Ihi Tb4 Cns Tumors

Front 1

Which CNS tumor is associated with tuberous sclerosis?

Back 1

subependymal gaint cell astrocytoma, pilocytic astrocytomas and hamaratomas

Front 2

Which CNS tumor is associated with NF1?

Back 2

neurofibromas - plexiform and elephantiasis; and optic gliomas

Front 3

Which CNS tumor is associated with NF2?

Back 3

bilateral CN8 schwannomas and mutliple meningiomas and gliomas of the spinal cord

Front 4

Which CNS tumor is associated with von Hippel-Lindau disease?

Back 4

hemangioblastoma in the retina, brainstem and cerebellum

Front 5

Which CNS tumor is associated with basal cell carcinoma (Gorlin syndrome)?

Back 5

medulloblastoma

Front 6

Which CNS tumor is associated with cowden syndrome?

Back 6

dysplastic gangliocytoma of the cerebellum

Front 7

Which CNS tumor is associated with turcot1?

Back 7

glioblastoma

Front 8

Which CNS tumor is associated with turcot 2?

Back 8

medulloblastoma

Front 9

Which CNS tumor is associated with retinoblastoma?

Back 9

pineoblastoma

Front 10

Which CNS tumor is associated with Li-Fraumeni syndrome?

Back 10

many - if a tumor is in a patient who should be too young for it, think Li-Fraumeni

Front 11

what is the most common solid extracranial malignant tumor during the first two years of life?

Back 11

neuroblastic tumors - neuroblastoma

Front 12

what is the most common location of neuroblastic tumors?

Back 12

adrenal gland

Front 13

What are the 3 neuroblastic tumor subtypes?

Back 13

1. neuroblastoma (most common) 2. ganglioneuroblastoma and 3. ganglioneuroma

Front 14

what are the 5 types of neurofibromas?

Back 14

1. localized cutaneous (most common) 2. diffuse cutaneous 3. localized intraneural - circumscribed mass in peripheral nerve, 4. plexiform (NF1) and 5. elephantiasis (NF1)

Front 15

a tumor of the peripheral nerve where the axon is incorporated into the tumor?

Back 15

neurofibroma

Front 16

where is the most common location for schwannomas?

Back 16

cerebellopontine angle = CN8 (presents with tinnis, hearing loss)

Front 17

robbery nodular dumbbell shaped tumor that is encapsulated and has a compressed nerve at its periphery?

Back 17

Schwannoma

Front 18

what is associated with a poor prognosis for neuroblastomas?

Back 18

MYCN gene amplification and double minute or trisomy 17q

Front 19

what is associated with a good prognosis for neuroblastomas?

Back 19

expression of TrkA receptor

Front 20

what type of differentiation is suggested with ultrastructural dense core granules?

Back 20

neuronal/neuroendocrine differentiation

Front 21

what type of differentiation is suggested with ultrastructural intracellular adhesion structures, microvilli and cilia?

Back 21

ependymal

Front 22

what type of differentiation is suggested by the presence of synaptophysin and chromogranin?

Back 22

neuroendocrine

Front 23

what type of differentiation is suggested by neurofilamen, Neu-N?

Back 23

neuronal differentiation

Front 24

what type of differentiation is suggested by glial fibrillary acidic protein (GFAP)?

Back 24

glial differentiation

Front 25

Where do medulloblastomas arise from?

Back 25

external granular cell layer of the cerebellum

Front 26

where are medulloblastomas located?

Back 26

posterior fossa - midline cerebellum, affects vermis (lateral lesions more likely to be seen in adults)

Front 27

histology: small hyperchromatic cells with small nuclei in a solid 'patternless' sheet

Back 27

medulloblastoma

Front 28

what is the most common malignant pediatric tumor?

Back 28

medulloblastoma

Front 29

what genetic mutations are associated with medulloblastoma?

Back 29

chromosome 17 and EGFR amplifications

Front 30

what tumor expresses Trk-C?

Back 30

medulloblastoma

Front 31

what tumor shows no morphological differentiation but on ICC shows neuroendocrine, astrocytic and neuronal differentiation?

Back 31

medulloblastoma

Front 32

benign tumor with peculiar fibrillary structures that look like hair

Back 32

pilocytic astrocytoma

Front 33

what age group is affected by pilocytic astrocytoma?

Back 33

infants and children; peak age: 8-13

Front 34

where are pilocytic astrocytomas located?

Back 34

cerebellum mostly

Front 35

what disease are pilocytic astrocytomas associated with?

Back 35

NF1 - located in optic nerve and hypothalamus when associated with NF1

Front 36

what is different about pilocytic astrocytomas compared to the other astrocytomas?

Back 36

pilocytic astrocytomas are well demarcated, the others are not

Front 37

what tumor contains glomeruloid vascular structures and rosenthal fibers?

Back 37

pilocytic astrocytoma

Front 38

which of the astrocytoma tumors does NOT enhance on MRI?

Back 38

astrocytoma - due to lack of leaky blood vessels

Front 39

which tumors are 'butterfly tumors"?

Back 39

anaplastic astrocytomas and glioblastomas - because they cross the midline through the corpus callosum

Front 40

where are the astrocytoma like tumors located?

Back 40

cerebrum, except for the pilocytoma which is located in the cerebellum

Front 41

what age group is affected by oligodendrogliomas?

Back 41

40-50 yoas

Front 42

where are oligodendrogliomas located?

Back 42

cerebral hemispheres

Front 43

histology: polygonal cells with small central round nuclei and a perinuclear halo = 'fried egg' appearance

Back 43

oligodendrogliomas

Front 44

which genetic mutations are associated with oligodendrogliomas that are responsive to therapy?

Back 44

deletions on 1p or 19q

Front 45

what age group is affected by ependyomas?

Back 45

bimodal: infants and children and young adults

Front 46

where are ependyomas located when they affect kids?

Back 46

infratentorial (4th ventricle)

Front 47

where are ependyomas located when they affect adults?

Back 47

supratentorial; in patients with NF2 - spinal cord

Front 48

gross: grayish cualiflower-like exophytic growth that protrudes into a ventricle

Back 48

ependymoma

Front 49

histology: cells arranged in rosettes perivascularly with the formation of ependymal canal

Back 49

ependymoma

Front 50

what are the unique features of an ependymoma seen with an EM?

Back 50

junctional complexes, microvilli, cilia in 9+2 arrangment and no basement membrane present

Front 51

what tumor has EM features of cilia arranged in 9+2?

Back 51

ependymoma

Front 52

what tumor has EM features of junctional complexes, microvilli, cilia and no basement membrane?

Back 52

ependymoma

Front 53

do ependymomas have basement membranes?

Back 53

NO

Front 54

what is unique about choroid plexus carcinomas?

Back 54

they affect young infants almost exclusively

Front 55

benign tumor with features of choroid plexus (looks like epithelium)?

Back 55

choroid plexus papilloma

Front 56

what age group is affected by choroid plexus papillomas?

Back 56

mostly <10yoa

Front 57

where are choroid plexus papillomas located?

Back 57

lateral and 4th ventricle

Front 58

gross: well demarcated pink cauliflower-like exophtic mass that fills the ventricle

Back 58

choroid plexus papilloma

Front 59

how many layers of cells do choroid plexus papillomas have?

Back 59

1 layer of cells, but it closely resembles the normal architecture

Front 60

do choroid plexus carcinomas maintain the papillary architecture?

Back 60

no, they appear as solid sheets with highly pleomorphic cells that are mitotically active (looks like an adenocarcinoma

Front 61

benign tumor with features of arachnoid cells?

Back 61

meningioma

Front 62

what age group and sex is affected by meningiomas?

Back 62

45-55 yoa and women

Front 63

where are meningiomas located?

Back 63

leptomeninges and dura mostly

Front 64

gross: well demarcated dural based tumor that doesn't adhere to or invade the brain?

Back 64

meningioma

Front 65

histology: concentric cellular whorls

Back 65

meningioma

Front 66

what is the sign of malignant behavior in meningiomas?

Back 66

brain invasion, NOT bone, sinus or soft tissue invasion

Front 67

what is a risk factor of meningiomas?

Back 67

radiation

Front 68

a patient has had radiation exposure, which CNS tumor are we worried about?

Back 68

meningioma

Front 69

locally aggressive tumor with features of notochord

Back 69

chordoma

Front 70

what age group is affected by chordoma?

Back 70

mostly adults

Front 71

where are chordomas located?

Back 71

cranial base and in lumbosacral spine

Front 72

histology: bubbly cytoplasm with blusih mucoid background

Back 72

chordoma

Front 73

what are the features of epithelial cell differentiation?

Back 73

intracellular adhesions and cytokeratin filaments

Front 74

where are hemangioblastomas located?

Back 74

paramedian cerebellum mostly

Front 75

gross: bluish-red to brownish-red "liver like" well circumscribed tumor

Back 75

hemangioblastoma

Front 76

histology: medium sized cells with clear cytoplasm and bland nuclei arranged in nests embedded with rich vascular network

Back 76

hemangioblastoma

Front 77

what feature is commonly seen in hemangioblastomas?

Back 77

cystic changes - 2/3 of tumors

Front 78

what tumor arises from epithelial cells derived from Rathke's pouch?

Back 78

craniopharyngioma

Front 79

gross: cystic tumor often with calcifications that contains fibrous CT and epithelium resembling squamous epithelium?

Back 79

craniopharyngioma

Front 80

histology: resembles ameloblastoma or squamous odontogenic tumors of the jaw

Back 80

craniopharyngioma

Front 81

what tumor arises from residual germ cell tissues left behind during migration?

Back 81

germ cell tumors

Front 82

what age group is affected by germ cell tumors?

Back 82

10-20, males

Front 83

where are germ cell tumors located?

Back 83

midline- commonly in the pineal and suprasellar regions

Front 84

histology: cells resemble gonadal cells

Back 84

germ cell tumors

Front 85

what tumor is comprised of diffuse large B-cells?

Back 85

primary CNS lymphoma

Front 86

what is the most common CNS neoplasm in the immunosuppressed?

Back 86

primary CNS lymphoma

Front 87

where are primary lymphomas located?

Back 87

deep gray matter and white matter - cerebrum, lateral and 3rd ventricle more frequently than cerebellum

Front 88

histology: intesnse perivascular clustering of anaplastic lymphocytes in the Virchow-Robin space

Back 88

primary CNS lymphoma

Front 89

what is associated with primary CNS lymphomas in the immunocompromised?

Back 89

EBV

Front 90

what are the top 3 sources of metastatic brain cancer?

Back 90

1. lung, 2. breast, 3. skin/melanoma

Front 91

what are the top 4 sources of metastatic brain cancer that compresses the spinal cord?

Back 91

1. breast, 2. lung, 3. prostate, 4. malignant lymphoma

Front 92

where do metastatic brain tumors like to go?

Back 92

to the gray/white junction - where there is a reduction in the caliber of blood vessels

Front 93

Does adenocarcinoma or SCC like to go to the brain?

Back 93

adenocarcinoma

Front 94

where do renal cell carcinomas like to go?

Back 94

cerebellum mostly

Front 95

which metastasize to the brain more often, choriocarcinoma or germ cell tumors?

Back 95

choriocarcinoma