{ "passages": [ { "id": "cns-who5-overview", "datasets": [ "ebr-coarse30" ], "entities": [], "topic": "taxonomy", "he_observable": false, "text": "WHO CNS5 follows WHO 2016 in large part but revises the glioma and neuronal tumour chapters because of molecular genetics, so diagnosis combines histopathology and molecular findings. The word 'anaplastic' is removed from names such as anaplastic astrocytoma and anaplastic oligodendroglioma but kept for anaplastic meningioma. Arabic numerals are used for CNS WHO grade, and grading is done within a tumour type, so astrocytoma IDH-mutant can be grade 2, 3 or 4.", "source_id": "pmc9427889", "locator": "classification overview", "quote_type": "paraphrase", "facts": [] }, { "id": "cns-adult-diffuse-gliomas", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:astrocytoma-idh-mutant", "cns:oligodendroglioma", "cns:gbm-idh-wt" ], "topic": "taxonomy", "he_observable": false, "text": "The three adult-type diffuse glioma types are astrocytoma IDH-mutant, oligodendroglioma IDH-mutant and 1p/19q-codeleted, and glioblastoma IDH-wildtype. IDH-mutant gliomas have better outcomes than IDH-wildtype glioblastoma. CDKN2A/B homozygous deletion confers a worse prognosis in all three.", "source_id": "pmc10216527", "locator": "abstract and conclusion", "quote_type": "paraphrase", "facts": [] }, { "id": "cns-astrocytoma-idh-histology", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:astrocytoma-idh-mutant" ], "topic": "cytomorphology", "he_observable": true, "text": "IDH-mutant astrocytoma shows diffuse infiltration of neoplastic astrocytic cells into surrounding brain within a fibrillary neuropil background. Resections are hypercellular with an indistinct boundary against normal brain, and entrapped neurons can be seen inside the tumour. The cells have elongated hyperchromatic nuclei and scant cytoplasm, with glassy eosinophilic cytoplasm in gemistocytic variants, and nuclear pleomorphism is often present.", "source_id": "pmc10216527", "locator": "IDH-mutant astrocytoma, histology", "quote_type": "paraphrase", "facts": [ { "s": "cns:astrocytoma-idh-mutant", "r": "has_feature", "o": "cns:diffuse-infiltration", "he_observable": true }, { "s": "cns:astrocytoma-idh-mutant", "r": "has_feature", "o": "cns:fibrillary-background", "he_observable": true }, { "s": "cns:astrocytoma-idh-mutant", "r": "has_feature", "o": "cns:elongated-hyperchromatic-nuclei", "he_observable": true } ] }, { "id": "cns-astrocytoma-idh-grading", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:astrocytoma-idh-mutant" ], "topic": "grading", "he_observable": true, "text": "IDH-mutant astrocytoma is grade 2, 3 or 4. Grade 2 shows diffuse infiltration with a mild-to-moderate increase in cellularity, uncommon mitoses, and no microvascular proliferation or necrosis. Grade 3 is more cellular with more nuclear pleomorphism, and increased mitoses are the definitive criterion for grade 3. Microvascular proliferation or necrosis makes it grade 4, and so does CDKN2A/B homozygous deletion regardless of histology. A grade 4 IDH-mutant tumour is no longer called glioblastoma.", "source_id": "pmc10216527", "locator": "IDH-mutant astrocytoma, grading", "quote_type": "paraphrase", "facts": [ { "s": "cns:astrocytoma-idh-mutant", "r": "grade_range", "o": "CNS WHO grade 2 to 4", "he_observable": false }, { "s": "cns:astrocytoma-idh-mutant", "r": "distinguished_from", "o": "cns:gbm-idh-wt", "feature": "idh-mutation", "he_observable": false } ] }, { "id": "cns-astrocytoma-idh-ihc", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:astrocytoma-idh-mutant" ], "topic": "immunohistochemistry", "he_observable": false, "text": "IDH-mutant astrocytoma is expected to show cytoplasmic IDH1 R132H staining, loss of nuclear ATRX staining, and strong p53 overexpression. It is strongly associated with ATRX and TP53 mutations. Ki67 is variably raised. This is not visible on H&E.", "source_id": "pmc10216527", "locator": "IDH-mutant astrocytoma, immunohistochemistry", "quote_type": "paraphrase", "facts": [] }, { "id": "cns-oligodendroglioma-histology", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:oligodendroglioma" ], "topic": "cytomorphology", "he_observable": true, "text": "Oligodendroglioma is a diffuse neoplasm with infiltrating cells that have monomorphic ovoid-to-round nuclei with stippled chromatin and perinuclear haloes, the 'fried-egg' appearance. A delicate capillary network forms a 'chicken-wire' pattern. Secondary structures include perivascular accumulation, aggregates in the subpial space and perineuronal satellitosis. Microcalcification, haemorrhage and cystic degeneration with myxoid material can occur. Some cases have minigemistocytes that are strongly GFAP positive.", "source_id": "pmc10216527", "locator": "oligodendroglioma, histology", "quote_type": "paraphrase", "facts": [ { "s": "cns:oligodendroglioma", "r": "has_feature", "o": "cns:fried-egg-cells", "he_observable": true }, { "s": "cns:oligodendroglioma", "r": "has_feature", "o": "cns:chicken-wire-capillaries", "he_observable": true }, { "s": "cns:oligodendroglioma", "r": "has_feature", "o": "cns:microcalcification", "he_observable": true }, { "s": "cns:oligodendroglioma", "r": "has_feature", "o": "cns:perineuronal-satellitosis", "he_observable": true }, { "s": "cns:oligodendroglioma", "r": "distinguished_from", "o": "cns:astrocytoma-idh-mutant", "feature": "cns:fried-egg-cells", "he_observable": true }, { "s": "cns:oligodendroglioma", "r": "distinguished_from", "o": "cns:astrocytoma-idh-mutant", "feature": "cns:chicken-wire-capillaries", "he_observable": true }, { "s": "cns:oligodendroglioma", "r": "distinguished_from", "o": "cns:astrocytoma-idh-mutant", "feature": "1p19q-codeletion", "he_observable": false } ] }, { "id": "cns-oligodendroglioma-molecular-grading", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:oligodendroglioma" ], "topic": "grading", "he_observable": false, "text": "Oligodendroglioma is defined by an IDH1 or IDH2 mutation plus codeletion of chromosome arms 1p and 19q, and shows retained nuclear ATRX and a wild-type p53 pattern. It is grade 2 or 3, with mitotic activity, microvascular proliferation and necrosis separating grade 3. CDKN2A/B homozygous deletion can mark grade 3 in borderline histology. The legacy name anaplastic oligodendroglioma corresponds to grade 3 of this type.", "source_id": "pmc10216527", "locator": "oligodendroglioma, molecular features and grading", "quote_type": "paraphrase", "facts": [ { "s": "cns:oligodendroglioma", "r": "grade_range", "o": "CNS WHO grade 2 to 3", "he_observable": false } ] }, { "id": "cns-gbm-histology", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:gbm-idh-wt" ], "topic": "cytomorphology", "he_observable": true, "text": "Conventional glioblastoma is a densely cellular infiltrative tumour of fibrillary astrocytic cells with highly hyperchromatic, pleomorphic nuclei and brisk mitotic activity. Necrosis and microvascular proliferation are readily found. Histologic variants include giant cell, sarcomatous (gliosarcoma), epithelioid, granular cell, and small cell types, the last of which can resemble a high-grade oligodendroglioma.", "source_id": "pmc10216527", "locator": "glioblastoma, histology", "quote_type": "paraphrase", "facts": [ { "s": "cns:gbm-idh-wt", "r": "has_feature", "o": "cns:necrosis", "he_observable": true }, { "s": "cns:gbm-idh-wt", "r": "has_feature", "o": "cns:microvascular-proliferation", "he_observable": true }, { "s": "cns:gbm-idh-wt", "r": "has_feature", "o": "cns:brisk-mitoses", "he_observable": true }, { "s": "cns:gbm-idh-wt", "r": "distinguished_from", "o": "cns:astrocytoma-idh-mutant", "feature": "cns:necrosis", "he_observable": true }, { "s": "cns:gbm-idh-wt", "r": "distinguished_from", "o": "cns:astrocytoma-idh-mutant", "feature": "cns:microvascular-proliferation", "he_observable": true } ] }, { "id": "cns-gbm-definition", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:gbm-idh-wt", "cns:anaplastic-astrocytoma-idh-wt-legacy" ], "topic": "grading", "he_observable": false, "text": "Glioblastoma IDH-wildtype is grade 4 and is IDH-wildtype and H3-wildtype by definition. An IDH-wildtype diffuse astrocytoma without grade 4 histology is still diagnosed as glioblastoma when it has EGFR amplification, combined chromosome 7 gain with chromosome 10 loss, or a TERT promoter mutation. TERT promoter mutations are the most frequent of these, but they are less specific and also occur in oligodendroglioma and melanoma.", "source_id": "pmc10216527", "locator": "glioblastoma, diagnostic criteria", "quote_type": "paraphrase", "facts": [ { "s": "cns:gbm-idh-wt", "r": "grade_range", "o": "CNS WHO grade 4", "he_observable": false } ] }, { "id": "cns-gliosarcoma", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:gliosarcoma" ], "topic": "definition", "he_observable": true, "text": "Gliosarcoma is the sarcomatous histologic variant of glioblastoma. The source lists it as one subtype among giant cell, epithelioid, granular cell and small cell variants and gives no further morphologic description.", "source_id": "pmc10216527", "locator": "glioblastoma, histologic variants", "quote_type": "paraphrase", "facts": [ { "s": "cns:gliosarcoma", "r": "variant_of", "o": "cns:gbm-idh-wt", "he_observable": true }, { "s": "cns:gliosarcoma", "r": "has_feature", "o": "cns:sarcomatous-component", "he_observable": true } ] }, { "id": "cns-legacy-names", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:astrocytoma-idh-mutant", "cns:gbm-idh-wt", "cns:oligodendroglioma", "cns:anaplastic-astrocytoma-idh-wt-legacy" ], "topic": "labeling_note", "he_observable": false, "text": "The dataset uses older class names. Under CNS5, glioblastoma IDH-mutant and anaplastic astrocytoma IDH-mutant are no longer separate types but grade 4 and grade 3 of astrocytoma IDH-mutant, and diffuse astrocytoma IDH-mutant is grade 2. Anaplastic oligodendroglioma is grade 3 of oligodendroglioma. An IDH-wildtype diffuse astrocytoma with at least one of the glioblastoma molecular criteria counts as glioblastoma IDH-wildtype, so the legacy name anaplastic astrocytoma IDH-wildtype does not map to a separate CNS5 type. Labels that differ only by grade or legacy naming can look alike on H&E.", "source_id": "pmc10216527", "locator": "WHO CNS5 nomenclature changes (also in pmc9427889 classification overview)", "quote_type": "paraphrase", "facts": [] }, { "id": "cns-pilocytic", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:pilocytic-astrocytoma" ], "topic": "definition", "he_observable": true, "text": "Circumscribed astrocytic gliomas have well-defined margins and less infiltrative growth. Pilocytic astrocytoma is the most common of them and stays CNS WHO grade 1. It can be diagnosed by classical histopathology, or as a low-grade piloid astrocytic neoplasm with a single MAPK alteration such as a KIAA1549::BRAF fusion. Pilomyxoid astrocytoma is a subtype with somewhat worse prognosis but still grade 1. The source gives no further morphologic description.", "source_id": "pmc9427889", "locator": "circumscribed astrocytic gliomas", "quote_type": "paraphrase", "facts": [ { "s": "cns:pilocytic-astrocytoma", "r": "has_feature", "o": "cns:circumscribed-margin", "he_observable": true }, { "s": "cns:pilocytic-astrocytoma", "r": "grade_range", "o": "CNS WHO grade 1", "he_observable": false }, { "s": "cns:pilocytic-astrocytoma", "r": "distinguished_from", "o": "cns:astrocytoma-idh-mutant", "feature": "cns:circumscribed-margin", "he_observable": true } ] }, { "id": "cns-ganglioglioma", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:ganglioglioma" ], "topic": "taxonomy", "he_observable": false, "text": "Ganglioglioma belongs to the glioneuronal and neuronal tumours, which are neuronal or mixed glial-neuronal tumours. BRAF V600 mutations are found in ganglioglioma, as in pleomorphic xanthoastrocytoma. The source gives no histologic description of ganglioglioma.", "source_id": "pmc9427889", "locator": "tables of tumour groups and molecular markers", "quote_type": "paraphrase", "facts": [] }, { "id": "cns-ependymoma", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:ependymoma" ], "topic": "taxonomy", "he_observable": false, "text": "Ependymomas are classified by histopathology, anatomic site and molecular features. Supratentorial tumours are split by ZFTA or YAP1 fusion, posterior fossa tumours by PFA or PFB group (H3 K27 trimethylation), and spinal tumours include a MYCN-amplified group with poor course. The papillary, clear cell and tanycytic morphologic subtypes have no clinical relevance and are no longer listed. The source gives no histologic description.", "source_id": "pmc9427889", "locator": "ependymomas", "quote_type": "paraphrase", "facts": [] }, { "id": "cns-meningioma-grading", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:meningioma-transitional", "cns:meningioma-fibrous", "cns:meningioma-angiomatous", "cns:meningioma-meningothelial", "cns:meningioma-secretory", "cns:meningioma-atypical", "cns:meningioma-anaplastic" ], "topic": "grading", "he_observable": true, "text": "In CNS5 meningioma is a single tumour type with 15 subtypes and three grades. Meningothelial, fibrous, transitional, psammomatous, angiomatous, microcystic, secretory and lymphoplasmacyte-rich subtypes are grade 1 or 2 by histologic criteria. Atypical meningioma, which includes brain-invasive tumours, is grade 2, as are chordoid and clear cell subtypes. Anaplastic (malignant) meningioma is grade 3. The source lists grades and genetics but does not describe the morphology of the subtypes.", "source_id": "pmc9427889", "locator": "meningiomas, grading table", "quote_type": "paraphrase", "facts": [ { "s": "cns:meningioma-atypical", "r": "grade_range", "o": "CNS WHO grade 2", "he_observable": false }, { "s": "cns:meningioma-anaplastic", "r": "grade_range", "o": "CNS WHO grade 3", "he_observable": false } ] }, { "id": "cns-meningioma-genetics", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:meningioma-fibrous", "cns:meningioma-transitional", "cns:meningioma-secretory", "cns:meningioma-anaplastic" ], "topic": "molecular", "he_observable": false, "text": "NF2 mutation is linked to convexity meningiomas and to the fibrous and transitional subtypes, which are more often grade 2 or 3. TRAF7 mutation is linked to the secretory subtype. TERT promoter mutation and CDKN2A/B loss are linked to grade 3. These are not visible on H&E.", "source_id": "pmc9427889", "locator": "meningiomas, genetic alteration table", "quote_type": "paraphrase", "facts": [] }, { "id": "cns-medulloblastoma", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:medulloblastoma" ], "topic": "taxonomy", "he_observable": false, "text": "Embryonal tumours are all grade 4. Medulloblastoma has four histologic types (classic, desmoplastic/nodular, extensive nodularity, large cell/anaplastic) combined into one type 'histologically defined', and four molecular groups: WNT-activated, SHH-activated, and non-WNT/non-SHH, which merges groups 3 and 4. Histologic and molecular features should both enter the integrated diagnosis.", "source_id": "pmc9427889", "locator": "embryonal tumours", "quote_type": "paraphrase", "facts": [] }, { "id": "cns-nerve-sheath", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:schwannoma" ], "topic": "taxonomy", "he_observable": false, "text": "Schwannoma is listed with neurofibroma, perineurioma, hybrid nerve sheath tumour and malignant peripheral nerve sheath tumour among cranial and paraspinal nerve tumours, which can be sporadic or occur in NF1 and NF2 syndromes. The source gives no histologic description of schwannoma.", "source_id": "pmc9427889", "locator": "cranial and paraspinal nerve tumours", "quote_type": "paraphrase", "facts": [] }, { "id": "cns-sellar", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:craniopharyngioma-adamantinomatous", "cns:pituitary-adenoma" ], "topic": "taxonomy", "he_observable": false, "text": "In CNS5 adamantinomatous and papillary craniopharyngiomas are separate, distinct types. Pituitary adenomas are diagnosed according to the WHO endocrine tumours volume, which this source does not reproduce. No histologic description of either is given.", "source_id": "pmc9427889", "locator": "tumours of the sellar region", "quote_type": "paraphrase", "facts": [] }, { "id": "cns-haemato-mets", "datasets": [ "ebr-coarse30" ], "entities": [ "cns:cns-dlbcl", "cns:lch", "cns:metastasis" ], "topic": "taxonomy", "he_observable": false, "text": "Lymphomas and histiocytic tumours are grouped together. The most common primary CNS lymphoma is diffuse large B-cell lymphoma of the CNS, formerly called primary CNS lymphoma, and it is important to tell primary from secondary CNS involvement. Metastases are divided into those of brain and spinal cord parenchyma and those of the meninges. No histologic description is given.", "source_id": "pmc9427889", "locator": "haematolymphoid tumours and metastases", "quote_type": "paraphrase", "facts": [] } ] }