Robbins

Question 1

most common group of primary brain tumors

Answer 1

Gliomas

Question 2

two major categories of astrocytic tumors

Answer 2

diffusely infiltrating astrocytomas (WHO grade II to IV) and the more localized astrocytomas (ie. pilocytic astrocytoma, WHO grade I)

Question 3

Secondary glioblastomas and their lower-grade precursors are associated with driver mutations of (2)

Answer 3

IDH1 and IDH2

Question 4

T/F most primary glioblastomas are IDH–mutant, WHO grade IV

Answer 4

False, most primary glioblastomas are IDH–wild type, WHO grade IV

Question 5

Diffuse Astrocytoma vs Anaplastic

Answer 5

more densely cellular, more notable nuclear atypia and presence of mitosis

Question 6

Anaplastic astrocytoma vs Glioblastoma

Answer 6

features of anaplastic + necrosis and hypervascularity

Question 7

Mutations in primary glioblastoma (4)

Answer 7

TERT, EGFR, CDKN2A, TP53

Question 8

Primary vs secondary glioblastoma

Answer 8

Primary - older, no precursor lesion, IDH-wild type, TERT mutations

Secondary - younger, with precursor astrocytoma, IDH mutant, ATRX mutant

Question 9

Oligodendroglioma is molecularly defined by IDH ____ and codeletion of

Answer 9

mutant, and codeletion of 1p and 19q

Question 10

brain tumor composed of sheets of regular cells with spherical nuclei containing finely granular chromatin surrounded by a clear halo of vacuolated cytoplasm and typically contains a delicate network of anastomosing capillaries, resembling “chicken wire.”

Answer 10

Oligodendroglioma

Question 11

distinct genetic alteration in Pilocytic astrocytoma (WHO grade 1)

Answer 11

KIAA1549-BRAF gene fusion/ duplication

Question 12

brain tumor with a biphasic architecture combining loose “microcystic” and compact, densely fibrillar areas.

Answer 12

Pilocytic astrocytoma

Question 13

T/F presence of microvascular proliferation or necrosis in Pilocytic astrocytoma implies an unfavorable prognosis

Answer 13

False

Question 14

Characteristic finding in Pilocytic astrocytoma (4)

Answer 14

biphasic, limited brain invasion, hair-like processes, rosenthal fibers

Question 15

tumors that most often arise in proximity to the ependyma-lined ventricular system, including the oft-obliterated central canal of the spinal cord.

Answer 15

Ependymoma

Question 16

commonly mutated in ependymomas arising in the spinal cord

Answer 16

NF2 gene

Question 17

distinct lesions that occur in the filum terminale of the spinal cord. Cuboidal tumor cells are arranged in a variably papillary architecture around mucin-rich fibrovascular cores.

Answer 17

Myxopapillary ependymomas (WHO grade I)

Question 18

tumors composed of a mixture of mature neuronal and glial cells. They are typically superficial lesions that present with seizures and are the most common neuronal tumors in the CNS

Answer 18

Gangliogliomas (WHO grade I)

Question 19

Approximately 20% to 50% of gangliogliomas have an activating mutation in

Answer 19

BRAF V600E

Question 20

most common brain embryonal neoplasm which occurs predominantly in children and exclusively in the cerebellum

Answer 20

medulloblastoma

Question 21

Molecular subtypes of medulloblastoma reveal alterations of signaling pathways (2)

Answer 21

sonic hedgehog–patched (SHH) pathway and WNT/β-catenin signaling pathway

Question 22

characteristic finding in classic subtype of medulloblastoma

Answer 22

Homer Wright rosettes

Question 23

most common CNS neoplasm in immunosuppressed individuals, including those with AIDS and immunosuppression after transplantation

Answer 23

Primary CNS lymphoma

Question 24

Primary CNS lymphoma in the setting of immunosuppression, the malignant B cells are usually latently infected by

Answer 24

EBV

Question 25

In those who are not immunosuppressed, primary CNS large B-cell lymphomas often have amplification and overexpression of what gene?

Answer 25

PDL1

Question 26

Of sporadic meningiomas, 50% to 60% harbor mutations in what gene?

Answer 26

NF2

Question 27

Mutations in TSC1 or TSC2 lead to increased and unregulated

Answer 27

mTOR activity

Question 28

T/F Genetic changes that indicate an IDH-wild type astrocytoma will behave aggressively and should be graded as Glioblastoma, grade IV even if traditional morphologic findings of glioblastoma aren’t present

Answer 28

True

Question 29

characteristic finding in ependymoma

Answer 29

pseudorosettes

Question 30

meningioma subtypes that are considered grade 2

Answer 30

choroid and clear cell

Question 31

meningioma subtypes that are considered grade 3

Answer 31

papillary and rhabdoid

Question 32

herniation type that occurs when unilateral or asymmetric expansion of a cerebral hemisphere displaces the cingulate gyrus under the falx.This may lead to compression of the anterior cerebral artery and its branches, resulting in secondary infarcts.

Answer 32

Subfalcine (cingulate) herniation

Question 33

herniation type that occurs when the medial aspect of the temporal lobe is compressed against the free margin of the tentorium, third cranial nerve is compromised, resulting in pupillary dilation and impaired ocular movements on the side of the lesion.

Answer 33

Transtentorial(uncal,mesialtemporal) herniation

Question 34

Progression of transtentorial herniation is often accompanied by secondary hemorrhagic lesions in the midbrain and pons, termed

Answer 34

Duret hemorrhages

Question 35

herniation type that refers to displacement of the cerebellar tonsils through the foramen magnum.This pattern of herniation is life-threatening because it causes brainstem compression and compromises vital respiratory and cardiac centers in the medulla.

Answer 35

Tonsillar herniation

Question 36

the most common neural tube defect and may be an asymptomatic bony defect or a severe malformation with a flattened, disorganized segment of spinal cord, associated with an overlying meningeal outpouching.

Answer 36

spina bifida

Question 37

refers to an extrusion of malformed brain tissue through a midline defect in the cranium

Answer 37

Encephalocele

Question 38

refers to extension of CNS tissue through a defect in the vertebral column

Answer 38

Myelomeningocele

Question 39

is a malformation of the anterior end of the neural tube that leads to absence of most of the brain and calvarium

Answer 39

Anencephaly

Question 40

posterior fossa anomaly which consists of a small posterior fossa, a misshapen midline cerebellum with downward extension of vermis through the foramen magnum

Answer 40

Arnold-Chiari malformation (Chiari type II malformation)

Question 41

posterior fossa anomaly which consists of low-lying cerebellar tonsils extend down into the vertebral canal.

Answer 41

Chiari type I malformation

Question 42

posterior fossa anomaly which is characterized by an enlarged posterior fossa. The cerebellar vermis is absent or present only in rudimentary form in its anterior portion; in its place is a large midline cyst that is lined by ependyma and is contiguous with leptomeninges on its outer surface.

Answer 42

Dandy-Walker malformation

Question 43

depressed, retracted, yellowish brown patches involving the crests of gyri, most commonly those that are located at the sites of contrecoup injuries (inferior frontal cortex, temporal and occipital poles); these lesions are called

Answer 43

plaque jaune

Question 44

the pathognomonic microscopic finding in rabies called _____, are cytoplasmic, round to oval, eosinophilic inclusions found in pyramidal neurons of the hippocampus and Purkinje cells of the cerebellum

Answer 44

negri bodies