Tumors/cysts Flashcards

1
Q

Astrocytoma

A

Pilocytic astrocytoma
SEGA
Pleomorphic xanthoastrocytoma
Diffuse astrocytoma
Anaplastic astrocytoma
Glioblastoma multiforme
Oligodendroma

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2
Q

Neuronal and mixed neuronal glial tumors

A

Ganglioglioma
Desmoplastic infantile astrocytoma/ganglioglioma
Dysembryonic neuroeoithelial tumor (DNET)
Central neurocytoma

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3
Q

Pineal region tumors

A

Pineoblastoma
Pineocytoma

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4
Q

Embryonal tumors

A

Medulloblastoma

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5
Q

Meningeal tumors

A

Meningioma

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6
Q

Cranial nerve tumors

A

Schwannoma
Neurofibroma
Optic nerve glioma
Carcinomatosis meningitis
Lymphomatous meningitis

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7
Q

Lymphomas and hematopoietic tumors

A

Hemangioblastoma
CNS lymphoma
Intravascular lymphoma
Hemangiomas
Multiple myeloma
Langerhans cell histocytosis

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8
Q

Germ cell tumors

A

Germinoma

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9
Q

Sella region tumors

A

Craniopharyngioma
Pituitary macroadenoma
Pituitary microadenoma
Hypothalamic hamartoma

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10
Q

Familial tumor syndromes of CNS

A

NF-1
NF-2
TS
Sturge Weber
Von Hippel Lindau
Li-Fraumeni
Codes syndrome
Turcot

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11
Q

Paraneoplastic disorders

A

Limbus encephalitis
Paraneoplastic cerebellar degeneration
Lambert Eaton

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12
Q

A pediatric, slow growing tumor composed of astrocytes.

A

Pilocytic astrocytoma

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13
Q

Syndromes can develop Pilocytic astrocytomas?

A

TS
NF1
Li-Fraumeni

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14
Q

Symptoms of Pilocytic astrocytoma:

A

Increased ICP - HA, N/V, vision loss, ataxia, CN nerve deficits
Location based

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15
Q

What tumors are associated with NF1?

A

Optic nerve and chiasm glioma

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16
Q

Imaging findings for Pilocytic astrocytoma

A

Cystic mass w/ contrast rim enhancing mural nodule
Minimal vasogenic edema

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17
Q

Where are Pilocytic astrocytomas most commonly found?

A

Cerebellum

Supratentorial hemispheres > optic pathway/hypothal > spinal cord

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18
Q

Characteristic gene fusion for pilocytic astrocytoma

A

KIAA1549-BRAF

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19
Q

Pathology characteristics of pilocytic astrocytoma

A

Rosenthal fibers
Eosinophilia granular bodies in stacked bipolar cells

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20
Q

Describe the classic presentation of a pilocytic astrocytoma in children

A

D w/ increased ICP/ataxia found to have a cystic cerebellar mass

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21
Q

Which patients are subependymal giant cell astrocytoma almost exclusively seen in?

A

Tuberous sclerosis

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22
Q

When do SEGAs become symptomatic?

A

If they become large enough to cause obstructive hydrocephalus if blocking the foramen of Monro

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23
Q

What is seen on pathology of SEGAs/

A

Large polygonal cells with eosinophilic cytoplasm and a smaller number of giant pyramidal ganglioid astrocytes

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24
Q

What is the treatment for SEGAs?

A

Resection

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25
Q

What tumor is commonly found in younger patients presenting with temporal lobe epilepsy?

A

Pleomorphic xanthoastrocytoma (PXA)

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26
Q

What is seen in imaging of a Pleomorphic xanthoastrocytoma (PXA)?

A

Supratentorial peripheral cystic and contrast-enhancing mass abutting the leptomeninges with enhancing dural tail sign and scalloping of overlying bone

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27
Q

What mutation are PXA associated with?

A

BRAF

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28
Q

What age group is most common for diffuse astrocytoma?

A

Young adults 20-45

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29
Q

Common presentation for diffuse astrocytoma:

A

Seizure and headaches

30
Q

What genes are diffuse astrocytoma associated with?

A

Isocitrate dehydrogenase (IDH)
TP53
ATRX

31
Q

What is a gemistocytic astrocytoma?

A

Subtype of IDH mutant diffuse astrocytoma
More aggressive behavior

32
Q

What are the MRS findings for diffuse astrocytoma?

A

Elevated choline peak, low NAA peak, elevated choline: creatine ratio

33
Q

Treatment for diffuse astrocytoma:

A

Maximal safe resection
Field radiation
Chemo

34
Q

What indicated a better prognosis in anaplastic astrocytoma?

A

IDH1/2 mutation and MGMT methylation

35
Q

What does imaging appear as for anaplastic astrocytoma?

A

An infiltrating mass that predominantly involves hemispheric white matter with variable enhancement

36
Q

What pathologic findings are seen in anaplastic astrocytoma?

A

Increased cellularity
Marked mitotic activity
Distinct nuclear atypia
No necrosis or endothelial proliferation

37
Q

What is the characteristic pattern seen in imaging for a glioblastoma (GBM)?

A

Butterfly lesion
Contrast-enhancing multicystic lesions with significant vasogenic edema
Internal necrosis and can extend through the corpus callosum

38
Q

What can indicate a better response to alkylating chemotherapy for GBM?

A

MGMT promoter methylation

39
Q

If a GBM has a IDH-1/2 mutation what could this mean?

A

Secondary GBM arising from a lower-grade glioma

40
Q

What is the pathology associated with GBM?

A

Increased mitotic activity with pseudopalisading necrosis
Microvascular endothelial proliferation

41
Q

Treatment for GBM

A

Maximal safe resection
Intensity-modulated radiation therapy (IMRT) and temozolomide

42
Q

In GBM what indicated a better treatment response to temozolomide?

A

Methylation of the MGMT gene

43
Q

What chemotherapy can be used in recurrent or progressive GBM?

A

Bevacizumab
Inhibits vascular endothelial growth factor (VEGF)

44
Q

What grade are oligodendromas?

A

WHO grade II or WHO grade III

45
Q

What genetic findings are associated with oligodendromas?

A

1p/19q co-deletion and IDH mutations

46
Q

What mutation has a better prognosis in oligodendromas?

A

1p/19q co-deletion

47
Q

Where are oligodendromas generally found in the brain?

A

Frontal or temporal lobes

48
Q

Pathologic findings in oligodendroma:

A

Monotonous round nuclei, surrounded by prominent perinuclear halos
“Fried egg” appearance

49
Q

Pathologic findings in ependymal tumors:

A

Monomorphic cells with round/oval nuclei
Salt and pepper chromatin
Perivascular pseudorosettes

50
Q

What age group of choroid plexus papilloma or carcinoma occur in?

A

Usually infants, before age one

51
Q

What are the symptoms of choroid plexus papilloma?

A

Symptoms of increased ICP due to overproduction of CSF

52
Q

Imaging findings for ganglioma:

A

A cystic mass with a mural nodule
Usually temporal lobe

53
Q

What is a symptoms based on location of a ganglioma?

A

Refractory epilepsy

54
Q

Mutations associated with gangliomas:

A

BRAF
IDH negative

55
Q

Pathology of ganglioma:

A

Dysmorphic ganglion cells with binucleated forms

56
Q

What are the characteristics of a Desmoplastic infantile astrocytoma and ganglioglioma?

A

WHO grade 1
Present before age 2
M>F
Rapid increase in head size

57
Q

Imaging findings for Desmoplastic infantile astrocytoma and ganglioglioma:

A

Large cystic tumor with a peripheral solid component attached to the meninges of frontal or parietal lobes

58
Q

What are the characteristics of a Dysembryonic neuroepithelial tumor (DNET)?

A

WHO grade 1
Arises from cortical gray matter
Associated with cortical dysplasia
Often temporal lobes

59
Q

What do DNETs tend to cause?

A

Temporal lobe epilepsy

60
Q

Pathology associated with DNET:

A

Myxoid clear areas with floating neurons

61
Q

Imaging associated with DNET:

A

A wedge-shaped cystic cortical mass lesion “soap-bubble” appearance
Typically non-enhancing

62
Q

What are the characteristics of a central neurocytoma?

A

WHO grade II
Signs/symptoms of increased ICP

63
Q

Imaging findings for a central neurocytoma:

A

Cystic/bubbly lesion within the lateral ventricle near the foramen of Monro
Often attached to septum pellucidum

64
Q

Pathologic findings in central neurocytoma:

A

Neurocytic rosettes and uniform round blue cells

65
Q

What are the characteristics of a pineoblastoma?

A

Seen in children
Can lead to hydrocephalous

66
Q

Imaging findings in pineoblastoma?

A

A calcified heterogeneous soft tissue mass in the pineal region with enhancement

67
Q

Pathologic findings in pineoblastoma:

A

Densely cellular primitive neuroectodermal tumors that often form Homer Wright (medulloblastoma-type) rosettes

68
Q

What symptoms will pineocytoma present with?

A

Hydrocephalus or mass effect

69
Q

What is the most common malignant brain tumor in childhood?

A

Medulloblastoma

70
Q
A