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Neurology > CNS Tumors > Flashcards

CNS Tumors Flashcards

1
Q

In adults, are most primary brain tumors infratentorial or supratentorial?

A

supratentorial

opposite from kids, where most are infratentorial

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2
Q

What is the most common symptoms of a brain tumor?

A

headache
seizure
altered mental status

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3
Q

What chromosome is neurofibromatosis 1 on? What tumors do you get in NFT1?

A

17

gliomas of the optic nerve and ependymoma

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4
Q

What chromosome is neurofibromatosis 2 on? What tumors do you get in NFT2?

A

22q12

meningioma and glioma

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5
Q

What chromosome is von Hippel-Lindau on? What tumor go you get with VHL?

A

3p25

hemangioblastoma

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6
Q

What chromosome is the Li-Fraumeni cancer family syndrome on? What brain tumors do you get?

A

17p13.1 (an inherited p53 mutation)

glioma and medulloblastoma

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7
Q

What are the two most important prognostic factors for brain tumors?

A

histologic type and patient age

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8
Q

What are the two most common primary brain tumor types?

A

gliomas and meningiomas

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9
Q

Glioma is a generic histologic term used for what four different CNS tumors?

A

astrocytoma
oligodendroglioma
ependmoma
choroid plexus papilloma

(it’s basically just a tumor of the glial cells)

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10
Q

What cell type does glioblastoma multiforme arise from?

A

astrocytoma (it’s just a grade IV astrocytoma)

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11
Q

What is the peak age of onset for GBM?

A

40-60 yo

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12
Q

Describe the pathology of GBM.

A

highly malignant tumor with anaplsia, high cellularity, round and pleomorphic cells, nuclear atypia, vascular proliferation, and necrosis

(the necrosis and neovascular proliferation are what differentiate a GBM from a grade III astrocytoma)

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13
Q

Where in the brain are GBMs typically located?

A

deep white matter, basal ganglia, or thalamus (rarely infratentorial)

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14
Q

What will a GBM look like on CT or MRI?

A

usually a solitary mass with contrast enhancement and surrounding edema

often infiltrating the corpus callosum producing the typical “butterfly” pattern

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15
Q

What is the typical treatment for a GBM?

A
  1. maximal safe surgical resection
  2. radiation with concurrent temozolomide
  3. adjuvant temozolomide for 6 months

in addition: nitrosoureas are considered for combo therapy and bevacizumab can be used for local recurrences

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16
Q

What is the life expectancy in GBM?

A

not good. less than 3-5 yr survival

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17
Q

What will a low-grade astrocytoma look like on MRI with contrast?

A

bright on T2 and FLAIR, usually without enhancement (unlike the GBM which usually enhances)

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18
Q

What is the treatment for grade 1 and 2 astrocytomas?

A

close observation with serial imaging may be the first approach depending on prognostic factors because these can be very slow growing

surgical removal can be curative for grade I astrocytomas and can be considered for grade II if the gross total resection is possible (but is often not curative)

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19
Q

What is the median survival for low grade astrocytoma?

A

around 7 years

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20
Q

What is the “genetic signature” of oligodendrogliomas?

A

codeletion of chromosomal arms 1p and 19q

and people with this codeletion tend to respond better to therapy

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21
Q

Where is the most common location for an oligodendroglioma?

A

frontal lobe most common

but also basal ganglia and thalamus

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22
Q

Describe the pathology of an oligodendroglioma.

A

most distinctive microscopic feature is the “fried egg” appearance with perinuclear halos with swollen cytoplasm

calcifications are common

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23
Q

What will an oligodendroglioma look like on MRI? CT?

A

MRI with low intensity on T1, high intensity on T2, vasogenic edema is NOT common. If it enhances, that’s a bad prognostic factor

CT scan is better to see the calcifications

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24
Q

What is the treatment for an oligodendroglioma?

A

total resection if possible, local radiation and PCV (procarbazine, lomustine and vincristine) chemotherapy

Temozolomide is used increasingly

25
Q

What is the prognosis for oligodendroglioma?

A

better survival with surgery plus radiation; can be decades

however, it tends to recur locally and progress into a malignant form

26
Q

What cells do meningiomas originate from?

A

the meningothelial cells of the dura mater

27
Q

Meningiomas rarely occur intracranially, but when they do, where are they?

A

cerebral convexities, falx cerebri, and the sphenoid wing

28
Q

What genetic condition is associated with a higher risk for meningiomas?

A

NF-2

29
Q

Describe the pathology of a meningioma.

A

sheets of plump, uniform meningothelial cellswith the tendency to form whorlds

30
Q

What receptors are found frequently in meningiomas?

A

progesterone (probably why they’re more common in women)

31
Q

What will a meningioma look like on MRI?

A

a rounded extra-axial mass adjacent to the dura

isointense on T1 and T2 with intense contrast enhancement

32
Q

What will a meningioma look like on CT?

A

calcifications seen on CT
angiography can show rich vascularization

can also see hyperostosis, which is an osteoblastic reaction that occurs if the tumor is invading the skull

33
Q

What is the treatment for meningiomas?

A

surgical removal, often preceded by endovascular embolization of the feeding vessels

stereotactic radiosurgery is an option when resection is difficult or dangerous

34
Q

What is the prognosis for meningiomas?

A

5-yr survival rate at 70-95%

malignant transformation is rare

35
Q

What is the origin of medulloblastoma?

A

primarily at the medullary velum of the 4th ventricle

it’s one of the primitive neuroectodermal tumors

36
Q

Medulloblastoma makes up what percentage of pediatric primary brain tumors?

A

30%

37
Q

Describe the pathology of medulloblastoma.

A

small round cells with a high mitotic index

38
Q

What is the typical presentation of a medulloblastoma?

A

It’s rapidly growing and infiltrates surrounding tissue and the 4th ventricle, so you get a hydrocephalus with morning headaches, unsteadiness, nausea and vomiting

39
Q

What will a medulloblastoma look like on MRI?

A

a heterogeneous contrast enhancing midline tumor compressing the 4th ventricle

40
Q

Why do you also do an MRI of the c-spine after a diagnosis of medulloblastoma?

A

it can metastasize to the subarachnoid along the spinal cord

41
Q

What is the treatment for medulloblastoma?

A

surgery plus radiation and chemo

corticosteroids for vasogenic edema

42
Q

What is the prognosis of medulloblastoma

A

20-30% will relapse after initial treatment

poor prognosis with metastatic disease or subtotal resection ( no duh)

good prognosis with radical resection and radiation dose above 50 Gy to the entire neuroaxis, in which case recurrence-free survival at 5 years is over 50%

43
Q

Where is the most common location for a schwannoma?

A

cranial nerve VIII - an acoustic neuroma occupying the cerebellopontine angle

44
Q

Bilateral schwannomas is associated with what disorder?

A

NF-2

45
Q

Describe the pathology of a schwannoma.

A

sheets of uniform spindle cells forming palisades called “Verocay bodies”

46
Q

Where do hemangiomas occur in the brain?

A

cerebellum - it’s a cystic lesion

47
Q

Primary CNS lymphoma is rare, but has a higher incidence in what patients?

A

AIDS patients, particularly men

48
Q

WHat is the treatment of choice for CNS lymphoma?

A

high-dose systemic methotrexate-based chemo +/- radiation

49
Q

What is the prognosis for CNS lymphoma?

A

poor

1.5 months if untreated, 10-18 months after radiation and 44 months after chemo plus radiation

50
Q

What is a common complication in patients who survive over 18 months with CNS lymphoma after radiation?

A

dementia (so shitty)

51
Q

Primary CNS lymphoma can have a dramatic response to what class of medication? (although it invariably recurs within months).

A

steroids

52
Q

What is the size difference between a microadenoma and macroadenoma of the pituitary?

A

1 mm

53
Q

Surgical removal of the pituitary is via what approach?

A

transsphenoidal

54
Q

What tumor affecting the pituitary comprises 50% of suprasellar tumors in children?

A

craniopharyngioma

55
Q

What is the radiologic hallmark of a craniopharyngioma?

A

suprasellar calcified cyst

56
Q

What are the most common tumors that cause metastases to the brain?

A 
lung
skin (melanoma)
renal cell carcinoma
breast
colon
57
Q

What are the two routes malignant cells will take to seed the CNS?

A

via the bloodstream crossing the BBB

through Batson’s plexus (pelvic and GI tumors)

58
Q

In general, where in the brain are mets located?

A

at the junction of the white-gray matter

Neurology (25 decks)

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