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
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Diffuse astrocytic and
oligodendroglial tumours
Other astrocytic tumours
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Mesenchymal, nonmeningothelial tumours
Melanocytic tumours
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Ependymal tumours
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Histiocytic tumours
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Other gliomas
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Germ cell tumours
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Choroid plexus tumours
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Familial tumour syndromes
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Tumours of the sellar region
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Neuronal and mixed neuronal–
glial tumours
Tumours of the pineal region
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Metastatic tumours
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Embryonal tumours
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Tumours of the cranial and
paraspinal nerves
Meningiomas
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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
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Histopathological name followed by the genetic features, for example, diffuse astrocytoma,
isocitrate dehydrogenase (IDH)-mutant and medulloblastoma, and tumors in wingless
(WNT)-activated
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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
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For a tumor lacking a genetic mutation: The term wild type can be used, for example,
glioblastoma and IDH-wild type
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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
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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