling in Models of AT/RT and CNS-PNET.

Clin Cancer Res 2021 Mar 29;27(6):1807-1820. Epub 2020 Dec 29.

Health Research Institute of Navarra (IdiSNA), Pamplona, Navarra, Spain.

Purpose: Atypical teratoid/rhabdoid tumors (AT/RT) and central nervous system primitive neuroectodermal tumors (CNS-PNET) are pediatric brain tumors with poor survival and life-long negative side effects. Here, the aim was to characterize the efficacy and safety of the oncolytic adenovirus, Delta-24-RGD, which selectively replicates in and kills tumor cells.

Experimental Design: Delta-24-RGD determinants for infection and replication were evaluated in patient expression datasets. Read More

View Article and Full-Text PDF

Is Ovarian Tissue Transplantation Safe in Patients with Central Nervous System Primitive Neuroectodermal Tumors?

J Clin Med 2020 Dec 18;9(12). Epub 2020 Dec 18.

Gynecology Research Unit, Institut de Recherche Expérimentale et Clinique, Université Catholique de Louvain, Av. Mounier 52, 1200 Brussels, Belgium.

The risk of reseeding malignancy harbored in cryopreserved and transplanted ovarian tissue has been a source of concern. This study aimed to determine the potential relationship between frozen-thawed ovarian tissue transplantation and primary cancer recurrence. Three patients with cerebral primitive neuroectodermal tumors (PNET) were included in this study. Read More

View Article and Full-Text PDF
December 2020

An audit of brain tumor patients treated in 5 years at a single institute: Our regional cancer center experience.

J Cancer Res Ther 2020 Oct-Dec;16(6):1466-1469

Department of Radiotherapy, Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India.

Introduction: Brain tumors constitute a small presentation of all cancers (1.4%) and cancer related deaths (2.5%). Read More

View Article and Full-Text PDF
December 2020

18F-FDG PET/CT for Staging and Response Evaluation in a Rare Case of Childhood Ovarian Primitive Neuroectodermal Tumor.

Clin Nucl Med 2021 May;46(5):e266-e267

Pathology and Laboratory Services, Apollo Gleneagles Hospital, Kolkata, India.

Abstract: Primitive neuroectodermal tumor (PNET) is an aggressive small cell malignancy. Apart from the central nervous system, they can arise from peripheral sites as well. Ovarian PNETs are extremely rare neoplasms. Read More

View Article and Full-Text PDF

Validation of human microRNA target pathways enables evaluation of target prediction tools.

Nucleic Acids Res 2021 01;49(1):127-144

Institute of Human Genetics, Saarland University, 66421 Homburg, Germany.

MicroRNAs are regulators of gene expression. A wide-spread, yet not validated, assumption is that the targetome of miRNAs is non-randomly distributed across the transcriptome and that targets share functional pathways. We developed a computational and experimental strategy termed high-throughput miRNA interaction reporter assay (HiTmIR) to facilitate the validation of target pathways. Read More

View Article and Full-Text PDF
January 2021

Immunophenotypic Characterization of Germ Cell Tumor-Derived Primitive Neuroectodermal Tumors: Evidence for Frequent Neuronal and/or Glial Differentiation.

Arch Pathol Lab Med 2020 Dec 8. Epub 2020 Dec 8.

Department of Pathology and Laboratory Medicine, Indiana University, Indianapolis (Ulbright, Idrees).

Context.—: Primitive neuroectodermal tumors (PNETs) may arise as a somatic-type malignancy in germ cell tumors. In this setting, most PNETs resemble those of the central nervous system and lack chromosome 22 translocations. Read More

View Article and Full-Text PDF
December 2020

Detailed Clinical and Histopathological Description of 8 Cases of Molecularly Defined CNS Neuroblastomas.

J Neuropathol Exp Neurol 2021 01;80(1):52-59

Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf.

Central nervous system neuroblastoma with FOXR2 activation (CNS NB FOXR2) has recently been described as a class of brain tumors sharing common genetic events and a highly similar DNA methylation profile. Most of these tumors have previously been diagnosed as primitive neuroectodermal tumor (PNET). Whereas the entity of PNET has been removed from the WHO classification of brain tumors in its current edition, CNS neuroblastoma was kept as an entity, but still lacks any molecular detail. Read More

View Article and Full-Text PDF
January 2021

Activated leukocyte cell adhesion molecule expression correlates with the WNT subgroup in medulloblastoma and is involved in regulating tumor cell proliferation and invasion.

PLoS One 2020 3;15(12):e0243272. Epub 2020 Dec 3.

Department of Neurosurgery, Osaka University Graduate School of Medicine, Osaka, Japan.

Cluster of differentiation (CD) 166 or activated leukocyte cell adhesion molecule (ALCAM) is a transmembrane molecule known to be an intercellular adhesion factor. The expression and function of ALCAM in medulloblastoma (MB), a pediatric brain tumor with highly advanced molecular genetics, remains unclear. Therefore, this study aimed to clarify the significance and functional role of ALCAM expression in MB. Read More

View Article and Full-Text PDF
January 2021

Patient-derived orthotopic xenograft models of medulloblastoma lack a functional blood-brain barrier.

Neuro Oncol 2021 05;23(5):732-742

The University of Queensland Diamantina Institute, Translational Research Institute, The University of Queensland, Woolloongabba, Queensland, Australia.

Background: Novel targeted therapies for children diagnosed with medulloblastoma (MB), the most common malignant pediatric brain tumor, are urgently required. A major hurdle in the development of effective therapies is the impaired delivery of systemic therapies to tumor cells due to a specialized endothelial blood-brain barrier (BBB). Accordingly, the integrity of the BBB is an essential consideration in any preclinical model used for assessing novel therapeutics. Read More

View Article and Full-Text PDF

Histone demethylase UTX/KDM6A enhances tumor immune cell recruitment, promotes differentiation and suppresses medulloblastoma.

Cancer Lett 2021 02 27;499:188-200. Epub 2020 Nov 27.

Department of Physiology, University of Texas Southwestern Medical Center, Dallas, TX, 75390, USA. Electronic address:

The deregulation of epigenetic pathways has been implicated as a critical step in tumorigenesis including in childhood brain tumor medulloblastoma. The H3K27me3 demethylase UTX/KDM6A plays important roles in development and is frequently mutated in various types of cancer. However, how UTX regulates tumor development remains largely unclear. Read More

View Article and Full-Text PDF
February 2021

Glabrescione B delivery by self-assembling micelles efficiently inhibits tumor growth in preclinical models of Hedgehog-dependent medulloblastoma.

Cancer Lett 2021 02 26;499:220-231. Epub 2020 Nov 26.

Department of Molecular Medicine, University La Sapienza, Roma, Italy; Laboratory Affiliated to Istituto Pasteur Italia-Fondazione Cenci Bolognetti, Department of Molecular Medicine, University La Sapienza, Roma, Italy. Electronic address:

Aberrant activation of the Hedgehog (Hh) pathway leads to the development of several tumors, including medulloblastoma (MB), the most common pediatric brain malignancy. Hh inhibitors acting on GLI1, the final effector of Hh signaling, offer a valuable opportunity to overcome the pitfalls of the existing therapies to treat Hh-driven cancers. In this study, the toxicity, delivery, biodistribution, and anticancer efficacy of Glabrescione B (GlaB), a selective GLI1 inhibitor, were investigated in preclinical models of Hh-dependent MB. Read More

View Article and Full-Text PDF
February 2021

Prox1 inhibits neurite outgrowth during central nervous system development.

Cell Mol Life Sci 2021 Apr 28;78(7):3443-3465. Epub 2020 Nov 28.

Center for Basic Research, Biomedical Research Foundation of the Academy of Athens, 4 Soranou Efesiou Street, 115 27, Athens, Greece.

During central nervous system (CNS) development, proper and timely induction of neurite elongation is critical for generating functional, mature neurons, and neuronal networks. Despite the wealth of information on the action of extracellular cues, little is known about the intrinsic gene regulatory factors that control this developmental decision. Here, we report the identification of Prox1, a homeobox transcription factor, as a key player in inhibiting neurite elongation. Read More

View Article and Full-Text PDF

C19MC amplification and expression of Lin28A and Olig2 in the classification of embryonal tumors of the central nervous system: A 14-year retrospective study from a tertiary care center.

Childs Nerv Syst 2021 Apr 24;37(4):1067-1075. Epub 2020 Nov 24.

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

Introduction: CNS embryonal tumors (CET) other than medulloblastomas (MB) and atypical teratoid/rhabdoid tumors (AT/RTs), previously designated as 'central nervous system primitive neuroectodermal tumors' ('CNS PNETs'), are a heterogenous subset of tumors with poorly defined diagnostic criteria. Other than the subset of embryonal tumor with multilayered rosettes (ETMR) defined by C19MC amplification, most CETs are diagnosed by exclusion of other molecularly defined entities and histological mimics including MB, AT/RTs, and high-grade gliomas, and termed as CET, not otherwise specified (NOS) in the 2016 WHO classification.

Aim: To reclassify 'CNS PNETs' as per WHO 2016, and estimate the true proportion of CET, NOS in a tertiary healthcare setting, and to evaluate the diagnostic utility of C19MC amplification, Lin28A and Olig2 expression in the subclassification of CETs. Read More

View Article and Full-Text PDF

Clinical characteristics and treatment outcomes of pigmented tumors in central nervous system: Focusing on melanocytic tumors.

J Clin Neurosci 2020 Nov 29;81:83-89. Epub 2020 Sep 29.

Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, China. Electronic address:

Pigmented tumors are rare neoplasm of central nervous system. Melanocytic tumor, including primary and metastatic lesions, is the most common type. Owing to the rarity, the differential diagnosis of pigmented tumors and clinical management of melanocytic tumor remain challenge. Read More

View Article and Full-Text PDF
November 2020

Malignant Ependymoblastoma Mimicking a Benign Pilocytic Astrocytoma.

Neurol Int 2020 Oct 30;12(3):41-47. Epub 2020 Oct 30.

Department of Radiology, Hanoi Medical University, Ha Noi 100000, Vietnam.

Ependymoblastoma is an uncommon, exceedingly malignant brain neoplasm that adversely influences children's quality of life. Ependymoblastoma represents a subtype of primitive neuroectodermal tumors, categorized as grade IV, according to the 2007 World Health Organization (WHO) classification of central nervous system tumors. Ependymoblastomas are often located in the supratentorial zone and often associated with the ventricular system. Read More

View Article and Full-Text PDF
October 2020

Unsaturated mannuronate oligosaccharide ameliorates β-amyloid pathology through autophagy in Alzheimer's disease cell models.

Carbohydr Polym 2021 Jan 25;251:117124. Epub 2020 Sep 25.

Shenzhen Key Laboratory of Marine Bioresources and Ecology, College of Life Sciences and Oceanography, Shenzhen University, Shenzhen 518060, China. Electronic address:

Unsaturated mannuronate oligosaccharide (MOS) is an enzymatic depolymerization product from alginate-derived polymannuronate (PM). In this study, we investigated for the first time the potential therapeutic effect of MOS on Alzheimer's disease (AD) and its molecular mechanism in N2a-sw cells and 3×Tg-AD primary cortex neurons. Our results showed that MOS ranges from mannuronate dimer to mannuronate undecamer (M2-M11) with an unsaturated nonreducing terminal structure and with a double bond and 1,4-glycosidic linkages. Read More

View Article and Full-Text PDF
January 2021