GLIAL AND GLIO- NEURONAL TUMORS - Pathology€¦ · GLIAL AND GLIO-NEURONAL TUMORS Arie Perry, M.D....
Transcript of GLIAL AND GLIO- NEURONAL TUMORS - Pathology€¦ · GLIAL AND GLIO-NEURONAL TUMORS Arie Perry, M.D....
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GLIAL AND GLIO-NEURONAL TUMORS
Arie Perry, M.D.
School of Medicine
DISCLOSURES (Arie Perry, MD)
• I have no financial relationships to disclose.
- and -
• I will not discuss off label use or investigational use in my presentation
School of Medicine
NEUROPATHOLOGY AND REAL ESTATE
• Location
• Location
• Location
• Patient Age
• Neuroimaging
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School of Medicine
GLIOMAS
• Astrocytomas (A)
• Oligodendrogliomas (O)
• Mixed oligoastrocytomas (MOA)?
• Ependymomas
• Diffuse glioma = A, O, or MOA
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GLIOMA GRADING: WHO 2016
• Grade I = Benign
• Grade II = Low-grade
• Grade III = “Anaplastic”
• Grade IV = High-grade malignant, e.g. “GBM”
Distribution of Malignant Primary Brain and CNS Tumors by CBTRUS Histology Groupings and Histology (N = 117,023), CBTRUS Statistical Report: NPCR and SEER, 2008-2012.
Quinn T. Ostrom et al. Neuro Oncol 2015;17:iv1-iv62
© The Centers for Disease Control. Published by Oxford University Press on behalf of the Society for Neuro-Oncology in cooperation with the Central Brain Tumor Registry 2015
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ASTROCYTOMAS
• Diffuse (75%)
– Fibrillary
– Gemistocytic
– Giant Cell
– Small Cell
– Granular Cell
– Epithelioid
• Circumscribed / Favorable (25%)
– Pilocytic
– PXA
– SEGA
– DIA
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ASTROCYTOMA, IDHm (WHO GRADE II)
• Age 30-40
• Insidious / Slow growing
• Non-enhancing
• Frequent progression to grades III or IV
• Survival ~5-8 years
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DIFFUSE ASTROCYTOMA (WHO II)
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ASTROCYTOMA (WHO GRADE II)
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DIFFUSE ASTROCYTOMA (II)
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SECONDARY STRUCTURES OF SCHERER
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ANAPLASTIC ASTROCYTOMA, IDHm (WHO GRADE III)
• Age 40-50
• More rapid onset
• Frequent progression to grade IV
• Survival ~2-3 years
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ANAPLASTIC ASTROCYTOMA, WHO III
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GLIOBLASTOMA (GBM), WHO GRADE IV
• Age 50-60
• Rapid onset and progression
• Rim (or ring)-enhancing
• Survival ~1-year
• Primary form (IDHwt): ~90%
• Secondary form (IDHm): ~10%
17
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GLIOBLASTOMA (WHO GRADE IV)
18
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GLIOBLASTOMA (WHO GRADE IV)
19
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GBM VARIANTS / PATTERNS
• Fibrillary (Classic)
• Gemistocytic
• Giant Cell
• Gliosarcoma
• Adenoid / Epithelioid / Metaplastic
• Lipidized
• Inflammation-rich
• Granular Cell
• Small Cell
• GBM with primitive neuronal foci
• Epithelioid
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GIANT CELL GBM
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GFAP
GLIOSARCOMA
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GFAPReticulin
SMA CD34
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• Average age 30-40 years• Corticotropism / seizures common• Cerebral, especially frontal lobe• Slow progression• Survival ~10-20 years for oligo (grade
II); 5-10 years for anaplastic oligo (grade III): microvascular proliferation, high mitotic index, and/or necrosis
OLIGODENDROGLIOMA, IDHm and 1p19q-codel (WHO GRADE II or III)
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OLIGODENDROGLIOMA
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N
N
N
OLIGODENDROGLIOMA, WHO GRADE II
29
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OLIGODENDROGLIOMA
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GFAP
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ANAPLASTIC OLIGODENDROGLIOMA
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SYN
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1p321q42
19p1319q13
Oligodendroglioma, IDH-mutant and 1p/19q-codeleted (WHO 2016)
IDH1
PreneoplasticCell
IDHm
TP53mATRXm
IDHmTERTm
1p19q‐codel
IDHwtEGFR‐ampTERTm
9p (CDKN2A/B) LOH
PIK3CAm?
Astro, IDHm
AA, IDHm
GBM, IDHm
Oligo, IDHm, 1p19q‐codel
AO, IDHm, 1p19q‐codel
GBM, IDHwt
Diffuse midlineglioma, H3‐K27Mm
PIK3R1/PIK3CAm
CICmFUBP1m
4q LOH?
Note: no oligoastro!
(H3 G34R/V)
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PILOCYTIC ASTROCYTOMA
PILOCYTIC ASTROCYTOMA
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Nat Genet 45: 927-932, 2013
MAPKpathway
BRAFKIAA1549
PILOMYXOID ASTROCYTOMA, WHO?
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Komotar et al. Neurosurgery 54:72, 2004
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LONG-TERM EPILEPSY ASSOCIATED TUMORS (LEAT)
• Glioneuronal– Ganglioglioma (GG), WHO I
– Dysembryoplastic neuroepithelial tumor (DNET), WHO I
– Others: mixed GG/DNET, PGNT, EVN
• Gliomas– Pleomorphic xanthoastrocytoma (PXA), WHO II/III
– Diffuse LGGs, WHO II
– Pilocytic astrocytoma, WHO I
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GANGLIOGLIOMA (WHO I)
• Most common LEAT
• Children/young adults
• Temporal lobe
• Benign/surgically curable
• Anaplasia rare: definition?– WHO grade III (grade II eliminated in 2007)
• BRAF-V600E in up to half
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GANGLIOGLIOMA (WHO I)
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GANGLIOGLIOMA (WHO I)
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SYN NFP
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Neu-N
CD34
BRAF V600E
Ganglioglioma
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ANA GG, WHO GRADE III
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DNET (WHO I)
• LEAT of children/young adults
• Temporal lobe
• Benign/surgically curable
• Tumor vs. hamartoma
• Simple, complex, ‘non-specific’ variants
• BRAF-V600E: 0-50%; FGFR1-alt: 58%?, IDHwt, no 1p19q-codeletion
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GFAP
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PXA, WHO II-III
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PXA, WHO GRADE II
PXA, WHO GRADE II
PXA, WHO GRADE II
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Reticulin
PXA, WHO GRADE II
GFAP
PXA, WHO GRADE II
SYN NFP
PXA, WHO GRADE II
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CD34
PXA, WHO GRADE II
PXA c ANA transformation, WHO GRADE III
Ki-67
PXA c ANA transformation, WHO GRADE III
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BRAF-V600E
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EPENDYMOMA (WHO GRADE I, II, III)
• Kids: 4th ventricle, supratentorial
• Adults: spinal cord
• Prognostic Variables
– Extent of resection / location
– Patient age (poor if <2 years old)
– Histologic grade?
• Subependymoma or MPE, WHO grade I
• Ependymoma, WHO grade II
• Anaplastic ependymoma, WHO grade III
– Molecular subgroups
Subependymoma, WHO GRADE I
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MPE, WHO GRADE I
TrichromePAS
MPE, WHO GRADE I
NFP
Ependymoma, WHO GRADE II
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Ependymoma, WHO GRADE II
Ependymoma, WHO GRADE II
GFAP
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EMA CD99
Tanycytic ependymoma
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EPENDYMOMA: PROGNOSIS
Tihan T, et al., Mod Pathol 2008: 21, 165–177
Pajtler et al., 2015, Cancer Cell 27, 728–743
Clear cell ependymoma
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Clear cell ependymoma
L1CAM
Clear cell (RELA fusion+) ependymoma
UCSF 500 NGS
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H3K27me3
PF-A Ependymoma PF-B Ependymoma
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Neuropathology CD Recording
• Neuropathology songs
• Common disorders
• 2nd Year medical students
• Memorization aid
• Fun way to learn!
www.neuropathsongs.comiTunes or amazon.com
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School of Medicine
O----ligodendroglio-oh-oh-ma, diffuse cerebral tumor of adultsInvading cortex, causing epilepsy; on imaging, often you are calcifiedAnd although, you tend to progress over time, for long periods your fineYou're famous for your rounded nuclei, Clear haloes look like honeycombs or fried eggsWith branching chicken wire capillaries, and perineuronal satellitosis
O----ligodendroglio-oh-oh-ma, genetically, you are quite uniqueWith 1p and 19q deletions, from translocation with loss of one derivativeRepresents a genetically favorable set, when FISH criteria are met
Anaplastic cases grow more rapidly, assigned a W.H.O. grade IIIWith microvascular proliferation, or increased mitotic activityO----ligodendroglio-oh-oh-ma
OligodendrogliomaMusic to Ave Maria by F. Schubert, Lyrics by Arie Perry, MD