scholarly journals GENE-05. THE LOSS OF SUCCINATE DEHYDROGENASE B EXPRESSION IS FREQUENTLY IDENTIFIED IN HEMANGIOBLASTOMA OF THE CENTRAL NERVOUS SYSTEM

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi98-vi98
Author(s):  
Se-Hyuk Kim ◽  
Tae Hoon Roh ◽  
Hyunee Yim ◽  
Jin Roh ◽  
Kyi Beom Lee ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Succinate Dehydrogenase ◽  
Alternative Pathway ◽  
Krebs Cycle ◽  
High Sensitivity ◽  
Cns Tumors ◽  
Transport Chain ◽  
Assembly Factor ◽  
The Central Nervous System

Abstract Succinate dehydrogenase (SDH) is a mitochondrial enzyme that plays an important role in both the Krebs cycle and the electron transport chain. SDH inactivation is associated with tumorigenesis in certain types of tumor. SDH consists of subunits A, B, C and D (SDHA, SDHB, SDHC, and SDHD, respectively). Immunohistochemistry for SDHB is a reliable method for detecting the inactivation of SDH by mutations in SDHA, SDHB, SDHC, SDHD and SDH complex assembly factor 2 (SDHAF2) genes with high sensitivity and specificity. SDHB immunohistochemistry has been used to examine the inactivation of SDH in various types of tumors. However, data on central nervous system (CNS) tumors are very limited. In the present study, we investigated the loss of SDHB immunoexpression in 90 cases of CNS tumors. Among the 90 cases of CNS tumors, only three cases of hemangioblastoma showed loss of SDHB immunoexpression. We further investigated SDHB immunoexpression in 35 cases of hemangioblastoma and found that 28 (80%) showed either negative or weak-diffuse pattern of SDHB immunoexpression, which suggests the inactivation of SDH. Our results suggest that SDH inactivation may represent an alternative pathway in the tumorigenesis of hemangioblastoma.

scholarly journals Co-Deregulated miRNA Signatures in Childhood Central Nervous System Tumors: In Search for Common Tumor miRNA-Related Mechanics

Cancers ◽  
2021 ◽  
Vol 13 (12) ◽  
pp. 3028
Author(s):  
George I. Lambrou ◽  
Apostolos Zaravinos ◽  
Maria Braoudaki
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cns Tumors ◽  
Normal Brain ◽  
Cns Tumor ◽  
Different Types ◽  
Receiver Operator Curve Analysis ◽  
The Central Nervous System ◽  
Tumor Types ◽  
Operator Curve

Despite extensive experimentation on pediatric tumors of the central nervous system (CNS), related to both prognosis, diagnosis and treatment, the understanding of pathogenesis and etiology of the disease remains scarce. MicroRNAs are known to be involved in CNS tumor oncogenesis. We hypothesized that CNS tumors possess commonly deregulated miRNAs across different CNS tumor types. Aim: The current study aims to reveal the co-deregulated miRNAs across different types of pediatric CNS tumors. Materials: A total of 439 CNS tumor samples were collected from both in-house microarray experiments as well as data available in public databases. Diagnoses included medulloblastoma, astrocytoma, ependydoma, cortical dysplasia, glioblastoma, ATRT, germinoma, teratoma, yoc sac tumors, ocular tumors and retinoblastoma. Results: We found miRNAs that were globally up- or down-regulated in the majority of the CNS tumor samples. MiR-376B and miR-372 were co-upregulated, whereas miR-149, miR-214, miR-574, miR-595 and miR-765 among others, were co-downregulated across all CNS tumors. Receiver-operator curve analysis showed that miR-149, miR-214, miR-574, miR-595 and miR765 could distinguish between CNS tumors and normal brain tissue. Conclusions: Our approach could prove significant in the search for global miRNA targets for tumor diagnosis and therapy. To the best of our knowledge, there are no previous reports concerning the present approach.

scholarly journals Epidemiologic Study of Central Nervous System Tumors in Rwanda

2020 ◽  
pp. 1-14
Author(s):  
David Hakizimana ◽  
Agabe Emmy Nkusi ◽  
David Hakizimana ◽  
Eric Shingiro ◽  
Paulin Munyemana ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Brain Tumors ◽  
Age Groups ◽  
Epidemiologic Study ◽  
Cns Tumors ◽  
Central Nervous System Tumors ◽  
Secondary Neoplasms ◽  
Nervous System Tumors ◽  
The Central Nervous System

Introduction: Tumors of the central nervous system (CNS) are primary or secondary neoplasms located within the craniovertebral cavity. The incidence of CNS tumors is not uniform with variation between different countries, age groups and races. Objective: Our study aim was to generate new knowledge of the epidemiology of central nervous system tumors in Rwanda. Method: This was an observational retrospective study of all patients diagnosed with CNS tumors in Rwanda over a period of 10 years, from 1st January 2006 to 31st December 2015. Results: 466 patients enrolled, (52.2% females, 47.8% males). The median age at diagnosis of was 37 years. Brain tumors were 82.7%; spine tumor patients were 16.4%. The average annual age-standardized incidence of CNS tumors was 0.43/100, 0000 person-years and varied with age groups. Tumors of meningothelial cells represented the majority of brain tumors (31.8%). Metastatic tumors were the far most common spine tumors category. 55.8 % of CNS tumors reported in our study were histologically confirmed and of nonmalignant meningiomas were the commonest (33.9%). Conclusion: This is the very first study done on epidemiology of CNS tumors in Rwanda, and generated data about incidence of CNS tumors in Rwanda and their location and histological distribution.

A CORRELATION OF CLINICOPATHOLOGICAL AND RADIOLOGICAL FINDINGS OF CENTRAL NERVES SYSTEM TUMOURS

2021 ◽  
pp. 17-20
Author(s):  
Monika hemin Shroff ◽  
Abhishek Rajawat ◽  
Kavita choudhary ◽  
Geeta w. Mukhiya
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Posterior Fossa ◽  
Annual Incidence ◽  
Cerebral Hemispheres ◽  
Cns Tumors ◽  
Intracranial Tumors ◽  
Radiological Findings ◽  
The Central Nervous System

The annual incidence of tumors of the central nervous system ranges from 10 to 17 per 100,000 persons for intracranial tumors and 1 to 2 per 100,000 persons for intraspinal tumors. Tumors of the CNS account for nearly 20% of all cancers of childhood. Seventy percent of childhood CNS tumors arise in the posterior fossa; a comparable number of tumors in adults arise within the cerebral hemispheres above the tentorium

scholarly journals A study on morphologic and histological pattern of the central nervous system tumors

2018 ◽  
Vol 6 (12) ◽  
pp. 3879
Author(s):  
Ajay Singh Thakur ◽  
Renuka Gahine ◽  
Vishal Kulkarni
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Who Classification ◽  
Epidemiologic Studies ◽  
Cns Tumors ◽  
International Agency ◽  
Metastatic Tumors ◽  
Medical College ◽  
Oligodendroglial Tumors ◽  
The Central Nervous System

Background: It has been revealed by International Agency for Research on Cancer that the worldwide incidence rate of CNS tumors in 2002 was 3.7/100,000 population among males and 2.6/100,000 population among females. Central Nervous System (CNS) tumors account for 85% of brain tumors and 15 % of spinal cord tumors, however metastatic tumors are usually extradural. According to the WHO classification tumors of CNS comprise more than 50 clinico-pathological entities. The major categories include the tumors of neuroepithelial tissue, cranial and paraspinal nerves, meninges, sellar region, lymphomas haematopoietic neoplasms, germ cell and metastatic tumors. An understanding of the epidemiology is needed to facilitate prevention, early detection and treatment of CNS tumours.Methods: The study was conducted in the Department of Pathology, Pt. J.N.M. Medical College Raipur, India from January 2009 to August 2017. The present study was a retrospective study, hence H and E section of every case was re-examined and histopathological reports were reviewed. Sections were stained with H&E. Diagnosis is made according to the WHO classification and diagnostic criteria for CNS neoplasms.Results: CNS tumours occur more frequently in the age group of 41-50 years (57 cases -21.1%) followed by 31-40 years (53 cases -19.7%). The most common entity in the present study were tumours of neuroepithelial tissue 127 cases (47.2%) followed by tumors of meninges 59 cases (22%) and nerve sheath tumours 42 cases (15.6%). The tumors of neuroepithelial tissue comprised mainly of astrocytic tumors 86 cases (32%) followed by embryonal tumors 15 cases (5.6%), ependymal tumors 11 cases (4.1%), oligodendroglial tumors 09 cases (3.3%).Conclusions: Within the scope and limitations of the present study we believe that this effort would help in establishing the grounds for future epidemiologic studies that would, eventually, contribute to give insight into the epidemiology of CNS tumors.

scholarly journals Targeted Deletion of the PEX2 Peroxisome Assembly Gene in Mice Provides a Model for Zellweger Syndrome, a Human Neuronal Migration Disorder

1997 ◽  
Vol 139 (5) ◽  
pp. 1293-1305 ◽  
Author(s):  
Phyllis L. Faust ◽  
Mary E. Hatten
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neuronal Migration ◽  
Zellweger Syndrome ◽  
Peroxisomal Biogenesis ◽  
Migration Disorder ◽  
Assembly Factor ◽  
The Central Nervous System ◽  
Deficient Mice ◽  
Peroxisomal Function

Zellweger syndrome is a peroxisomal biogenesis disorder that results in abnormal neuronal migration in the central nervous system and severe neurologic dysfunction. The pathogenesis of the multiple severe anomalies associated with the disorders of peroxisome biogenesis remains unknown. To study the relationship between lack of peroxisomal function and organ dysfunction, the PEX2 peroxisome assembly gene (formerly peroxisome assembly factor-1) was disrupted by gene targeting. Homozygous PEX2-deficient mice survive in utero but die several hours after birth. The mutant animals do not feed and are hypoactive and markedly hypotonic. The PEX2-deficient mice lack normal peroxisomes but do assemble empty peroxisome membrane ghosts. They display abnormal peroxisomal biochemical parameters, including accumulations of very long chain fatty acids in plasma and deficient erythrocyte plasmalogens. Abnormal lipid storage is evident in the adrenal cortex, with characteristic lamellar–lipid inclusions. In the central nervous system of newborn mutant mice there is disordered lamination in the cerebral cortex and an increased cell density in the underlying white matter, indicating an abnormality of neuronal migration. These findings demonstrate that mice with a PEX2 gene deletion have a peroxisomal disorder and provide an important model to study the role of peroxisomal function in the pathogenesis of this human disease.

scholarly journals Histopathological pattern of central nervous system tumor: A three year retrospective study

1970 ◽  
Vol 1 (1) ◽  
pp. 22-25 ◽  
Author(s):  
G Aryal
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Metastatic Tumor ◽  
University Hospital ◽  
Cns Tumors ◽  
World Health ◽  
Central Nervous System Tumor ◽  
Medical College ◽  
Frequent Type ◽  
The Central Nervous System

Background: There is no documented data regarding the histopathological spectrum of the central nervous system (CNS) tumors in Nepal. The aim of this study was to establish the relative frequency of biopsy proven tumors of the CNS from a single university hospital in Nepal. Materials and Methods: In the period between1998-2000, we retrospectively analyzed data on 57 patients (28 male and 29 female) diagnosed with CNS tumors according to the World Health Organization’s diagnostic criteria. Patient data were retrieved from the archives of the department of Pathology, Nepal Medical College. Results: A total of 57 CNS tumors were diagnosed during a three year period. Of these, 49 (86%) were primary, and 8 (14%) were metastatic. The most frequent type of CNS tumors was astrocytoma (22 cases, 38.6%), followed by meningioma (8 cases, 14%) and metastatic tumor (8 cases, 14%). Among the 8 metastatic tumors, the most common histologic type was adenocarcinoma (7 cases, 87.5%). Conclusion: The most frequent type of CNS tumors in this study was astrocytoma, followed by meningioma and metastatic tumor. The ratio of male to female for all CNS tumors was 0.9:1. Female outnumbered male in meningioma (1:0.3). Keywords: Astrocytoma; Meningioma; Tumor; Nepal; Central nervous system   DOI: 10.3126/jpn.v1i1.4445 Journal of Pathology of Nepal (2011) Vol.1, 22-25

scholarly journals RETROSPECTIVE ANALYSIS OF INTRACRANIAL AND INTRASPINAL SPACE OCCUPYING LESIONS AT A TERTIARY CARE CENTER- A FIVE YEAR STUDY

2018 ◽  
Vol 6 (10) ◽  
pp. 100-109
Author(s):  
Meena B. Patil ◽  
Manjiri N. Karandikar
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Who Classification ◽  
Frozen Section ◽  
Tertiary Care ◽  
Cns Tumors ◽  
Low Grade ◽  
Wide Range ◽  
The Central Nervous System

Background: Tumors of Central Nervous System constitute approximately 2% of all malignancies. Cancers of the central nervous system (CNS) are considered to be among the most notorious of all cancers. Low-grade cns tumors have been found over time to progress to high grade tumors. CNS is a common site for metastasis from other organs. Due to newer techniques used in radio diagnosis and stereotactic biopsies, more & more CNS tumors are being diagnosed & are being operated upon. Aims and objectives: The objective of this article is to provide an overview of intracranial and intraspinal space occupying lesions at a single tertiary care referral center. The aim was to study incidence of various lesions in light of the   WHO classification of tumours of the central nervous system, 2016 and to study relevant statistics. Material and methods: A total of 124 cases were received between January,2007 & December, 2011 at Bharati Vidyapeeth Deemed University Medical College and Hospital, Pune. Intraoperative diagnosis was desired in 70 cases. The final diagnoses in all the cases were made on Hematoxylin and Eosin stained slides of routinely processed tissue. Results: Neoplastic lesions comprised 83.88 %, including metastatic tumors while 16.12 % were nonneoplastic.  A wide range of histopathological spectrum of CNS tumors was observed and was classified according to WHO classification of CNS tumors, 2016. The primary CNS tumors were graded from Grade I to IV according to WHO grading system, 2016. Astrocytic tumors constituted the largest category with 33 cases and most of the astrocytomas were grade II at the time of diagnosis. Incidence was more in females than males and maximum number of lesions were seen in fifth and sixth decades of life. Conclusion: This study highlights the histological diversity of CNS tumors in both adults and children. The most common destructive but nonneoplastic lesions like, infections/abscesses, infarct and cysts can be diagnosed, by crush cytology and frozen section for the definitive management.

EPID-26. ALIGNING THE CBTRUS HISTOLOGY GROUPS WITH 2016 WHO CLASSIFICATION OF TUMORS OF THE CENTRAL NERVOUS SYSTEM

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi91-vi91
Author(s):  
Carol Kruchko ◽  
Nirav Patil ◽  
Gino Cioffi ◽  
Daniel Brat ◽  
Janet Bruner ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Who Classification ◽  
The United States ◽  
Cns Tumors ◽  
World Health ◽  
Mesenchymal Tumors ◽  
The Central Nervous System ◽  
Health Organization

Abstract BACKGROUND The Central Brain Tumor Registry of the United States (CBTRUS) uses a histology grouping scheme modeled after the World Health Organization (WHO) Classification of Tumours of the Central Nervous System (CNS) to classify cancer registry records for clinically relevant statistical reporting. Molecular studies have identified genetic features which precisely stratify tumor types, resulting in the 2016 update to the WHO Classification incorporating these markers. To continue providing clinically relevant statistics, the histology groupings have been aligned with the 2016 update. Resulting changes to groupings were assessed. METHODS In collaboration with four consulting neuropathologists the scheme was reviewed and realigned to the 2016 update. Obsolete histology nomenclature and ICD-O-3 codes were identified. Evaluation of the frequency of affected codes in the 2013-2017 data was conducted. RESULTS 417,767 total cases of primary brain and CNS tumors were diagnosed during 2013-2017 in the US. After review of the CBTRUS grouping scheme, 67 codes were noted to be obsolete, 51 codes were re-classified and 12 new codes were incorporated. This reorganization could result in grouping assignment or reporting changes for 2,588 cases (0.6%). The histology groups most significantly affected were mesenchymal tumors and neuronal and mixed neuronal glial tumors. CONCLUSIONS The 2016 revision to WHO Classification has affected collection and reporting of CNS tumors. The CBTRUS data edits program is now undergoing revision, which will become the basis of reporting. Some histology-specific molecular markers require additional data to distinguish between cases. In collaboration with CBTRUS, the NAACCR SSDI Committee developed a new variable for collection of molecular information. This variable was included in Uniform Data Standards beginning on January 1, 2018 and will be available for reporting in 2021. A 2021 update to the WHO Classification is scheduled for release later this year, requiring further classification updates.

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