Tumors of the Central Nervous System Dr. Yuriy Chomolyak, MD, PhD Associate Professor of Neurosurgery Department of Neurology, Neurosurgery and Psychiatry Medical Faculty Uzhhorod National University Epidemiology 50% of the tumors are primary, arising from the nervous system cells or meninges; the others — metastases of malignant tumors into the nervous system Data from the National Cancer Registry of Ukraine on the incidence of primary brain tumors: 1990 - 4.0; 2000 - 4.6; currently 10.9-12.8 per 100 thousand population Data published by CBTRUS: 1990 - 8.2; 1995 - 10.9; 2000 - 12.8 per 100 thousand population Classification of CNS tumors • • Diffuse astrocytic and oligodendroglial tumours Other astrocytic tumours • Mesenchymal, nonmeningothelial tumours Melanocytic tumours • Ependymal tumours • Histiocytic tumours • Other gliomas • Germ cell tumours • Choroid plexus tumours • Familial tumour syndromes • • Tumours of the sellar region • Neuronal and mixed neuronal– glial tumours Tumours of the pineal region • Metastatic tumours • Embryonal tumours • Tumours of the cranial and paraspinal nerves Meningiomas • • Acta Neuropathol 2016 Jun;131(6):803-20. doi: 10.1007/s00401-016-1545-1. Epub 2016 May 9. PMID: 27157931 DOI: 10.1007/s00401-016-1545-1 WHO grade classification G1 Tumors do not meet any of the criteria. These tumors are slow growing, nonmalignant, and associated with long-term survival G2 Tumors meet only one criterion, i.e., only cytological atypia. These tumors are slow growing but recur as higher-grade tumors. They can be malignant or nonmalignant G3 Tumors meet two criteria, i.e., anaplasia and mitotic activity. These tumors are malignant and often recur as higher-grade tumors G4 Tumors meet three or four of the criteria, i.e., showing anaplasia, mitotic activity with microvascular proliferation, and/or necrosis. These tumors reproduce rapidly and are very aggressive malignant tumors J Neurosci Rural Pract. 2017 Oct-Dec; 8(4): 629–641. doi: 10.4103/jnrp.jnrp_168_17 PMCID: PMC5709890 PMID: 29204027 New nomenclature • Histopathological name followed by the genetic features, for example, diffuse astrocytoma, isocitrate dehydrogenase (IDH)-mutant and medulloblastoma, and tumors in wingless (WNT)-activated • For entities with more than one genetic determinant: Histopathological name followed by the multiple molecular features are included in the name, for example, oligodendroglioma, IDHmutant, and1p/19q-codeleted • For a tumor lacking a genetic mutation: The term wild type can be used, for example, glioblastoma and IDH-wild type • For laboratory lacking any access to molecular diagnostic testing, the term not otherwise specified (NOS) can be used (i.e., NOS). NOS is also applicable to tumors, in which genetic assay testing is inconclusive. An NOS designation implies that there is insufficient information to assign a most specific code • For tumor entities, in which a specific genetic alteration is present, the terms “positive” can be used is present, for example, ependymoma and RELA fusion-positive The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Louis DN at al. Acta Neuropathol. 2016 Jun; 131(6):803-20. Main molecular markers Isocitrate dehydrogenase (IDH) 1p/19q co-deletion O6-methylguanine-DNA methyltransferase methylation (MGMT) TERT (Telomerase reverse transcriptase) promoter mutations Alpha-thalassemia/mental retardation syndrome X-linked (ATRX) Tumor protein p53 Tumors in wingless and sonic hedgehog activation C19MC alteration H3 K27M-mutation RELA fusion C11orf95 Reporting format Neurol Med Chir (Tokyo). 2017 Jul; 57(7): 301–311. Published online 2017 Jun 8. doi: 10.2176/nmc.ra.2017-0010 Natural history of the CNS tumors Presentation 5,8% - acute 12,3%-subacute 81,9%-chronic Course Progressive /permamemt progression/ - 63% Progredient /with periods of stabilisation/ - 27% Remitting - 10% Clinical presentation Progessive neurologic deficit 68% • Motor weakness 45% Headache 54% Seizures 26% Neurological examination Diffuse neurologic symptoms Headache, nusea, vomiting, vertigo Focal neurologic deficit Pereses, sensory loss,… Epileptic seizures Meningeal symptoms (rare) Focal neurologic deficits Frontal lobe: abulia, dementia, personality changes, hemiparesis or dysphasia Temporal lobe: auditory or olfactory hallucinations. deja vu. memory impairment, contralateral superior quadrantanopsia may be detected on visual field testing Parietal lobe: contralateral motor or sensory impairment, homonymous hemyanopsia, agnosias, apraxias Occipital lobe: contralateral visual field deficit, alexia (corpus callosum involvement) Posterior fossa: cranial nerve deficits, ataxia M.Greenberg, 2006 Neuroimaging Ionizing radiation studies X-ray, CT, Angiography Magnetic resonance imaging MR-tomography, МR-spectroskopy, MR-perfusion etc. Functional imaging PET, SPECT Ultrasound studies Echoencephaloskopy Drug treatment Corticosteroid therapy - dexamethasone Osmotic diuretics - mannitol 0.5-1.5 mg/kg/day Saluretics - furosemide Surgery Safety of the operation and quality of life in the postoperative period Extracerebral tumors are subject to total removal, intracerebral - maximal safe resection Radiosurgery (Gamma Knife, LINAC, Cyber Knife) The method is effective and can be used in the presence of pathological foci no larger than 3-3.5 cm The effect of radiosurgical treatment is considered positive if it is possible to control the growth or reduction of the size of the pathological focus over time. One of the inclusion criteria is the patient's condition, usually more than 70 points on the Karnofsky Performance Score (KPS) ranking Glioblastoma ( GBM, grade IV ) age of patients: 45 - 70 years (however it can be any) highly malignant tumor with a heterogeneous internal structure, with the presence of a large area of necrosis in the center; CT: iso- / hypodensive lesion; MRI: ↓ intensity on T1WI; ↑ intensity of MRS on T2WI and FLAIR; there is no restriction of diffusion on DWI; perifocal edema and mass effect - ↑↑↑; necrosis - present (= hallmark); pathologically tortuous vascular structures, intra-tumor hemorrhages and cysts - often; calcifications - rarely (in comparison with ASC NHS); contrast enhancement: usually heterogeneous, peripheralannular type (so-called "crown effect") Glioblastoma (GBM) Tumours of the cranial and paraspinal nerves Schwannoma Anaplastic Schwannoma Malignant Schwannoma Neurofibroma Neurofibrosarcoma Acoustic Schwannoma Hourglass tumor Tumours of the sellar region Mesenchymal, non-meningothelial tumours Hemangioma Hemangioblastoma Hemangioendothelioma Hemangiopericytoma Angiosarcoma Hemangiopericytoma Meningiomas Parasagittal/falcine (25%) Convexity (19%) Sphenoid ridge (17%) Suprasellar (9%) Posterior fossa (8%) Olfactory groove (8%) Middle fossa/Meckel's cave (4%) Tentorial (3%) Peri-torcular (3%) Joung H. Lee (2008-12-11). Meningiomas: Diagnosis, Treatment, and Outcome. Springer Science & Business Media. pp. 3–13. ISBN 978-1-84628-784-8. Suprasellar meningioma Convexity meningiomas Thank you