The 2007 WHO Classification of Tumours of the Central Nervous System
TL;DR: The fourth edition of the World Health Organization (WHO) classification of tumours of the central nervous system, published in 2007, lists several new entities, including angiocentric glioma, papillary glioneuronal tumour, rosette-forming glioneurs tumour of the fourth ventricle, Papillary tumourof the pineal region, pituicytoma and spindle cell oncocytoma of the adenohypophysis.
Abstract: The fourth edition of the World Health Organization (WHO) classification of tumours of the central nervous system, published in 2007, lists several new entities, including angiocentric glioma, papillary glioneuronal tumour, rosette-forming glioneuronal tumour of the fourth ventricle, papillary tumour of the pineal region, pituicytoma and spindle cell oncocytoma of the adenohypophysis. Histological variants were added if there was evidence of a different age distribution, location, genetic profile or clinical behaviour; these included pilomyxoid astrocytoma, anaplastic medulloblastoma and medulloblastoma with extensive nodularity. The WHO grading scheme and the sections on genetic profiles were updated and the rhabdoid tumour predisposition syndrome was added to the list of familial tumour syndromes typically involving the nervous system. As in the previous, 2000 edition of the WHO ‘Blue Book’, the classification is accompanied by a concise commentary on clinico-pathological characteristics of each tumour type. The 2007 WHO classification is based on the consensus of an international Working Group of 25 pathologists and geneticists, as well as contributions from more than 70 international experts overall, and is presented as the standard for the definition of brain tumours to the clinical oncology and cancer research communities world-wide.
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TL;DR: The Central Brain Tumor Registry of the United States (CBTRUS), in collaboration with the Centers for Disease Control and Prevention and National Cancer Institute, is the largest population-based registry focused exclusively on primary brain and other central nervous system (CNS) tumors in the US.
Abstract: The Central Brain Tumor Registry of the United States (CBTRUS), in collaboration with the Centers for Disease Control (CDC) and National Cancer Institute (NCI), is the largest population-based registry focused exclusively on primary brain and other central nervous system (CNS) tumors in the United States (US) and represents the entire US population. This report contains the most up-to-date population-based data on primary brain tumors (malignant and non-malignant) and supersedes all previous CBTRUS reports in terms of completeness and accuracy. All rates (incidence and mortality) are age-adjusted using the 2000 US standard population and presented per 100,000 population. The average annual age-adjusted incidence rate (AAAIR) of all malignant and non-malignant brain and other CNS tumors was 23.79 (Malignant AAAIR=7.08, non-Malignant AAAIR=16.71). This rate was higher in females compared to males (26.31 versus 21.09), Blacks compared to Whites (23.88 versus 23.83), and non-Hispanics compared to Hispanics (24.23 versus 21.48). The most commonly occurring malignant brain and other CNS tumor was glioblastoma (14.5% of all tumors), and the most common non-malignant tumor was meningioma (38.3% of all tumors). Glioblastoma was more common in males, and meningioma was more common in females. In children and adolescents (age 0-19 years), the incidence rate of all primary brain and other CNS tumors was 6.14. An estimated 83,830 new cases of malignant and non-malignant brain and other CNS tumors are expected to be diagnosed in the US in 2020 (24,970 malignant and 58,860 non-malignant). There were 81,246 deaths attributed to malignant brain and other CNS tumors between 2013 and 2017. This represents an average annual mortality rate of 4.42. The 5-year relative survival rate following diagnosis of a malignant brain and other CNS tumor was 23.5% and for a non-malignant brain and other CNS tumor was 82.4%.
9,802 citations
TL;DR: Recurrent mutations in the active site of isocitrate dehydrogenase 1 (IDH1) occurred in a large fraction of young patients and in most patients with secondary GBMs and were associated with an increase in overall survival.
Abstract: Glioblastoma multiforme (GBM) is the most common and lethal type of brain cancer. To identify the genetic alterations in GBMs, we sequenced 20,661 protein coding genes, determined the presence of amplifications and deletions using high-density oligonucleotide arrays, and performed gene expression analyses using next-generation sequencing technologies in 22 human tumor samples. This comprehensive analysis led to the discovery of a variety of genes that were not known to be altered in GBMs. Most notably, we found recurrent mutations in the active site of isocitrate dehydrogenase 1 (IDH1) in 12% of GBM patients. Mutations in IDH1 occurred in a large fraction of young patients and in most patients with secondary GBMs and were associated with an increase in overall survival. These studies demonstrate the value of unbiased genomic analyses in the characterization of human brain cancer and identify a potentially useful genetic alteration for the classification and targeted therapy of GBMs.
5,250 citations
TL;DR: Astrocyte functions in healthy CNS, mechanisms and functions of reactive astrogliosis and glial scar formation, and ways in which reactive astrocytes may cause or contribute to specific CNS disorders and lesions are reviewed.
Abstract: Astrocytes are specialized glial cells that outnumber neurons by over fivefold. They contiguously tile the entire central nervous system (CNS) and exert many essential complex functions in the healthy CNS. Astrocytes respond to all forms of CNS insults through a process referred to as reactive astrogliosis, which has become a pathological hallmark of CNS structural lesions. Substantial progress has been made recently in determining functions and mechanisms of reactive astrogliosis and in identifying roles of astrocytes in CNS disorders and pathologies. A vast molecular arsenal at the disposal of reactive astrocytes is being defined. Transgenic mouse models are dissecting specific aspects of reactive astrocytosis and glial scar formation in vivo. Astrocyte involvement in specific clinicopathological entities is being defined. It is now clear that reactive astrogliosis is not a simple all-or-none phenomenon but is a finely gradated continuum of changes that occur in context-dependent manners regulated by specific signaling events. These changes range from reversible alterations in gene expression and cell hypertrophy with preservation of cellular domains and tissue structure, to long-lasting scar formation with rearrangement of tissue structure. Increasing evidence points towards the potential of reactive astrogliosis to play either primary or contributing roles in CNS disorders via loss of normal astrocyte functions or gain of abnormal effects. This article reviews (1) astrocyte functions in healthy CNS, (2) mechanisms and functions of reactive astrogliosis and glial scar formation, and (3) ways in which reactive astrocytes may cause or contribute to specific CNS disorders and lesions.
4,075 citations
TL;DR: The authors found that approximately 5% of patients with malignant gliomas have a family history of glioma and most of these familial cases are associated with rare genetic syndromes, such as neurofibromatosis types 1 and 2, the Li−Fraumeni syndrome (germ-line p53 mutations associated with an increased risk of several cancers), and Turcot's syndrome (intestinal polyposis and brain tumors).
Abstract: Approximately 5% of patients with malignant gliomas have a family history of gliomas. Some of these familial cases are associated with rare genetic syndromes, such as neurofibromatosis types 1 and 2, the Li−Fraumeni syndrome (germ-line p53 mutations associated with an increased risk of several cancers), and Turcot’s syndrome (intestinal polyposis and brain tumors). 10 However, most familial cases have
3,823 citations
Technische Universität München1, ETH Zurich2, University of Bern3, Harvard University4, National Institutes of Health5, University of Debrecen6, University Hospital Heidelberg7, McGill University8, University of Pennsylvania9, French Institute for Research in Computer Science and Automation10, University at Buffalo11, Microsoft12, University of Cambridge13, Stanford University14, University of Virginia15, Imperial College London16, Massachusetts Institute of Technology17, Columbia University18, Sabancı University19, Old Dominion University20, RMIT University21, Purdue University22, General Electric23
TL;DR: The Multimodal Brain Tumor Image Segmentation Benchmark (BRATS) as mentioned in this paper was organized in conjunction with the MICCAI 2012 and 2013 conferences, and twenty state-of-the-art tumor segmentation algorithms were applied to a set of 65 multi-contrast MR scans of low and high grade glioma patients.
Abstract: In this paper we report the set-up and results of the Multimodal Brain Tumor Image Segmentation Benchmark (BRATS) organized in conjunction with the MICCAI 2012 and 2013 conferences Twenty state-of-the-art tumor segmentation algorithms were applied to a set of 65 multi-contrast MR scans of low- and high-grade glioma patients—manually annotated by up to four raters—and to 65 comparable scans generated using tumor image simulation software Quantitative evaluations revealed considerable disagreement between the human raters in segmenting various tumor sub-regions (Dice scores in the range 74%–85%), illustrating the difficulty of this task We found that different algorithms worked best for different sub-regions (reaching performance comparable to human inter-rater variability), but that no single algorithm ranked in the top for all sub-regions simultaneously Fusing several good algorithms using a hierarchical majority vote yielded segmentations that consistently ranked above all individual algorithms, indicating remaining opportunities for further methodological improvements The BRATS image data and manual annotations continue to be publicly available through an online evaluation system as an ongoing benchmarking resource
3,699 citations
References
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Book•
01 Jan 2007
TL;DR: The current edition of the WHO Classification of Tumours of the Central Nervous System will serve as an indispensable textbook for all of those involved in the diagnosis and management of patients with tumors of the CNS, and will make a valuable addition to libraries in pathology, radiology, oncology, and neurosurgery departments.
Abstract: Representing the first volume in the fourth edition series of the World Health Organization (WHO) Classification of Tumours, this book provides a welcome mix of old and new. Similar to prior versions, it opens with a summary of the recently revised WHO Classification of Tumours of the Central Nervous System (CNS), the remainder of the book being dedicated to a comprehensive yet succinct presentation of the most current knowledge relative to each individual tumor and familial tumor syndrome. The 73 contributing authors have likewise adopted a familiar standardized format with the following subheadings: definition, grading, incidence, age and sex distribution, localization, clinical features, neuroimaging, macroscopy, histopathology, proliferation, genetic susceptibility, genetics, histogenesis, and prognostic and predictive factors. Although a fair number of images have been recycled from previous editions, the majority is new and includes more than 400 full-color photomicrographs and macroscopic images, as well as numerous neuroimages, informative diagrams and charts. A number of tumor entities new to this version of the WHO Classification are explored in detail, including pilomyxoid astrocytoma, atypical choroid plexus papilloma, angiocentric glioma, extraventricular neurocytoma, papillary glioneuronal tumor, rosetteforming glioneuronal tumor of the fourth ventricle, papillary tumor of the pineal region, pituicytoma, and spindle cell oncocytoma of the adenohypophysis. Perhaps the most noticeable improvement comes by way of a voluminous expansion in the genetics sections of the majority of tumor categories. This update parallels the recent explosion of research utilizing high-resolution genome screening and other molecular techniques. The authors have done an outstanding job in distilling the information housed in over 2,500 cited references into a readerfriendly authoritative reference of CNS neoplasia. In summation, the current edition of the WHO Classification of Tumours of the Central Nervous System will serve as an indispensable textbook for all of those involved in the diagnosis and management of patients with tumors of the CNS, and will make a valuable addition to libraries in pathology, radiology, oncology, and neurosurgery departments.
5,018 citations
Book•
01 Jun 1990
TL;DR: This list of diseases for oncology includes cancers of the central nervous system, as well as other types of diseases such as lymphoma, leukaemia, and so on.
Abstract: International classification of diseases for oncology : , International classification of diseases for oncology : , کتابخانه مرکزی دانشگاه علوم پزشکی تهران
3,776 citations
Book•
01 Jan 2000
TL;DR: Tumours of the haemopoietic system Malignant lymphomas Histiocytic tumours, Familial tumour syndromes, and metastatic tumours ofThe CNS.
Abstract: 1: Astrocytic tumours Diffuse astrocytomas Low-grade diffuse astrocytomas Anaplastic astrocytoma Glioblastoma Giant cell glioblastoma Gliosarcoma Pilocytic astrocytoma Pleomorphic xanthoastrocytoma 2: Oligodendroglial tumours and mixed gliomas Oligodendroglioma Anaplastic oligodendroglioma Mixed gliomas Oligoastrocytoma Anaplastic oligoastrocytoma Other mixed gliomas 3: Ependymal tumours Ependymoma and variants Anaplastic ependymoma Myxopapillary ependymoma 4: Choroid plexus tumours 5: Neuroepithelial tumours of uncertain origin Astroblastoma Polar spongioblastoma Gliomatosis cerebri 6: Neuronal and mixed neuronal-glial tumours Gangliocytoma and ganglioglioma Desmoplastic infantile astrocytoma and ganglioglioma Dysembryoplastic neuroepithelial tumours Paraganglioma 7: Pineal parenchymal tumours Pineoblastoma Pineocytoma Mixed pineoblastoma/pineocytoma 8: Embryonal tumours Medulloepithelioma Central neuroblastoma and ganglioneuroblastoma Ependymoblastoma Medulloblastoma Medullomyoblastoma Melanotic medulloblastoma Lipomatous medulloblastoma Supratentorial PNET Atypical teratoid/rhabdoid tumour 9: Peripheral neuroblastic tumours Olfactory neuroblastoma Neuroblastic tumours of the adrenal gland and sympathetic nervous system 10: Tumours of cranial and peripheral nerves Schwannoma Neurofibroma Malignant peripheral nerve sheath tumour (MPNST) 11: Meningeal tumours Meningiomas Mesenchymal, non-meningothelial tumours Haemangiopericytoma Melanocytic lesions 12: Tumours of the haemopoietic system Malignant lymphomas Histiocytic tumours 13: Germ cell tumours 14: Familial tumour syndromes Neurofibromatosis type 1 Neurofibromatosis type 2 Von Hippel-Lindau disease and capillary haemangioblastoma Tuberous sclerosis complex and subependymal giant cell astrocytoma TP53 germline mutations and the Li-Fraumeni syndrome Cowden syndrome Turcot syndrome Naevoid basal cell carcinoma syndrome 15: Tumours of the sellar region Craniopharyngioma 16: Metastatic tumours of the CNS.
1,651 citations
TL;DR: The new edition of the World Health Organization (WHO) book on ‘Histological Typing of Tumours of the Central Nervous System’ reflects the progress in brain tumour classification which has been achieved since publication of the first edition in 1979.
Abstract: The new edition of the World Health Organization (WHO) book on 'Histological Typing of Tumours of the Central Nervous System' reflects the progress in brain tumour classification which has been achieved since publication of the first edition in 1979. Several new tumour entities have been added, including the pleomorphic xanthoastrocytoma, central neurocytoma, the infantile desmoplastic astrocytoma/ganglioglioma, and the dysembryoplastic neuroepithelial tumour. The list of histological variants has also been expanded. In line with recent morphological and molecular data on glioma progression, the glioblastoma is now grouped together with astrocytic tumours. The classification of childhood tumours has been largely retained, the diagnosis primitive neuroectodermal tumour (PNET) only being recommended as a generic term for cerebellar medulloblastomas and neoplasms that are histologically indistinguishable from medulloblastoma but located in the CNS at sites other than the cerebellum. The WHO grading scheme was revised and adapted to new entities but its use, as before, remains optional.
1,532 citations
"The 2007 WHO Classification of Tumo..." refers background in this paper
...The WHO classiWcation of tumours of the nervous system includes a grading scheme that is a ‘malignancy scale’ ranging across a wide variety of neoplasms rather than a strict histological grading system [ 25 , 52]....
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