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CNS > Malignant > Flashcards

Malignant Flashcards

1
Q

Most common primary CNS malignancy in adults

A

Glioblastoma, IDH wild type (CNS WHO grade 4)
Also H3-wildtype

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2
Q

What are the secondary structures of scherer

A

Perineuronal satellitosis
Subpial spread
Perivascular accumulation
Growth along white matter tracts

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3
Q

To diagnose glioblastoma you should have at least one of the following

A

Microvascular proliferation
Pseudopalisading necrosis
Polysomy 7 and monosomy 10 (+7/-10)
EGFR gene amplification
TERT promoter mutation

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4
Q

This is a diffusely infiltrating astrocytic neoplasm with a mutation, and either I DH1 or I DH2 and intact chromosomes 1p and 19q
IHC

A

Astrocytoma, IDH mutant (CNS WHO grade 2-4)
IDH1 R132H mutation
ATRX loss and TP53 mutant

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5
Q

Differentiate grades 2 to 4 of astrocytoma

A

G2: lack significant mitotic activity. No CDKN2A/B homozygous deletion
G3: brisk mitotic activity. No CDKN2A/B homozygous deletion
G4: at least has 1 of the ff:
MVP
Pseudopalisading necrosis
CDKN2A/B homozygous deletion

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6
Q

This is a diffusely infiltrating glial neoplasm with monomorphic round nuclei with nuclear halos. They’re also microcalcifications and cystic degeneration. Also seen is chicken wire vasculature
IHC
Grades

A

Oligodendroglioma, IDH- mutant and 1p/19q co-deleted (CNS WHO Grade 2-3)
Wild type TP53 and ATRX
+ Olig2, variable GFAP

G2: low mitotic activity. No MVP or necrosis
G3: at least one of the ff: brisk mitotic activity, MVP, pseudopalisading necrosis

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7
Q

A tumor in the brainsten of a five year old male was seen. It consists of infiltrating tumor cells with small and monomorphic nuclei with some mitoses.
(+) H3K27M, Olig2, GFAP
Dx

A

Diffuse midline glioma, H3K27-altered (CNS WHO Grade 4)
Poor prognosis
Sometimes with tp53 and atrx mutation

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8
Q

It is the most common glioma in children
And can be seen in NF1 syndrome
Dx
Morpho
Molecular

A

Pilocytic astrocytoma (CNS, WHO, grade 1)
It is biphasic with compact zone containing bipolar piloid cells and Rosenthal fibers, and a loose zone with microcysts, myxoid background, and eosinophilic granular bodies (EGBs)
May have MVP but not aggressive
+ gfap, olig2; low ki67
KIAA1549-BRAF fusion MC

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9
Q

Astrocytic tumor with large, pleomorphic, and frequently multinucleated spindled and lipidized cells. Dense reticulin network, EGBs and perivascular lymphocytes.
Dx
Mc location molecular
Grades

A

Pleomorphic xanthoastrocytoma (CNS WHO Grade 2-3)
Temporal lobe
BRAF V600E and CDKN2A/B homozygous deletion
G2:<5 mitoses/10hpfs
G3: >5

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10
Q

This is a benign, slow growing, well circumscribed tumor, composed of large gemistocytic astrocytes with abundant pink glassy cytoplasm. Frequently has giant ganglion like cells with prominent nucleoli.
Diagnosis
Most common location
Association with syndromes

A

Subependymal giant cell astrocytoma (Grade 1)
Lateral ventricles
Tuberous sclerosis
Good prognosis

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11
Q

This is a circumscribed glial neoplasm with around, cuboidal, or columnar cells invariably papillary, or very vascular growth patterns with hyalinized blood vessels
+ GFAP, Olig2, EMA (diffuse, membranous, dor-like or ring-like)
Dx
Molecular

A

Astroblastoma, MN1-altered
MN1 alteration, MC fusion: MN1-Abend2 fusion

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12
Q

This is a well circumscribed tumor was clusters and cords of epithelial neoplastic glial cells. It is solid with variably mucinous stroma.
+TTF1, GFAP, CD34
Dx
Mc location
Molecular

A

Chordoid glioma (grade 2)
Third ventricle
PRKCA D463H mutation

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13
Q

Tumor composed of sheets of uniform, round cells with salt and pepper, chromatin, and perinuclear, halos and calcifications
+ synaptophysin

A

Central neurocytoma (grade 2)
Mc in lateral ventricles

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14
Q

It is a circumscribed glioma composed of uniform, small cells with round nuclei and speckled chromatin any Fibrillary matrix. There are perivascular pseudorosettes.
+ GFAP, EMA (perinuclear dot-like pattern)
Dx
Grade

A

Ependymoma (grade 2-3)
Grade based on anatomic location, histomorphology and molecular features

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15
Q

Type of ependymoma seen in NF2

A

Spinal ependymoma
Loss of chromosome 22
No MYCN amplification
(The other spinal ependymoma is MYCN-amplified and has poor prognosis)

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16
Q

CNS tumor with clusters of mostly nland cells embedded in abundant February matrix. There is frequent microcystic change.
+ GFAP and EMA (focal dot like pattern)

A

Subependymoma (grade 1)
4th and lateral ventricles

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17
Q

This is a tumor with delicate fiber, vascular fronds, covered by single layer of cuboidal to columnar epithelium. Mitosis is <2/10hpfs
+ CK7/-CK20
+S100

A

Choroid plexus papilloma (grade 1)
MC in lateral ventricles
Atypical CPP G2: >/=2/10hpfs mitosis, nuclear pleomorphism, solid growth, necrosis
Choroid plexus carcinoma, G3: at least 4 of the ff: >5/10hpf mitosis, increased cellular density, nuclear pleomorphism, blurring of papillary pttern with sheets, necrosis; assoc with Li-Frauneni syndrome (tp53 mutations)

18
Q

CNS tumor with densely packed small round blue cells and homer-wright rosettes with arrangement in patallel rows.
Reticulin: not significant
Dx
Molecular

A

Classic medulloblastoma
WNT-activated- childhood, APC mutation
Non-WNT/Non-SHH group 3- infancy/children
Non-WNT/Non-SHH group 4 (MC) - all age groups

19
Q

CNS tumor with pale nodules surrounded by densely, packed small round blue cells
pale nodules: low ki67, express neuronal markers
Internodular dark zones: high ki67, low expression of neuronal markers, reticulin-rich
Dx
Molecular

A

Desmoplastic/nodular medulloblastoma
SHH-activated
Tp53 wildtype
(Subgroups SHH1-4)

20
Q

CNS tumor with lobular architecture with elongated reticulin-free zones containing small neurocytic cells with a fibrillary bg
High ki67

A

Medulloblastoma with a extensive nodularity (MBEN)
SHH-activated
TP53- wildtype
Subgroups SHH1-4

21
Q

Subtype of Medulloblastoma with Marked nuclear pleomorphism and high mitotic index and numerous apoptotic bodies. Large cells have prominent nucleoli
Dx
Molecular

A

Large cell/anaplastic medulloblastoma
SHH activated
TP53 mutant
Subgroup SHH-3

22
Q

Medulloblastoma
Grade
Location
Ihc
Prognosis

A

Grade 4
4th ventricle or cerebellar parenchyma
Nonspecific neural markers
SHH-activated: +GAB1, lateral cerebellar hemisphere almost always this type
WNT-activated: +nuclear beta catenin
Desmoplasti and mben: +NeuN
Tp53 mutant: strong Tp53
Classic has excellent prognosis

23
Q

CNS neoplasm with a variable number of rhabdoid cells with eccentric nuclei, the vesicular chromatin, prominent nucleoli, abundant eosinophilic cytoplasm, numerous mitosis and geographic necrosis
Dx
Grade
Ihc

A

Atypical teratoid/rhabdoid tumor (grade 4)
(-) SMARCB1

24
Q

Sheet-like growth of uniform, small, mature cells with round nuclei and fine chromatin. There are also large rosettes.
+ synaptophysin, NSE, neurofilament
Ki67 <1%

A

Pineocytoma (grade 1)

Pineal parenchymal tumor of intermediate differentiation (PPTID, grade 2-3): increased cellularity, ki67 >5%

Pineoblastoma (grade 4)
Highly celular, patternless pattern of smal immature neuroepithelial cells
Ki67 >20%
Necrosis common

25
Q

This is the classic morphology of meningioma with whorls of syncytial epithelioid cells

A

Meningothelial

26
Q

This meningioma is the combination of meningothelial and fibrous histologies and is the most common type

A

Transitional

27
Q

These are considered grade 2 meningiomas

A

Chordoid and clear cell

28
Q

These are considered grade 3 meningiomaS

A

papillary and rhabdoid

29
Q

Most common intracranial tumor
Ihc
Grading

A

Meningioma
+ EMA, PR, SSTR2A

G2: 4-19 mitoses/10hpfs or brain invasion or choroid or clear cell, or at least 3 of the ff: high tumor cellularity, high n:c ratio (small cell change), prominent nucleoli, sheeting architecture, tumor necrosis

G3: >/= 20mf/hpf or anaplastic histology or rhabdoid/papillary or TERT promoter mutation or CDKN2A/B homozygous deletion

30
Q

SFT grading

A

G1: <5mf/10hpf
G2: >/=5 mf/10hpfs without necrosis
G3: >/=5 mf/10hpfs with necrosis

31
Q

CNS tumor with large stromal cells with vacuolated to clear cytoplasm and abundant vasculature
+inhibin, NSE, brachyury
- EMA, pax8, cd10
Dx
Molecular

A

Hemangioblastoma (grade 1)
VHL gene alterations
(Also looks like clear cell rcc)

32
Q

This neoplasm has chords in the ribbons of neoplastic cells in a myxoid background. Large cells have vacuoles, dated, bubbly cytoplasm. There is also extracellular matrix resembling hyaline cartilage.
+brachyury, EMA, CK, S100

A

Chordoma
Primary bone from notochord elements
If poorly diff - loss of smarcb1

33
Q

Pituitary adenoma IHC

A

+ synapto and chromo
- S100

34
Q

Pituitary blastoma molecular

A

DICER1 mutation
Elevated ACTH

35
Q

Adamantinomatous craniopharyngioma
Grade
Molecular
Ihorigin

A

Grade 1
Activating CTNNB1 mutations
+ nuclear beta catenin, p63
From rathke-pouch-related elements

36
Q

Papillary craniopharyngioma
Grade
Ihc
Molecular

A

Grade 1
+ p63 too
BRAF V600E mutation
Resembles a squamous papilloma

37
Q

Bland bipolar spindle cells in fascicularbor storiform pattern.
Tumor in posterior pituitary
+TTF1, S100
- synaptophysin

A

Pituicytoma

38
Q

Most Common lymphoma in the brain

A

DLBCL

39
Q

Most common metastasis in the brain

A

Men and women: lungs
Children: leukemia/lymphoma

40
Q

Dural lymphomas are MC what type

A

MZL

41
Q

Most common germ cell tumor in the brain

A

Pure Germinoma

CNS (1 decks)

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