intraaxial masses - astrocytoma - smirniotopoulos (afip 2008)

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Intraaxial Masses - Astrocytoma - Smirniotopoulos (AFIP 2008)

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USU Learning to Care for Those in Harms Way

Intraaxial Masses: Approach to AstrocytomaJames G. Smirniotopoulos, M.D. Uniformed Services University of the Health Sciences Bethesda, MD You are Here!*

First StepsRadiology - http://rad.usuhs.edu

Favor

Neoplasm

Primary Solitary Deep Large lesions +/+/- vasogenic edema

Secondary Multiple Subcortical Small lesion lots of vasogenic edema

Visit us at: http://rad.usuhs.edu http://rad.usuhs.edu

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

Primary vs. SecondaryRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.eduCourtesy of Paul Sherman Courtesy of R.D. Zimmerman

PRIMARY NEOPLASMS Neuroectoderm

Neuroectodermal

Radiology - http://rad.usuhs.edu

Embryologic Neural Tube Neuroepithelial Neuroepithelial Broad

Categories

Remember! 45 55% of metastasis present as a solitary lesion even on MR

Glial Tumors (Gliomas) Embryonal/Immature (P.N.E.T. (P.N.E.T.s) Neuronal (Neurocytoma) Mixed (Ganglioglioma)

USU Learning to Care for Those in Harms Way

WHO 1 vs. WHO 4 Some Some

Define the Problem:Low Grade Enhance Low Grade Do Not

Some

Low Grade => GBM Some Low Grade Do Not

1

Modified from Sem Rad Onc (1991); 1: 22-9 KernohanBenign (1) Benign (2) Anaplastic Anaplastic (3) 3 Anaplastic, PXA

GRADING SYSTEMSBergerAstrocytoma

WHO 19931993-20071 Pilocytic,SEGA 2 Astrocytoma, PXA

WHO Classification Defines

Histologic Subtypes Grades Biologic Potential Allows International Cooperation Ascending scale of Aggression from 11-4

Glioblastoma (4)

Glioblastoma

4 Glioblastoma

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WHO CORRELATIONRadiology - http://rad.usuhs.edu

Lesion Aggression and Grading

Low

Grade Grade 1Possible Surgical Cure

LongLong-Term Survival Stable Histology no progression

Blood volume Blood flow MTT Choline/Creatine MyoInositol Lip/Lac and NAA

Perfusion Imaging: CT, MR, Angio

MR Spectroscopy

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

AstrocytomaRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu Astrocytoma WHO 3-4 Infiltrating and Focal Heterogeneous Mass effect Enhancement Necrosis PWI Cho/Cr FDG Lac/Lip

AstrocytomaAstrocytoma WHO 3-4 Infiltrating and Focal Heterogeneous Mass effect Enhancement Necrosis PWI Cho/Cr FDG Lac/Lip

WHO 1 Circumscribed Enhancing Fluid secreting

WHO 2-3 Infiltrating Water signal Mild to Mod. Mass No enhancement

WHO 1 Circumscribed Enhancing Fluid secreting

WHO 2-3 Infiltrating Water signal Mild to Mod. Mass No enhancement

Location Cyst w/ Nodule

MyoInositol Perfusion

Pilocytic Subependymal GC

MyoInositol Perfusion

Increasing Aggression

Cellularity DWI matching ADC Mitoses Endothelial proliferation Metabolism PET FDG or Th SPECT

2

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AstrocytomaRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu Astrocytoma WHO 3-4 Infiltrating and Focal Heterogeneous Mass effect Enhancement Necrosis PWI Cho/Cr FDG Lac/Lip

Benign Benign ASTROCYTOMA Two

WHO 1 Circumscribed Enhancing Fluid secreting

WHO 2-3 Infiltrating Water signal Mild to Mod. Mass No enhancement

Pilocytic Subependymal GC

MyoInositol Perfusion

types Low grade benign benign Diffuse in Adults WHO Grade 2 Low grade special special Circumscribed in Children WHO Grade 1

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

Major ConceptRadiology - http://rad.usuhs.edu

Major ConceptGliomaAstrocyte

GliomaAstrocyte

CircumscribedPushing Margin

DiffuseInfiltrating Margin24

CircumscribedWHO 1 Pilocytic Subependymal Giant Cell

DiffuseWHO 2 - Astrocytoma WHO 3 Anaplastic A. WHO 4 - Glioblastoma

WHO 2-3 Pleomorphic Xantho

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

What can we Dx? Dx?Radiology - http://rad.usuhs.edu

Circumscribed AstrocytomaPilocytic Astrocytoma SEGA WHO Grade 1 Circumscribed Astrocytoma

Circumscribed

Astrocytoma

WHO Gr 1 Pilocytic Astrocytoma WHO Gr 1 Subependymal Giant Cell Astro. Astro. WHO 2,3 Pleomorphic Xanthoastrocytoma Diffuse

Astrocytoma

WHO Gr 2 ( (Astrocytoma Astrocytoma) WHO Gr 3 ( (Anaplastic Astrocytoma Astrocytoma) WHO Gr 4 ( (Glioblastoma Multiforme Multiforme - GBM)

3

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

AstrocytomaRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu Astrocytoma WHO 3-4 Infiltrating and Focal Heterogeneous Mass effect Enhancement Necrosis PWI Cho/Cr FDG Lac/Lip

WHO Gr1 - Pilocytic Astrocytoma

Pushing Margin

WHO 1 Circumscribed Enhancing Fluid secreting

WHO 2-3 Infiltrating Water signal Mild to Mod. Mass No enhancement

Circumscribed Mass: Fluid-secreting Cyst w/Nodule

Location Cyst w/ Nodule

MyoInositol Perfusion

ASTROCYTOMA:Circumscribed

PILOCYTIC ASTROCYTOMA

Special Special Astrocytomas Astrocytoma of Young Various Locations Well circumscribed (yet, no capsule) Do NOT spread along WM Do NOT change grade (except PXA) Constellation of findings correlates w/ Histology

Cystic Cerebellar Astrocytoma Juvenile Pilocytic Astrocytoma (PA PA or JPA JPA)

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PILOCYTIC ASTROCYTOMARadiology - http://rad.usuhs.edu

Pilocytic AstrocytomaCyst and Mural Nodule balanced morphology Wall may not enhance Cyst fluid has protein Not identical to CSF Nodule low density on CT may calcify up to 25% No increase in vascularity WHO Grade 1 Peak at ~10 yrs

Synonyms: Polar Spongioblastoma, Cystic Cerebellar Astrocytoma Cell of Origin: Astrocyte (bi(bi-polar, hairlike) Associations: in ON w/ NFNF-1 Incidence: 33-6% of ALL Cranial, 32% of Child Age: 5-15 (Zulch 3Sex: Slight F (11/9) 3-7) Location: Cerebellum, Chiasm/Hypothal, Optic Treatment: Surgery, patience Prognosis: 77% at 5 yrs, 75% at 10 yrs, 75% at 15 yrs

4

PILOCYTIC ASTROCYTOMARadiology Cerebellum, Majority

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Pilocytic AstrocytomaRadiology - http://rad.usuhs.edu

Diencephalon

rare in BS or Cerebrum

have significant cyst cyst

Cyst and Mural Nodule Nodule part of lining does NOT enhance

Nodule may be heterogeneous Exceptional purely solid Nodule Calcification

has increased water in 55-25%

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

Pilocytic AstrocytomaRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu

Cyst and Mural Nodule balanced morphology Wall may not enhance Cyst fluid has protein Not identical to CSF Nodule low density on CT may calcify up to 25% No increase in vascularity WHO Grade 1 Peak at ~10 yrs

Cyst

Nodule

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

Cyst with NoduleRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu

WHO Gr1 Pilocytic Astrocytoma

Pushing Margin Circumscribed Mass: Cyst w/Nodule

5

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PATHOLOGY Biphasic

Pilocytic AstrocytomaRadiology - http://rad.usuhs.edu

pattern

dense pilocytic glia Rosenthal fibers loose microcystic areas No

Variant Variant

Appearance Location

necrosis Low grade Abnormal capillaries allow enhancement fluid production

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Pilocytic AstrocytomaA Cyst with mural nodule? Neoplasm + thin rim of enhancing gliosis

USU Learning to Care for Those in Harms Way

Pilocytic AstrocytomaRadiology - http://rad.usuhs.edu

Radiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu

Not Always !!!

Courtesy of Paul Sherman

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

Pilocytic AstrocytomaAtypical: Dense Ca++, No Cyst, No Enhancement Radiology - http://rad.usuhs.edu

Courtesy of Paul Sherman

6

PILOCYTIC ASTROCYTOMA: Locations CEREBELLUM Chiasm

USU Learning to Care for Those in Harms Way

Radiology - http://rad.usuhs.edu

And Optic NerveDiencephalon

Hypothalmus/thalamus Cerebral Spinal

Hemisphere Cord (Intramedullary)

USU Learning to Care for Those in Harms Way

PILOCYTIC ASTROCYTOMA(Juvenile Pilocytic) Childhood,

Radiology - http://rad.usuhs.edu

Young Adults Benign, no mitosis/necrosis Circumscribed - Enhancing Cyst Formation, Mural Nodule Cerebellum and Diencephalon (Optic tracts, Hypothalmus)

USU Learning to Care for Those in Harms Way

WHO GRADE 1 TumorsRadiology - http://rad.usuhs.edu

Subependymal Giant Cell Astro

Circumscribed

Astrocytoma

JPA (Pilocytic) SGCA (Subependymal Giant Cell) Ganglioglioma Meningioma

85-95% associated with Tuberous Sclerosis

7

USU Learning to Care for Those in Harms Way

ASTROCYTOMASRadiology - http://rad.usuhs.edu

Circumscribed AstrocytomaCircumscribed Astrocytoma PXA WHO Grade 1 2 PXA 3 4

SPECIAL SPECIAL

ASTROCYTOMAS Circumscribed Growth: Pilocytic Subependymal Giant Cell Pleomorphic XanthoXantho-Astrocytoma

PLEOMORPHIC XANTHOASTROCYTOMA Rare

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Pleomorphic XanthoastrocytomaRadiology - http://rad.usuhs.edu

Variant of Astrocytoma from Subpial Astrocytes Affects Superficial Cerebral Cortex and Meninges Skull erosion (scalloped excavation) Temporal > Frontal > Parietal WHO Grade 2,3 50% progress over time Arises

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Ordinary AstrocytomasRadiology - http://rad.usuhs.edu

Astrocytes GFAP Stain

Diffuse

Infiltration of WM by: Fibrillary Astrocytes Protoplasmic Astrocytes Gemistocytic Astrocytes 2,3,4 (NOT 1)

WHO KS

& Mayo Grades 11-4

8

USU Learning to Care for Those in Harms Way

Normal appearing white matter few cell bodies

USU Learning to Care for Those in Harms Way

Diffuse Grade 2 Astrocytoma too many cells !

Radiology - http://rad.usuhs.edu USU Learning to Care for Those in Harms Way

Radiology - http://rad.usuhs.edu

Infiltrating Margin

Diffuse Mass Non-destructive Infiltration

AstrocytomaRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu Astrocytoma WHO 2-3 Infiltrating Water signal Homogeneous Mild Mass effect No enhancement No Necrosis WHO 3-4 Infiltrating and Focal Heterogeneous Mass effect Enhancement Necrosis PWI Cho/Cr FDG Lac/Lip

Daddy, Where do Glioblastomas come from?Progressive Transformation from lower grade diffuse astrocytoma - OR Arise de novo

WHO 1 Circumscribed Enhancing Fluid secreting

Pilocytic Subependymal GC

MyoInositol Perfusion

USU Learning to Care for Those in Harms Way

Astrocyte MutationGenetically Heterogeneous

USU Learning to Care for Those in Harms Way

Diffuse AstrocytomaRadiology - http://rad.usuhs.edu

Normal

Astrocyte Neoplastic Anaplastic GBMNormal WHO Gr 2Deletions: 17 p P53 22q NF2

WHO GR 4 WHO GR 3 WHO GR 2 Normal

Gr 3Deletions: 13q RB 19q 10

Gr 4 = GBMDeletions: 10 PTEN/MMAC1 9p P16,P15.P14

Excess production of: PDGF

Excess Excess production of: production of: EGFR CDK4 VEGF

9

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

A spectrum of of Pathology A spectrum tumorsRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu

Diffuse Astrocytoma too many cells !

Astrocytoma

Anaplastic Astrocytoma

Glioblastoma Multiforme

WHO 2

WHO 3

WHO 4

Mild cellular atypia

USU Learning to Care for Those in Harms Way

Glioblastoma multiforme too many cells, Atypia, Mitoses, Vessels, and Necrosis!

ASTROCYTOMARadiologic Grading TYPE

1 - WHO 2, KS Grade 11-2, Benign Benign

Homogeneous No Enhancement, No Vasogenic Edema TYPE

2 WHO Grade 3, Anaplastic

Variable Enhancement, Edema 50% enhance - 50% don dont TYPE

3 WHO Grade 4 Glioblastoma

Heterogeneous (Necrosis, Blood) Ring Enhancement, Edema

BENIGN ASTROCYTOMA: WHO 2, KS 1-2, Mayo 1 YOUNGER

Benign - Diffuse

PATIENT

CHILDHOOD Young Adults (20 (20s - 40 40s) NL

HOMOGENEOUS NO NECROSIS NO HEMORRHAGE INCREASED WATER DARK and Poorly Demarcated on CT Dark and Sharp on T1W BRIGHT and Sharp on T2W MICROCYST >>> MACROCYST (macrocysts occur in JPA, etc.)

VESSELS (NO NEOVASCULARITY)

BBB INTACT NO EDEMA NO ENHANCEMENT NO TUMOR VESSELS

10

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Gr 2 Fibrillary Astrocytoma

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Gr 2 Fibrillary AstrocytomaRadiology - http://rad.usuhs.edu

Radiology - http://rad.usuhs.edu

PD

T2

T1-non

T1-gad T2

USU Learning to Care for Those in Harms Way

Gliomatosis Cerebri

USU Learning to Care for Those in Harms Way

Astrocytoma: Microcystic changeRadiology - http://rad.usuhs.edu

Radiology - http://rad.usuhs.edu

T2

T1-gad

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Gliomatosis Cerebri: Diffuse Astrocytoma 2 lobes

USU Learning to Care for Those in Harms Way

Spread along White Matter TractsRadiology - http://rad.usuhs.edu

Radiology - http://rad.usuhs.edu

11

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

Gliomatosis CerebriRadiology - http://rad.usuhs.edu USU Learning to Care for Those in Harms Way Radiology - http://rad.usuhs.edu

Gliomatosis CerebriCHO Cr

NAA ?

{

USU Learning to Care for Those in Harms Way

MRS for the Complete IdiotRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu

MRS Hypothetical NeoplasmCho CR MI NAA Lipid Lactate

NAA CR Cho MI Glx Lipid Lactate

4

3

2

1 ppm

4

3

2

1 ppm

Hunters Angle

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

Diffuse AstrocytomaRadiology - http://rad.usuhs.edu

12

USU Learning to Care for Those in Harms Way

SPREAD ALONG WM TRACTS:Radiology - http://rad.usuhs.edu

Pontine Astrocytoma

Corona Corpus

Radiata

Peduncles

Callosum Commissure Arcuate Fibres Anterior

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USU Learning to Care for Those in Harms Way

Pontine Astrocytoma: WHO 2Radiology - http://rad.usuhs.edu USU Learning to Care for Those in Harms Way Radiology - http://rad.usuhs.edu

WHO 2 => GBM

Six Weeks Later

Expanded BrainRadiology - http://rad.usuhs.edu

Anaplastic Astrocytoma

Tracking Along WM Uncinate Fasciculus

13

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

Grade 3: Anaplastic AstrocytomaAstrocytoma WHO 2-3 Infiltrating Water signal Homogeneous Mild Mass effect No enhancement No Necrosis WHO 3-4 Infiltrating and Focal Heterogeneous Mass effect Enhancement Necrosis PWI Cho/Cr FDG Lac/Lip

WHO 1 Circumscribed Enhancing Fluid secreting

Grade III malignant glioma Less aggressive than GBM, malignant with somewhat better prognosis Frequency: highest in young adults (30 40 years) Recurrence: often as a higherhigher-grade glioma Challenge: difficult to remove completely with surgery Median survival: 3 4 years

Anaplastic Astrocytoma: Overall Characteristics

Radiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu

Radiology - http://rad.usuhs.edu

Pilocytic Subependymal GC

MyoInositol Perfusion

Anaplastic Astrocytoma: Cells, Vessels, but no necrosis

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Anaplastic AstrocytomaRadiology - http://rad.usuhs.edu

Enhancement But no necrosis

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

Anaplastic AstrocytomaNo Enhancement Abnl MRSRadiology - http://rad.usuhs.edu

Anaplastic Astrocytoma

14

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

Anaplastic Astrocytoma ( WHO 3 )

Increased Cellularity, +/+/- minimal vascular changes, no necrosis , no hemorrhage

Radiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu

Radiology - http://rad.usuhs.edu

No Enhancement Clear Mass Effect

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

AstrocytomaRadiology - http://rad.usuhs.edu Astrocytoma WHO 2-3 Infiltrating Water signal Homogeneous Mild Mass effect No enhancement No Necrosis WHO 3-4 Infiltrating and Focal Heterogeneous Mass effect Enhancement Necrosis PWI Cho/Cr FDG Lac/Lip

Glioblastoma

WHO 1 Circumscribed Enhancing Fluid secreting

Pilocytic Subependymal GC

MyoInositol Perfusion

Older patient

MALIGNANT MALIGNANT ASTROCYTOMA:

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Glioblastoma multiformeRadiology - http://rad.usuhs.edu

40 40s and up exceptions (PNET) ~ 1/2 arise from previous low grade (2(2-3)

Abnormal Vessels (neovascularity) - BBB abnormality vasogenic edema contrast enhancement irregular vessels, shunting, etc.

HETEROGENEOUS hemorrhage (old/new) tumor necrosis tumor itself

15

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USU Learning to Care for Those in Harms Way

Glioblastoma multiformeRadiology - http://rad.usuhs.edu USU Learning to Care for Those in Harms Way Radiology - http://rad.usuhs.edu

Glioblastoma Multiforme

USU Learning to Care for Those in Harms Way

Glioblastoma WHO Grade 4Radiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu

Glioblastoma Multiforme

A solitary, deep, irregular, heterogenous, ringenhancing mass with vasogenic edema.

Low NAA High Choline/Creatine

T1-gad

T2

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

(Gr 4) Glioblastoma: PWI-CBVRadiology - http://rad.usuhs.edu

MR Perfusion Imaging

*

*

Increased perfusion

Courtesy of James Provenzale, Duke University

16

USU Learning to Care for Those in Harms Way

USU Learning to Care for Those in Harms Way

CT PerfusionRadiology - http://rad.usuhs.edu USU Learning to Care for Those in Harms Way Radiology - http://rad.usuhs.edu

X-Ray Perfusion Imaging

Hypervascularity means rCBV and rCBF

Early Draining Veins mean Short MTT

USU Learning to Care for Those in Harms Way

GBM Thicker on SurfaceRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu

Two Different GBM GBMs

USU Learning to Care for Those in Harms Way

Center

of Abnl Density/Intensity RIM

GBMRadiology - http://rad.usuhs.edu

GBM - Glioblastoma

variegated necrosis ENHANCING

hypercellular, fleshy neoplasm greatest neovascularity Corona

of Abnl Signal

edematous edematous white matter areas of microscopic neoplastic infiltration

17

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USU Learning to Care for Those in Harms Way

Pseudopalisading NecrosisRadiology - http://rad.usuhs.edu Radiology - http://rad.usuhs.edu

18