the who 2000 tumor classification james g. smirniotopoulos, m.d. uniformed services university of...
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THE WHO 2000 TUMOR THE WHO 2000 TUMOR CLASSIFICATIONCLASSIFICATION
James G. Smirniotopoulos, M.D.James G. Smirniotopoulos, M.D.
Uniformed Services UniversityUniformed Services University
of the Health Sciencesof the Health Sciences
Bethesda, MDBethesda, MD
Visit us at: http://rad.usuhs.milVisit us at: http://rad.usuhs.mil
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The WHO 2000The WHO 2000Classification ofClassification of
Brain TumorsBrain Tumors
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DISCLAIMERDISCLAIMER::
The opinions expressed herein are those of the author(s), The opinions expressed herein are those of the author(s), and are not necessarily representative of the Uniformed and are not necessarily representative of the Uniformed Services University of the Health Sciences (Services University of the Health Sciences (USUHSUSUHS), the ), the Department of Defense (Department of Defense (DODDOD); or the World Health ); or the World Health Organization (Organization (WHOWHO). Medicine is a constantly changing ). Medicine is a constantly changing field, and medical information is subject to frequent field, and medical information is subject to frequent correction and revision. Therefore the reader is entirely correction and revision. Therefore the reader is entirely responsible for verifying the accuracy and relevance of the responsible for verifying the accuracy and relevance of the information contained herein. Portions copyright 1997 information contained herein. Portions copyright 1997 James G. Smirniotopoulos, M.D.James G. Smirniotopoulos, M.D.
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Traditional Tumor Traditional Tumor GradingGrading
PATHOLOGISTPATHOLOGIST– LOW GRADELOW GRADE– HIGH GRADEHIGH GRADE
RADIOLOGISTRADIOLOGIST– NON-ENHANCINGNON-ENHANCING– ENHANCINGENHANCING
NEUROSURGEONNEUROSURGEON– ““SUCKABLE”SUCKABLE”– ““NON-SUCKABLE”NON-SUCKABLE”
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Define the Problem:Define the Problem:
Some Low Grade EnhanceSome Low Grade Enhance Some Low Grade Do Not Some Low Grade Do Not
Some Low Grade => GBMSome Low Grade => GBM Some Low Grade Do NotSome Low Grade Do Not
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WHO ClassificationWHO Classification
Defines Histologic SubtypesDefines Histologic Subtypes Grades Biologic PotentialGrades Biologic Potential Allows International Allows International
CooperationCooperation
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WHO ClassificationWHO Classification
Biological PotentialBiological Potential
Ascending Scale of Aggression I - Ascending Scale of Aggression I - IVIV
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WHO CORRELATIONWHO CORRELATION
Low GradeLow Grade
– Long-Term SurvivalLong-Term Survival
– Stable History (No Progression)Stable History (No Progression)
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WHO GRADINGWHO GRADING
GRADE 1GRADE 1 JPAJPA SGCA SGCA GANG MENINGGANG MENING
GRADE 2GRADE 2 PXAPXA HPC HPC
GRADE 3GRADE 3 PXAPXA ANAPLASTIC ANAPLASTIC HPC HPC
GRADE 4GRADE 4 GBMGBM
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CNS NEOPLASM-GLIALCNS NEOPLASM-GLIALPrognostic FactorsPrognostic Factors
LocationLocation AgeAge
HistologyHistology
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MalignancyMalignancy
HISTOLOGIC MALIGNANCYHISTOLOGIC MALIGNANCY– microscopicmicroscopic
BIOLOGIC MALIGNANCYBIOLOGIC MALIGNANCY– macroscopicmacroscopic– labeling indiceslabeling indices– molecular biologymolecular biology
CLINICAL MALIGNANCYCLINICAL MALIGNANCY
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HISTOLOGIC MALIGNANCYHISTOLOGIC MALIGNANCY
CELLULAR ATYPIACELLULAR ATYPIA MITOSES (Mitotic Index)MITOSES (Mitotic Index) INFILTRATION INFILTRATION
(lack of margination or (lack of margination or encapsulation)encapsulation)
NECROSISNECROSIS(sign of uncontrolled growth)(sign of uncontrolled growth)
VASCULAR CHANGESVASCULAR CHANGES(tumor neovascularity)(tumor neovascularity)
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Kernohan-Sayre (AFIP)Kernohan-Sayre (AFIP)Grading System:Grading System:
GRADE IGRADE I - “BENIGN” or “Low-Grade”- “BENIGN” or “Low-Grade” GRADE II - “ “GRADE II - “ “ GRADE III - ANAPLASTICGRADE III - ANAPLASTIC
– cellular atypia, etc.cellular atypia, etc. GRADE IV- MALIGNANTGRADE IV- MALIGNANT
– NECROSIS !, Vascularity, MitosesNECROSIS !, Vascularity, Mitoses– GLIOBLASTOMA MULTIFORMEGLIOBLASTOMA MULTIFORME
NOTE: Numerous modifications exist, NOTE: Numerous modifications exist, most into three grades, e.g..: Low Grade most into three grades, e.g..: Low Grade (Benign), Anaplastic, and GBM (w/ (Benign), Anaplastic, and GBM (w/ NECROSIS).NECROSIS).
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HIGH-GRADE ASTROCYTOMASHIGH-GRADE ASTROCYTOMASMEDIAN SURVIVAL:MEDIAN SURVIVAL:
ANAPLASTIC (Grade 3/4) - 28Mos.ANAPLASTIC (Grade 3/4) - 28Mos.
GBM (Grade 4/4, has NECROSIS) - GBM (Grade 4/4, has NECROSIS) - 8 Mos.8 Mos.
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ASTROCYTOMAASTROCYTOMAFive Year SurvivalFive Year Survival
0 10 20 30 40 50 60 70 80 90 100
Percent Survival
Glioblastoma (IV)
Anaplastic (III)
Astrocytoma (I-II)
Pilocytic
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BIOLOGIC BIOLOGIC MALIGNANCY:MALIGNANCY:
RAPID GROWTHRAPID GROWTH HEMORRHAGE, NECROSISHEMORRHAGE, NECROSIS LOCAL EXTENSIONLOCAL EXTENSION HEMATOGENOUS DISSEMINATIONHEMATOGENOUS DISSEMINATION
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““BENIGN” ASTROCYTOMABENIGN” ASTROCYTOMA
Two types:Two types:–Low grade (“benign”)Low grade (“benign”)
DiffuseDiffuse (Adults)(Adults)–Low grade “special”Low grade “special”
Circumscribed Circumscribed (Children)(Children)
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ASTROCYTIC ASTROCYTIC NEOPLASMSNEOPLASMS
WHO ClassificationWHO Classification Astrocytoma (Diffuse)Astrocytoma (Diffuse)(fibrillary, protoplasmic, or gemistocytic (fibrillary, protoplasmic, or gemistocytic
astrocytes)astrocytes) Anaplastic Astrocytoma (AA)Anaplastic Astrocytoma (AA) Glioblastoma Multiforme (GBM)Glioblastoma Multiforme (GBM)
(Giant Cell GBM, Gliosarcoma)(Giant Cell GBM, Gliosarcoma)____________________________________________________________
Pilocytic Astrocytoma (Juvenile - JPA)Pilocytic Astrocytoma (Juvenile - JPA) Subependymal Giant Cell AstrocytomaSubependymal Giant Cell Astrocytoma Superficial Cerebral AstrocytomaSuperficial Cerebral Astrocytoma Pleomorphic Xanthoastrocytoma (PXA)Pleomorphic Xanthoastrocytoma (PXA)
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Grade vs. TypeGrade vs. TypeCircumscribed Diff use
StableHistology(Grade)
Pilocytic (1)SubependymalGiant Cell (1)
UnstableHistology(Grade)
PleomorphicXantho -astrocytoma (2- 3)
Astrocytoma (2)Anaplastic (3)GBM (4)
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Enhancement vs. TypeEnhancement vs. TypeCircumscribed Diff use
NoEnhancement
Astrocytoma (2)
VariableEnhancement
Anaplastic (3)
RoutineEnhancement
Pilocytic (1)SEGCA(1)PXA (2- 3)
GBM (4)
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PATTERN ANALYSISPATTERN ANALYSIS
Pilocytic(Juvenile)
Subependym alG iant
Cell Astrocytom a
Pleom orphicXanthoastrocytom a
Circum scribedAstrocytom a
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ASTROCYTOMA:ASTROCYTOMA:CircumscribedCircumscribed
““Special” astrocytomas, Special” astrocytomas, Astrocytoma of YoungAstrocytoma of Young
Well circumscribed (yet, no capsule)Well circumscribed (yet, no capsule) Various LocationsVarious Locations Do NOT change grade (except PXA)Do NOT change grade (except PXA) Do NOT spread along WMDo NOT spread along WM Constellation of findings correlates Constellation of findings correlates
w/ Histologyw/ Histology
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PILOCYTIC ASTROCYTOMAPILOCYTIC ASTROCYTOMA
Cystic Cerebellar Cystic Cerebellar Astrocytoma Juvenile Astrocytoma Juvenile Pilocytic AstrocytomaPilocytic Astrocytoma
(“PA” or “JPA”)(“PA” or “JPA”)
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PILOCYTIC ASTROCYTOMAPILOCYTIC ASTROCYTOMA Synonyms: Polar Spongioblastoma, Cystic Synonyms: Polar Spongioblastoma, Cystic
Cerebellar AstrocytomaCerebellar Astrocytoma Cell of Origin: Astrocyte (bi-polar, hairlike)Cell of Origin: Astrocyte (bi-polar, hairlike) Associations: in ON w/ NF-1Associations: in ON w/ NF-1 Incidence: 3-6% of ALL Cranial, 32% of ChildIncidence: 3-6% of ALL Cranial, 32% of Child Age: 5-15 (Zulch 3-7) Sex: Slight F Age: 5-15 (Zulch 3-7) Sex: Slight F
(11/9)(11/9) Location: Cerebellum, Chiasm/Hypothal, Location: Cerebellum, Chiasm/Hypothal,
OpticOptic Treatment: Surgery, patienceTreatment: Surgery, patience Prognosis: 77% at 5 yrs.Prognosis: 77% at 5 yrs.
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PILOCYTIC ASTROCYTOMAPILOCYTIC ASTROCYTOMARadiologyRadiology
Cerebellum, DiencephalonCerebellum, Diencephalon– rare in BS or Cerebrumrare in BS or Cerebrum
Majority have significant Majority have significant “cyst”“cyst”– ““Cyst and Mural Nodule”Cyst and Mural Nodule”
part of lining does NOT enhancepart of lining does NOT enhance
– Nodule may be heterogeneousNodule may be heterogeneous– Exceptional purely solidExceptional purely solid
Nodule NOT hyperdenseNodule NOT hyperdense Calcification in 5-25%Calcification in 5-25%
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PATHOLOGYPATHOLOGY
Biphasic patternBiphasic pattern– dense pilocytic gliadense pilocytic glia– Rosenthal fibersRosenthal fibers– loose microcystic areasloose microcystic areas
No necrosisNo necrosis Low gradeLow grade Abnormal capillariesAbnormal capillaries
– allow enhancement, allow enhancement, fluidfluid
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Grading GliomasGrading Gliomas51 51 Pilocytic (WHO Gr. 1)Pilocytic (WHO Gr. 1)
KERNOHAN KERNOHAN MAYO-ST.ANNE MAYO-ST.ANNE
1 26% 1 26% 1 2%1 2%
2 69%2 69% 2 55%2 55%
33 6% 6% 3 35%3 35%
4 0%4 0% 4 8%4 8%
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ASTROCYTOMAASTROCYTOMAFive Year SurvivalFive Year Survival
0 20 40 60 80 100
Percent Survival
Glioblastoma (IV)
Anaplastic (III)
Astrocytoma (I-II)
Pilocytic
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PILOCYTIC PILOCYTIC ASTROCYTOMA:ASTROCYTOMA:
LocationsLocations CEREBELLUMCEREBELLUM
Chiasm And Optic NerveChiasm And Optic Nerve
Hypothalmus/thalamusHypothalmus/thalamus Cerebral HemisphereCerebral Hemisphere Spinal Cord (Intramedullary)Spinal Cord (Intramedullary)
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Pilocytic Astrocytoma Pilocytic Astrocytoma HemangioblastomaHemangioblastoma
Enhance Enhance EnhanceEnhance
Cyst w/ NoduleCyst w/ Nodule Solid <--> CysticSolid <--> Cystic
Hypodense noduleHypodense nodule HyperdenseHyperdense
Calcification Calcification Never Ca++Never Ca++
NOT vascular NOT vascular Hypervascular, Flow Hypervascular, Flow VoidsVoids
Nodule varies Nodule varies Nodule is “Subpial”Nodule is “Subpial”
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PILOCYTIC ASTROCYTOMAPILOCYTIC ASTROCYTOMA(Juvenile Pilocytic)(Juvenile Pilocytic)
Childhood, Young AdultsChildhood, Young Adults Benign, no mitosis/necrosisBenign, no mitosis/necrosis Circumscribed - EnhancingCircumscribed - Enhancing Cyst Formation, Mural NoduleCyst Formation, Mural Nodule Cerebellum and DiencephalonCerebellum and Diencephalon
(Optic tracts, Hypothalmus)(Optic tracts, Hypothalmus)
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WHO GRADE IWHO GRADE I
Circumscribed Circumscribed AstrocytomaAstrocytoma– JPA (Pilocytic)JPA (Pilocytic)– SGCA (Subependymal)SGCA (Subependymal)
GangliogliomaGanglioglioma MeningiomaMeningioma
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CIRCUMSCRIBED vs CIRCUMSCRIBED vs DIFFUSEDIFFUSE
DiffuseAstrocytom a
Circum scribedAstrocytom a
Astrocytom a
NEUROECT ODERM ALNEOPLASM
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ASTROCYTOMASASTROCYTOMAS
““SPECIAL” ASTROCYTOMASSPECIAL” ASTROCYTOMAS– Circumscribed Growth:Circumscribed Growth:
PilocyticPilocyticSubependymal Giant CellSubependymal Giant CellPleomorphic Xantho-Pleomorphic Xantho-AstrocytomaAstrocytoma
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PLEOMORPHIC PLEOMORPHIC XANTHOASTROCYTOMAXANTHOASTROCYTOMA
Recently Described, Rare Recently Described, Rare Variant of AstrocytomaVariant of Astrocytoma
Arises from Subpial AstrocytesArises from Subpial Astrocytes Affects Superficial Cerebral Affects Superficial Cerebral
Cortex and MeningesCortex and Meninges Temporal > Frontal > ParietalTemporal > Frontal > Parietal
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PLEOMORPHIC PLEOMORPHIC XANTHOASTROCYTOMAXANTHOASTROCYTOMA IMAGING:IMAGING:– CT APPEARANCE:CT APPEARANCE:
Well-Circumscribed Well-Circumscribed Hypodense or Cystic MassHypodense or Cystic Mass
Often Isodense Solid Nodule Often Isodense Solid Nodule That Intensely EnhancesThat Intensely Enhances
May Mimic Juvenile Pilocytic May Mimic Juvenile Pilocytic AstrocytomaAstrocytoma
Calcifications RareCalcifications Rare
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PLEOMORPHIC PLEOMORPHIC XANTHOASTROCYTOMAXANTHOASTROCYTOMA
MR APPEARANCE:MR APPEARANCE:– Well-Circumscribed Mass of Variable Well-Circumscribed Mass of Variable
SizeSize– Superficial Cortical LocationSuperficial Cortical Location– T1: Low/Mixed Signal,T1: Low/Mixed Signal,– T2: High/Mixed SignalT2: High/Mixed Signal– Often with Cystic ComponentOften with Cystic Component– Solid Portion Intensely EnhancesSolid Portion Intensely Enhances– Adjacent Meninges May Enhance (Tail)Adjacent Meninges May Enhance (Tail)– Little or No Mass EffectLittle or No Mass Effect
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ASTROCYTOMASASTROCYTOMAS
““ORDINARY” ASTROCYTOMAORDINARY” ASTROCYTOMADiffuse Infiltration of WM:Diffuse Infiltration of WM:
FibrillaryFibrillary ProtoplasmicProtoplasmic GemistocyticGemistocytic
WHO 2,3,4 (NOT 1)WHO 2,3,4 (NOT 1) KS & Mayo Grades 1-4KS & Mayo Grades 1-4
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PATTERN ANALYSISPATTERN ANALYSISNeoplasmNeoplasm
Benign(G rade 1-2)(Fibrillary)
Anaplastic(G rade 2)
G lioblastom a(G rade 4)
DiffuseAstrocytom a
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KERNOHAN (KS)KERNOHAN (KS) 11 2 2 3 3 4 4
ANAPLASIAANAPLASIA 0 0 MinMin >1/2 Marked >1/2 Marked
CELLULARITY CELLULARITY Mild Mild Mild IncMild Inc Marked Marked
MITOSISMITOSIS 0 0 0 0 Plus Marked Plus Marked
ENDOTHELIALENDOTHELIAL 0 Min0 Min Min Min Marked Marked
NECROSISNECROSIS Marked Marked
TRANSITIONTRANSITION<== Broad <== Broad Sharp ==> Sharp ==>
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ST. ANNE-MAYOST. ANNE-MAYO (1-3) (1-3) 1 Point for 1 Point for Each Each
ATYPIAATYPIA
MITOSISMITOSIS
ENDOTHELIALENDOTHELIAL
NECROSISNECROSIS
Grade = TOTAL POINTSGrade = TOTAL POINTS
Grade 1 = 0-1, Grade 2 = 2 pointsGrade 1 = 0-1, Grade 2 = 2 points
Grade 3 = 3,4 pointsGrade 3 = 3,4 points
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ASTROCYTOMA:ASTROCYTOMA:DIFFUSEDIFFUSE
(Fibrillary, protoplasmic, etc.)(Fibrillary, protoplasmic, etc.)
““Adult type” or “Hemispheric” Adult type” or “Hemispheric” AstrocytomaAstrocytoma
Diffusely infiltrate brain, along WM tractsDiffusely infiltrate brain, along WM tracts
Continuum, from low-grade to high-gradeContinuum, from low-grade to high-grade
Genetic Alterations 17 => 9 => 10Genetic Alterations 17 => 9 => 10
Many Progress in Histology over time, Many Progress in Histology over time, changing from WHO Gr. 2 => Gr. 3 => changing from WHO Gr. 2 => Gr. 3 => Gr. 4 (GBM)Gr. 4 (GBM)
Imaging tends to correlate with histologyImaging tends to correlate with histology
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ASTROCYTOMAASTROCYTOMARadiologic GradingRadiologic Grading
TYPE 1 - (Benign, WHO 2 KS Grade 1-TYPE 1 - (Benign, WHO 2 KS Grade 1-2)2)– HomogeneousHomogeneous– No Enhancement, No EdemaNo Enhancement, No Edema
TYPE 2 - (Anaplastic - Grade 3)TYPE 2 - (Anaplastic - Grade 3)– Variable Enhancement, EdemaVariable Enhancement, Edema
TYPE 3 - (Glioblastoma - Grade 4)TYPE 3 - (Glioblastoma - Grade 4)– Heterogeneous (Necrosis, Blood)Heterogeneous (Necrosis, Blood)– Ring Enhancement, EdemaRing Enhancement, Edema
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““BENIGN” BENIGN” ASTROCYTOMA:ASTROCYTOMA:
WHO 2, KS 1-2, Mayo 1WHO 2, KS 1-2, Mayo 1 YOUNGER PATIENTYOUNGER PATIENT
– CHILDHOODCHILDHOOD– Young Adults (20’s - 40’s)Young Adults (20’s - 40’s)
NL VESSELS (NO NL VESSELS (NO NEOVASCULARITY)NEOVASCULARITY)– BBB INTACTBBB INTACT– NO EDEMANO EDEMA– NO ENHANCEMENTNO ENHANCEMENT– NO TUMOR VESSELS NO TUMOR VESSELS
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Benign - DiffuseBenign - Diffuse
HOMOGENEOUSHOMOGENEOUS– NO NECROSISNO NECROSIS– NO HEMORRHAGENO HEMORRHAGE– INCREASED WATERINCREASED WATER
DARK Poorly Demarcated on CTDARK Poorly Demarcated on CT
Sharp and Dark on T1W Sharp and Dark on T1W
Sharp and BRIGHT on T2WSharp and BRIGHT on T2W– MICROCYST >>> MACROCYSTMICROCYST >>> MACROCYST
(macrocysts occur in JPA, etc.)(macrocysts occur in JPA, etc.)
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MODES OF SPREADMODES OF SPREAD
1. Natural passages1. Natural passages
2. Along surfaces2. Along surfaces
3. Along tracts3. Along tracts
4. Across the meninges4. Across the meninges
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SPREAD ALONG SPREAD ALONG TRACTS:TRACTS:
CORONA RADIATACORONA RADIATA PEDUNCLESPEDUNCLES CORPUS CALLOSUMCORPUS CALLOSUM ANTERIOR COMMISUREANTERIOR COMMISURE ARCUATE FIBRESARCUATE FIBRES
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““MALIGNANT” MALIGNANT” ASTROCYTOMA:ASTROCYTOMA: Older patientOlder patient
– 40’s and up40’s and up– exceptions (PNET)exceptions (PNET)– ~ 1/2 arise from previous low grade (2-3)~ 1/2 arise from previous low grade (2-3)
Abnl. Vessels (neovascularity)Abnl. Vessels (neovascularity) -- -- BBB abnormalityBBB abnormality– vasogenic edemavasogenic edema– contrast enhancementcontrast enhancement– irregular vessels, shunting, etc.irregular vessels, shunting, etc.
HETEROGENEOUSHETEROGENEOUS– hemorrhage (old/new)hemorrhage (old/new)– tumor necrosistumor necrosis– tumor itselftumor itself
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GBMGBM
LOW DENSITY CENTERLOW DENSITY CENTER– variegated necrosisvariegated necrosis
ENHANCING RIMENHANCING RIM– hypercellular, fleshy neoplasmhypercellular, fleshy neoplasm– greatest neovascularitygreatest neovascularity
CORONA OF HYPODENSITYCORONA OF HYPODENSITY– ““edematous” white matteredematous” white matter– areas of neoplastic infiltrationareas of neoplastic infiltration
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GLIOBLASTOMA GLIOBLASTOMA MULTIFORMEMULTIFORME
(Malignant Astrocytoma)(Malignant Astrocytoma)
Adults over 40 yrs.Adults over 40 yrs. Malignant with mitoses, Malignant with mitoses,
neovascularityneovascularity Discrete ring-enhancing lesionDiscrete ring-enhancing lesion Central necrosis, vasogenic edemaCentral necrosis, vasogenic edema Cerebral hemispheresCerebral hemispheres
(cross the corpus callosum)(cross the corpus callosum)
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GLIAL TUMORSGLIAL TUMORSMR Grading*MR Grading*
Low Grade (KS 1-2)Low Grade (KS 1-2) High Grade (KS 3- High Grade (KS 3-4)4)
HomogeneousHomogeneous HeterogeneousHeterogeneous
Well definedWell defined Poorly definedPoorly defined
Min. MassMin. Mass More MassMore Mass
Min. Edema Min. Edema Vasogenic edemaVasogenic edema
No blood No blood HemosiderinHemosiderin
*Radiology (1990) 174: 411-415*Radiology (1990) 174: 411-415
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GRADING SYSTEMSGRADING SYSTEMSSem Rad Onc (1991); 1: 2-9Sem Rad Onc (1991); 1: 2-9
KernohanKernohan BergerBerger WHOWHO1 1
Pilocytic,SEGAPilocytic,SEGA
Benign (1)Benign (1) AstrocytomaAstrocytoma
2 Astrocytoma2 Astrocytoma
Benign (2)Benign (2)
AnaplasticAnaplastic 3 Anaplastic3 Anaplastic
Anaplastic (3)Anaplastic (3)
GlioblastomaGlioblastoma 4 Glioblastoma4 Glioblastoma
Malignant (4)Malignant (4)
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NEUROEPITHELIAL NEUROEPITHELIAL TUMORSTUMORS
WHO ClassificationWHO Classification AstrocyticAstrocytic OligodendroglialOligodendroglial EpendymalEpendymal Choroid Plexus TumorsChoroid Plexus Tumors NeuronalNeuronal Neuronal Mixed w/ GlialNeuronal Mixed w/ Glial PinealPineal Embryonal (PNET)Embryonal (PNET)
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NEOPLASMS OF THE NEOPLASMS OF THE MENINGESMENINGES
WHO ClassificationWHO Classification
1. MENINGIOMA:1. MENINGIOMA:
a. Meningioma (typical)a. Meningioma (typical)
b. Atypical Meningiomab. Atypical Meningioma
c. Anaplastic (Malignant) c. Anaplastic (Malignant) MeningiomaMeningioma
2. MESENCHYMAL (non-meningothelial)2. MESENCHYMAL (non-meningothelial)
3. Primary MELANOCYTIC Lesions3. Primary MELANOCYTIC Lesions
4. UNCERTAIN Origin4. UNCERTAIN Origin
a. Hemangiopericytomaa. Hemangiopericytoma
b. Hemangioblastomab. Hemangioblastoma
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MENINGEAL TUMORSMENINGEAL TUMORSWHO GradesWHO Grades
TYPETYPE GRADEGRADE
MENINGIOMAMENINGIOMA I I
ATYPICAL MENINGIOMAATYPICAL MENINGIOMA II II
PAPILLARY MENINGIOMAPAPILLARY MENINGIOMA II-IIIII-III
HEMANGIOPERICYTOMAHEMANGIOPERICYTOMA II-IIIII-III
ANAPLASTIC MENINGIOMAANAPLASTIC MENINGIOMA III III
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MENINGIOMAMENINGIOMA“Malignant Meningioma”“Malignant Meningioma”
Hemangio-Peri-Cytoma (HPC)Hemangio-Peri-Cytoma (HPC) Malignant Fibrous Histiocytoma Malignant Fibrous Histiocytoma
(MFH)(MFH) Papillary MeningiomaPapillary Meningioma ““Benign” Metastasizing MeningiomaBenign” Metastasizing Meningioma
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HEMANGIOPERICYTOMAHEMANGIOPERICYTOMA(HPC)(HPC)
Narrow dural baseNarrow dural base
(“Mushrooming”)(“Mushrooming”) No Hyperostosis, No No Hyperostosis, No
CalcificationCalcification Lobulated (not hemispheric)Lobulated (not hemispheric) Internal Signal Voids (on Internal Signal Voids (on
MRI)MRI) Hypervascular on AngioHypervascular on Angio
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DYSEMBRYOPLASTIC DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOR (DNT)NEUROEPITHELIAL TUMOR (DNT)
IMAGING:IMAGING:– MR APPEARANCEMR APPEARANCE
Focal cortical mass, usually temporal Focal cortical mass, usually temporal lobelobe
Hypointense on T1Hypointense on T1 Hyperintense on T2Hyperintense on T2 MultinodularMultinodular
– MicrocysticMicrocystic– Megagyric - may cause bony erosionMegagyric - may cause bony erosion
Occasional EnhancementOccasional Enhancement
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DYSEMBRYOPLASTIC DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOR (DNT)NEUROEPITHELIAL TUMOR (DNT)
IMAGING:IMAGING:– CT APPEARANCECT APPEARANCE
Hypodense MassHypodense Mass No EdemaNo Edema Rare CalcificationRare Calcification Calvarial ErosionCalvarial Erosion CT Normal in 10%CT Normal in 10%
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THE NEW WHOTHE NEW WHO
Biological PotentialBiological Potential
Ascending Scale of Ascending Scale of AggressionAggression
I - IVI - IV
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ASTROCYTOMAASTROCYTOMAFive Year SurvivalFive Year Survival
0 10 20 30 40 50 60 70 80 90 100
Percent Survival
Glioblastoma (IV)
Anaplastic (III)
Astrocytoma (I-II)
Pilocytic
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CORRELATIONCORRELATION
Low GradeLow Grade
Long-Term Long-Term SurvivalSurvival
Stable HistologyStable Histology
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WHO GRADE IWHO GRADE I
Circumscribed Circumscribed AstrocytomaAstrocytoma– JPA (Pilocytic)JPA (Pilocytic)– SGCA (Subependymal)SGCA (Subependymal)
GangliogliomaGanglioglioma MeningiomaMeningioma
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Define the Problem:Define the Problem:
Some Low Grade EnhanceSome Low Grade Enhance Some Low Grade Do Not Some Low Grade Do Not
Some Low Grade => GBMSome Low Grade => GBM Some Low Grade Do NotSome Low Grade Do Not
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Enhancement vs. TypeEnhancement vs. TypeCircumscribed Diff use
NoEnhancement
Astrocytoma (2)
VariableEnhancement
Anaplastic (3)
RoutineEnhancement
Pilocytic (1)SEGCA(1)PXA (2- 3)
GBM (4)
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