Lec 12: Pathology of Nervous System Tumors Flashcards

1
Q

Majority of the tumors in children are located

A. supratentorial
B. infratentorial
C. cerebral
D. extraparenchymal
E. intraparenchymal
A

B.

adult - 70% supratentorial
pedia - 70% infratentorial

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2
Q

These are the most common primary CNS tumors.

A. gliomas
B. choroid plexus tumors
C. embryonal tumors
D. metastatic tumors
E. neuromas
A

A. gliomas

These include

astrocytomas
oligodendrogliomas
ependymomas

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3
Q

T/F: Mixed type CNS tumors are usually benign.

A

T

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4
Q

True of the WHO grading scheme for nervous system tumors

A. predicts biologic behavior
B. most applicable to astrocytomas
C. grade 2 is infiltrative
D. A & B only
E. AOTA
A

E. AOTA

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5
Q

Enumerate the four features used in the WHO grading scheme.

A

A-M-E-N

atypia / nuclear pleomorphism
mitotic activity
endothelial / microvascular proliferation
necrosis

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6
Q

ID: Grade IV astrocytomas are also termed as

A

glioblastomas

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7
Q

This is the first mutation in the astrocytoma / oligodendroglioma tumor lineage.

A. IDH1 mutation
B. p53 mutation
C. 1p19q co-deletion
D. EGFR amplification
E. MGMT mutation
A

A. IDH1 mutation

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8
Q

This mutation drives the glioma tumor to progress into the oligodendroglioma lineage.

A. IDH1 mutation
B. p53 mutation
C. 1p19q co-deletion
D. EGFR amplification
E. MGMT mutation
A

C. 1p19q co-deletion

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9
Q

1p19q co-deletion is implicated in the formation of what tumor?

A. primary glioblastoma
B. secondary glioblastoma
C. medulloblastoma
D. meningioma
E. ganglioglioma
A

A.

Primary glioblastomas skip the glioma lineage, meaning the initial presentiation is already glioblastoma (grade IV).

Secondary glioblastomas run through the glioma - astrocytoma / oligodendroglioma - glioblastoma lineage.

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10
Q

T/F: The IDH1 mutation is a strong independent favorable prognostic marker in astrocytoma.

A

T

It allows for diagnostic and therapeutic targeting.

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11
Q

ID: This drug introduces methyl groups to DNA of tumor such that it becomes mutated and does not proliferate.

A

temozolomide

It is an alkylating agent involved in the methylation of DNA.

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12
Q

How do you specifically differentiate gliomas from reactive gliosis?

A. stain with GFAP
B. check for IDH1
C. stain with india ink
D. A & B only
E. AOTA
A

B. check for IDH1 mutation

Note: More GFAP is expressed in gliomas than in gliosis.

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13
Q

True of pilocytic astrocytomas EXCEPT

A. common in children and young adults
B. grade I in the WHO scheme
C. has Rosenthal fibers
D. p53 mutation is common
E. NOTA
A

D.

p53 mutations are rare in pilocytic astrocytomas.

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14
Q

ID: This is the molecular marker for pilocytic astrocytoma.

A

BRAF fusion gene

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15
Q

ID: Rosenthal fibers that aggregate and form clusters are called

A

eosinophilic granular bodies

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16
Q

Morphological characteristics of oligodendroglial tumors

A. fried egg appearance
B. chicken wire anastomosing capillaries
C. more fibrillar than astrocytomas
D. A & B only
E. AOTA
A

D.

They are LESS fibrillar than astrocytomas.

17
Q

ID: What do you call a grade III oligodendroglioma?

A

anaplastic oligodendroglioma

18
Q

ID: Perivascular pseudorosettes are characteristic of what tumor?

A

ependymoma

Ependymal cells line the ventricles and the central canal.

19
Q

This tumor leads to incresed CSF production.

A. medulloblastoma
B. ependymoma
C. choroid plexus papilloma
D. meningioma
E. NOTA
A

C.

Choroid plexus produces CSF

20
Q

Medulloblastomas are always graded initially as

A. I
B. II
C. III
D. IV
E. WHO grading scheme does not apply.
A

D. IV

They are high grade tumors with very poor prognosis.

21
Q

ID: Characteristic of medulloblastoma featuring a group of dark blue undifferentiated cells with no cavity nor blood vessel in the center.

A

Horner-Wright rosettes

Pseudorosettes have blood vessels in the middle.

22
Q

ID: Medulloblastoma-like tumors found supratentorially are termed as

A

primitive neuroectodermal tumors

23
Q

This molecular subgroup of medulloblastoma has the best prognosis.

A. wnt MB
B. shh MB
C. group 3 MB
D. group 4 MB
E. B & C only
A

A. wnt MB

Group 3 MB has the worst prognosis.

24
Q

Enumerate two meningioma subtypes that have grade II classification.

A

clear cell meningioma

chordoid meningioma

25
Enumerate two menigioma subtypes that have grade III classification.
rhabdoid meningioma | papillary meningioma
26
The following are associated with grade II meningiomas EXCEPT ``` A. >20 mitoses / 10 HPF B. sheeting architecture C. hypercellularity D. brain invasion E. NOTA ```
A. >20 are associated with grade III already. 4-20 are associated with grade II.
27
ID: Primary CNS lymphoma are typically composed of what cells?
B-cells
28
True of Schwannoma EXCEPT A. S-100 protein positive B. arise within the nerve C. associated with neurofibromatosis type 2 D. may lead to malignant peripheral nerve sheath tumor E. NOTA
B. They only arises beside the nerve, only displaces the nerve, and are easily separable from the nerve. Neurofibromas are the ones that arise within the nerve.
29
Spindle cells in bundles in Schwannomas are characteristic of ``` A. Antoni A B. Antoni B C. Verocay body D. von Hippel Lindau E. NOTA ```
A.
30
ID: What is the most classic manifestation of neurofibromatosis type II?
bilateral vestibulocochlear (CN8) nerve schwannoma