Tumors & Cysts Flashcards

1
Q

astrocytoma

A

slow-growing astrocytic tumor composed of bipolar “hair-like” (pilocytic) cells
most common glioma in children

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2
Q

astrocytoma associations

A

tuberous sclerosis, neurofibromatosis type 1 (NF1), and Li-Fraumeni syndrome
- optic nerve and chiasm glioma associated with NF1

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3
Q

astrocytoma presentation

A

symptoms of increased ICP (headache, nausea/vomiting), vision loss, ataxia, or cranial nerve deficits depending on location

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4
Q

astrocytoma on imaging

A

cystic mass with a contrast rim-enhancing nidus or mural nodule with minimal vasogenic edema, dorsally exophytic
most commonly found in the cerebellum
also prefers midline structures such as the brainstem, optic chiasm, hypothalamus, and deep gray matter (basal ganglia)

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5
Q

astrocytoma genes

A

KIAA1549-BRAF gene fusion is characteristic of this tumor type

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6
Q

astrocytoma pathology

A

hair-like cytoplasmic fibers (Rosenthal fibers) and eosinophilic granular bodies in stacked bipolar cells

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7
Q

astrocytoma prognosis

A

90% 10-year overall survival
can be treated with surgical resection alone, and rarely progresses to malignant glioma

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8
Q

classic patient presentation of astrocytoma

A

child presenting with increased ICP/ataxia found to have a cerebellar cystic mass lesion with enhancing mural nodule

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9
Q

subependymal giant cell astrocytoma (SEGA)

A

WHO grade 1 tumor almost exclusively seen in pediatric patients with tuberous sclerosis (TS) and before the age of 20
- seen in 5-15% of patients with TS

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10
Q

SEGA symptoms

A

often asymptomatic
but when symptomatic presents with obstructive hydrocephalus due to location in the foramen of Monro

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11
Q

SEGA imaging

A

well-circumscribed, partially-calcified intraventricular contrast-enhancing mass near the foramen of Monro

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12
Q

SEGA pathology

A

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

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13
Q

SEGA treatment

A

generally treated initially with mTOR inhibition with everolimus
if acutely symptomatic or growing, can be treated with surgical resection

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14
Q

Tuberous sclerosis review

A

classically presents with seizures, mental retardation, and adenoma sebaceum. Associated with TSC2/tuberin (most cases) or TSC1/hamartin with cortical or subependymal tubers, hamartomas, renal angiomyolipomas, and cardiac rhabdomyomas

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15
Q

pleomorphic xanthoastrocytoma (PXA)

A

found in young patients who present with temporal lobe epilepsy

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16
Q

pleomorphic xanthoastrocytoma (PXA) imaging

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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17
Q

pleomorphic xanthoastrocytoma (PXA) pathology

A

variable histological features (thus, pleomorphic) with spindle cells, polygonal cells, multinucleated cells, highly variable nuclear size, and astrocytes with eosinophilic granular bodies

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18
Q

pleomorphic xanthoastrocytoma (PXA) genetics

A

associated with BRAFV600E mutations and homozygous CDKN2A/B deletions

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19
Q

pleomorphic xanthoastrocytoma (PXA) treatment

A

surgical resection
local recurrence and malignant transformation are common so post-operative radiation is indicated for grade 3 tumors

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20
Q

adult-type diffuse gliomas

A

astrocytoma, IDH-mutant (grades 2-4)
glioblastoma, IDH-wildtype (grade 4)

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21
Q

astrocytoma, IDH-mutant

A

patients present with progressive neurologic symptoms dependent on tumor location and/or with seizures

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22
Q

astrocytoma, IDH-mutant imaging

A

T2-FLAIR mismatch sign often present, with T2 hyperintensity and relative hypointensity on FLAIR sequences
- MR spectroscopy will have an elevated choline peak, low NAA peak, and elevated choline:creatinine ratio

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23
Q

astrocytoma, IDH-mutant pathology grade 2

A

mitotic activity absent or low without microvascular proliferation, necrosis, or genetic markers that would upgrade the tumor (homozygous deletion of CDKN2A/B)

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24
Q

astrocytoma, IDH-mutant pathology grade 3

A

mitotic activity present without microvascular proliferation, necrosis, or genetic markers that would upgrade the tumor
- formally known as anaplastic astrocytoma

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25
Q

astrocytoma, IDH-mutant pathology grade 4

A

microvascular proliferation, necrosis, or genetic markers that would upgrade the tumor present

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26
Q

glioblastoma, IDH-wildtype (grade 4)

A

formally known as glioblastoma multiforme (GBM)
most common and most destructive of the diffuse gliomas

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27
Q

glioblastoma, IDH-wildtype (grade 4) imaging

A

contrast ring-enhancing lesions with significant vasogenic edema. lesions can also have internal necrosis and can extend through the corpus callosum (butterfly lesion)

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28
Q

glioblastoma, IDH-wildtype (grade 4) genetics

A

IDH-1/2 mutations are associated with secondary GBM arising from a lower-grade glioma
tumors without microvascular proliferation or necrosis can still be classified a “molecular” glioblastoma if genetic testing shows TERT promotor mutation, EGFR gene amplification, or gain of 7/loss of 10 chromosome copy number alterations

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29
Q

glioblastoma, IDH-wildtype (grade 4) pathology

A

cells with increased mitotic activity with pseudopalisading necrosis and microvascular endothelial proliferation

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30
Q

glioblastoma, IDH-wildtype (grade 4) treatment

A

maximal safe resection followed by intensity-modulated radiation therapy (IMRT) plus concomitant temozolomide (alkylating chemotherapy) followed by adjuvant temozolomide
- methylation of MGMT gene associated with better treatment response to temozolomide
- bevacizumab, a monoclonal antibody that inhibits vascular endothelial growth factor (VEGF) can be used in recurrent or progressive GBM
- another alkylating agent, lomustine, is often used as a second-line treatment or in recurrent gliomas

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31
Q

high yield gliomas

A

pilocytic astrocytoma (Grade 1)
diffuse astrocytoma (grade 2)
anaplastic astrocytoma (grade 3)
glioblastoma (grade 4)

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32
Q

pilocytic astrocytoma: grade

A

1

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33
Q

pilocytic astrocytoma: cell of origin

A

astrocyte

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34
Q

pilocytic astrocytoma: typical age

A

children, young adults

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35
Q

pilocytic astrocytoma: location

A

cerebellum, optic pathway

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36
Q

pilocytic astrocytoma: imaging

A

cystic mass with enhancing nodule

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37
Q

pilocytic astrocytoma: key molecular markers

A

BRAF-KIAA1549 fusion

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38
Q

pilocytic astrocytoma: MGMT status

A

not typically assessed

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39
Q

pilocytic astrocytoma: histology

A

biphasic (compact and loose areas), Rosenthal fibers

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40
Q

pilocytic astrocytoma: prognosis

A

excellent, often curable

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41
Q

pilocytic astrocytoma: treatment

A

surgery alone often curable

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42
Q

diffuse astrocytoma: grade

A

2

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43
Q

diffuse astrocytoma: cell of origin

A

astrocytes

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44
Q

diffuse astrocytoma: typical age

A

30-40 years

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45
Q

diffuse astrocytoma: location

A

cerebral hemispheres

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46
Q

diffuse astrocytoma: imaging

A

T2/FLAIR hyperintense, non-enhancing

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47
Q

diffuse astrocytoma: key molecular markers

A

IDH mutation, ATRX loss

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48
Q

diffuse astrocytoma: MGMT status

A

often methylated

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49
Q

diffuse astrocytoma: histology

A

low cellularity, no mitoses

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50
Q

diffuse astrocytoma: prognosis

A

5-year survival: 65-70%

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51
Q

diffuse astrocytoma: treatment

A

surgery, RT if progression

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52
Q

anaplastic astrocytoma: grade

A

3

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53
Q

anaplastic astrocytoma: cell of origin

A

astrocytes

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54
Q

anaplastic astrocytoma: typical age

A

40-50 years

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55
Q

anaplastic astrocytoma: location

A

cerebral hemispheres

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56
Q

anaplastic astrocytoma: imaging

A

T2/FLAIR hyperintense, may enhance

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57
Q

anaplastic astrocytoma: key molecular markers

A

IDH mutation, ATRX loss

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58
Q

anaplastic astrocytoma: MGMT status

A

often methylated

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59
Q

anaplastic astrocytoma: histology

A

increased cellularity, mitoses

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60
Q

anaplastic astrocytoma: prognosis

A

5-year survival: 25-30%

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61
Q

anaplastic astrocytoma: treatment

A

surgery, RT, +/- chemotherapy

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62
Q

glioblastoma: grade

A

4

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63
Q

glioblastoma: cell of origin

A

astrocytes

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64
Q

glioblastoma: typical age

A

50-60 years

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65
Q

glioblastoma: location

A

cerebral hemispheres

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66
Q

glioblastoma: imaging

A

enhancing mass with necrosis and edema

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67
Q

glioblastoma: key molecular markers

A

IDH wildtype (primary), EGFR amplification

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68
Q

glioblastoma: MGMT status

A

variable

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69
Q

glioblastoma: histology

A

high cellularity, mitoses, necrosis, microvascular proliferation

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70
Q

glioblastoma: prognosis

A

median survival: 12-15 months

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71
Q

glioblastoma: treatment

A

surgery, RT, chemotherapy (TMZ)

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72
Q

IDH

A

isocitrate dehydrogenase

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73
Q

ATRX

A

alpha-thalassemia/mental retardation syndrome X-linked

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74
Q

EGFR

A

epidermal growth factor receptor

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75
Q

MGMT

A

O6-methylguanine-DNA methyltransferase

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76
Q

TMZ

A

temozolomide

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77
Q

location of pediatric brain tumors by age: <3

A

supratentorial > infratentorial

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78
Q

location of pediatric brain tumors by age: 3-10 years old

A

infratentorial > supratentorial

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79
Q

location of pediatric brain tumors by age: 10+ years old

A

supratentorial = infratentorial

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80
Q

most common pediatric brain tumors: suprasellar

A

craniopharyngioma
optic hypothalamic glioma

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81
Q

most common pediatric brain tumors: overall

A

pilocytic astrocytoma
medulloblastoma
ependymoma

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82
Q

most common pediatric brain tumors: supratentorial/cerebral hemispheres

A

pilocytic astrocytoma
ganglioglioma
PXA pleomorphic xanthoastrocytoma

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83
Q

most common pediatric brain tumors: infratentorial/cerebellar

A

medulloblastoma
pilocytic astrocytoma
ependymoma

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84
Q

oligodendrogliomas: WHO grade

A

2 or 3

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85
Q

oligodendrogliomas: molecular markers

A

associated with 1p/19q co-deletion and IDH mutations
- 1p/19q co-deletion patients have a better overall prognosis compared to astrocytic tumors which are 1p/19q normal

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86
Q

oligodendrogliomas: imaging

A

partially calcified T2 heterogeneous, hyperintense subcortical/cortical mass with patchy or minimal contrast enhancement

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87
Q

oligodendrogliomas: location

A

most often found cortically in the frontal or temporal lobesq

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88
Q

oligodendrogliomas: typical age

A

4th or 5th decade of life

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89
Q

oligodendrogliomas: pathology

A

cells with a “Fried egg” appearance with monotonous round nuclei, surrounded by prominent perinuclear halos

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90
Q

oligodendrogliomas: treatment

A

surgical resection followed by radiation and chemotherapy

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91
Q

ependyma tumors classification

A

based on anatomic site, histopathology, and molecular features

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92
Q

supratentorial ependymomas: location

A

more frequently in frontal and parietal lobes

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93
Q

supratentorial ependymomas: imaging

A

irregular contrast enhancement, often with cysts or calcifications

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94
Q

supratentorial ependymomas: molecular findings

A

fusion gene involving ZFTA or YAP1

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95
Q

supratentorial ependymomas: treatment

A

surgical resection followed by radiation

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96
Q

posterior fossa ependymomas: location

A

most frequently in the fourth ventricle, can arise in the cerebellopontine angle

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97
Q

posterior fossa ependymomas: presentation

A

related to mass effect/hydrocephalus from fourth ventricular obstruction
- headache, vomiting, lethargy, rapid head circumference increase in infants

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98
Q

posterior fossa ependymomas: imaging

A

homogenous mass within the fourth ventricle

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99
Q

spinal ependymoma: location

A

intradural, intramedullary lesion of the spinal cord

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100
Q

spinal ependymoma: imaging

A

contrast-enhancing, well-circumscribed lesion often associated with syrinx

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101
Q

myxopapillary ependymoma: location

A

intradural, extramedullary lesion of the spinal cord, most often in the lumbar spine

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102
Q

myxopapillary ependymoma: imaging

A

contrast-enhancing, well-circumscribed lesion; often described as “sausage-like” in appearance

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103
Q

MYCN-amplified ependymoma: location

A

intradural, extramedullary lesion of the spinal cord, most often in the cervical spine

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104
Q

MYCN-amplified ependymoma: prognosis

A

aggressive compared to other ependymomas

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105
Q

spinal ependymomas

A

spinal ependymoma
myxopapillary ependymoma
MYCN-amplified ependymoma

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106
Q

subependymoma: grade

A

WHO grade 1

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107
Q

subependymoma: location

A

most often found incidentally in the ventricles

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108
Q

subependymoma: pathology

A

monomorphic cells with round/oval nuclei and salt and pepper chromatin and perivascular pseudorosettes

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109
Q

choroid plexus papilloma/carcinoma: cells of origin

A

choroid plexus cells

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110
Q

choroid plexus papilloma/carcinoma: typical age

A

often presents in infants, most occurring before age 1 but can occur at any age

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111
Q

choroid plexus papilloma/carcinoma: presentation

A

symptoms of increased ICP due to overproduction of CSF

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112
Q

choroid plexus papilloma/carcinoma: imaging

A

an intraventricular highly contrast-enhancing lesion usually located in the lateral or 4th ventricle

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113
Q

choroid plexus papilloma/carcinoma: treatment

A

surgical resection

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114
Q

neuronal and mixed neuronal-glial tumors

A

ganglioglioma
desmoplastic infantile astrocytoma and ganglioma
dysembrionic neuroepithelial tumor (DNET)
central neurocytoma

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115
Q

ganglioglioma: grade

A

low-grade tumor, but can be more aggressive

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116
Q

ganglioglioma: imaging

A

cystic mass with a mural nodule

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117
Q

ganglioglioma: location

A

often arising in the temporal lobe

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118
Q

ganglioglioma: presentation

A

can often lead to refractory epilepsy

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119
Q

ganglioglioma: typical age

A

most common in children and young adults

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120
Q

ganglioglioma: key molecular markers

A

BRAF mutations, IDH negative

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121
Q

ganglioglioma: pathology

A

biphasic tumors with neuronal and glial elements

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122
Q

which tumors are associated with BRAF mutations?

A

pilocytic astrocytoma
PXA
ganglioglioma

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123
Q

desmoplastic infantile astrocytoma and ganglioglioma: grade

A

WHO grade 1

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124
Q

desmoplastic infantile astrocytoma and ganglioglioma: typical age

A

often before age 2, more often in males

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125
Q

desmoplastic infantile astrocytoma and ganglioglioma: presentation

A

rapid increase in head size

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126
Q

desmoplastic infantile astrocytoma and ganglioglioma: imaging

A

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

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127
Q

desmoplastic infantile astrocytoma and ganglioglioma: treatment

A

surgical resection

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128
Q

desmoplastic infantile astrocytoma and ganglioglioma: location

A

meninges of frontal or parietal lobes

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129
Q

dysembrionic neuroepithelial tumor (DNET): grade

A

grade 1 tumor

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130
Q

dysembrionic neuroepithelial tumor (DNET): location

A

cortical gray matter associated with cortical dysplasia most often in temporal lobes

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131
Q

dysembrionic neuroepithelial tumor (DNET): complications

A

temporal lobe epilepsy

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132
Q

dysembrionic neuroepithelial tumor (DNET): pathology

A

myxoid clear areas with floating neurons

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133
Q

dysembrionic neuroepithelial tumor (DNET): imaging

A

wedge-shaped cystic cortical mass lesion with a “soap-bubble” appearance, typically non-enhancing

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134
Q

central neurocytoma: grade

A

grade 2

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135
Q

central neurocytoma: presentation

A

signs/symptoms of increased ICP

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136
Q

central neurocytoma: imaging

A

a cystic/bubbly lesion within the lateral ventricle near the foramen of Monro, often attached to the septum pellucidum

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137
Q

central neurocytoma: pathology

A

neurocytic rosettes and uniform round blue cells. they are “neurocytic” in morphology and may closely resemble oligodendrogliomas, but with no IDH mutation or 1p/19q co-deletion

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138
Q

central neurocytoma: treatment

A

surgical resection

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139
Q

pineal region tumors

A

pineoblastoma
pineocytoma

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140
Q

pineoblastoma: imaging

A

calcified heterogenous soft tissue mass in the pineal region with enhancement

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141
Q

pineoblastoma: typical age

A

seen primarily in children

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142
Q

pineoblastoma: presentation

A

can lead to hydrocephalus secondary to mass effect

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143
Q

pineoblastoma: pathology

A

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

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144
Q

pineoblastoma: treatment

A

gross total resection followed by radiation and chemotherapy

145
Q

pineocytoma: imaging

A

contrast-enhancing midline lesion with irregular borders

146
Q

pineocytoma: presentation

A

often present with symptoms of hydrocephalus or mass effect

147
Q

pineocytoma: pathology

A

small mature pinealocytes with round/ovoid nuclei forming pineocytomatous rosettes

148
Q

pineocytoma: treatment

A

often curative with surgical resection alone

149
Q

embryonal tumors

A

medulloblastoma

150
Q

medulloblastoma: frequency

A

most common malignant brain tumor in childhood (20-25% of pediatric CNS neoplasms)

151
Q

medulloblastoma: associations

A

Li-Fraumeni syndrome and familial adenomatous polyposis (FAP)

152
Q

medulloblastoma: imaging

A

a midline round lesion along the fourth ventricle between the brainstem and cerebellum

153
Q

medulloblastoma: pathology

A

Homer-Wright rosettes with no lumen, with necrosis, and pseudopalisades

154
Q

medulloblastoma: prognosis

A

can seed and develop disseminated metastatic disease all along the neuraxis

155
Q

medulloblastoma: treatment

A

gross total resection followed by craniospinal irradiation and chemotherapy

156
Q

tumors of the meninges

A

meningioma

157
Q

meningioma: imaging

A

well-circumscribed dural-based extra-axial diffuse and homogenous enhancing lesion with a “dural tail”

158
Q

meningioma: pathology

A

calcified concentric psammoma bodies with a “whorled architector”

159
Q

meningioma: associations

A

NF2 patients can present with multiple meningiomas

160
Q

meningioma: treatment

A

observation for asymptomatic low grade tumors
if symptomatic, resection is performed
radiotherapy is considered if the meningioma is WHO grade 3

161
Q

Foster Kennedy Syndrome

A

if located in the olfactory groove tumors (especially meningiomas) can cause Foster Kennedy Syndrome: unilateral visual loss with compressive optic atrophy in one eye and contralateral papilledema in the other, also with anosmia

162
Q

tumors of the cranial (and spinal) nerves

A

schwannoma
neurofibroma
optic nerve glioma
carcinomatosis meningitis
lymphomatous meningitis

163
Q

schwannoma: location

A

most commonly arise on CN VII/VIII complex but can arise on any cranial nerve except I and II, which do not have SChwann cells

164
Q

schwannoma: imaging

A

contrast enhancing mass usually within the internal auditory canal or cerebellar pontine angle

165
Q

schwannoma: pathology

A

areas of compact elongated cells with nuclear palisades called Verocay bodies

166
Q

schwannoma: association

A

bilateral acoustic schwannomas are pathognomonic for patients with NF2

167
Q

neurofibroma: grade and location

A

benign tumors, typically on peripheral nerves

168
Q

neurofigroma: associations

A

associated with neurofibromatosis type 1 (NF1)

169
Q

optic nerve glioma: location

A

typically occurs in CN II and along the visual pathway

170
Q

optic nerve glioma: types

A

astrocytomas, either fibrillary (60%) or pilocytic (40%)

171
Q

carcinomatosis meningitis: presentation

A

multiple cranial neuropathies

172
Q

lymphomatous meningitis: presentation

A

multiple cranial neuropathies

173
Q

lymphomas and hematopoietic tumors

A

hemangioblastoma
CNS lymphoma
intravascular lymphoma
hemangioma
multiple myeloma
langerhans cell histiocytosis

174
Q

hemangioblastoma: imaging

A

contrast-enhancing nodule surrounding a non-enhancing cyst in the posterior fossa with little vasogenic edema

175
Q

hemangioblastoma: pathology

A

large vacuolated stromal cells and variably sized small vascular structures

176
Q

hemangioblastoma: associations

A

commonly seen in patients with Von-Hippel Lindau (VHL) syndrome

177
Q

CNS lymphoma: imaging

A

can have a highly variable appearance ranging from diffusely infiltrative, to a solid margin, to lobulated margins
lesions can have variable enhancement and possible diffusion restriction

178
Q

CNS lymphoma: location

A

tend to be located within the basal ganglia or periventricular white matter and can involve the corpus callosum

179
Q

CNS lymphoma: immunocompetent patients

A

lesions more likely unifocal and are uniformly contrast enhancing

180
Q

CNS lymphoma: immunocompromised patients

A

lesions may be multifocal and are ring enhancing

181
Q

CNS lymphoma: pathology

A

positive staining for B cell marker, CD20

182
Q

CNS lymphoma: associations

A

the elderly and immunocompromised patients are at risk of CNS lymphoma, especially if EBV positive

183
Q

CNS lymphoma: treatment

A

standard initial treatment is combination therapy that includes high-dose methotrexate
in select patients, consolidative therapy with autologous stem cell transplant is performed after initial treatment

184
Q

intravascular lymphoma: presentation

A

presents as a B-cell driven disease that has multi-organ involvement that may involve the central nervous system
if CNS involvement, patients present with multiple small brain infarcts

185
Q

intravascular lymphoma: pathology

A

lymphoma cells within the blood vessels

186
Q

hemangiomas: imaging

A

lesion with a “popcorn” appearance with a perimeter of increased signal due to hemosiderin

187
Q

hemangiomas: complications

A

lesions at an increased risk of bleeding

188
Q

multiple myeloma: associations

A

will involve the bony structures and can lead to compression fractures

189
Q

multiple myeloma: imaging

A

salt and pepper pattern with diffuse heterogeneous lesions of vertebrae

190
Q

langerhans cell histiocytosis: cells of origin

A

due to uncontrolled monoclonal proliferation of Langerhans cells

191
Q

langerhans cell histiocytosis: pathology

A

Langerhans cells have large, convoluted, and folded nuclei and are CD1a positive

192
Q

langerhans cell histiocytosis: typical age

A

most common in children and female predominant

193
Q

langerhans cell histiocytosis: imaging

A

lytic lesions in the skull and severe vertebral compression fractures with vertebra plana configuration

194
Q

germ cell tumors

A

germinoma

195
Q

germinoma: epidemiology

A

most common pineal region neoplastic lesion

196
Q

germinoma: imaging

A

solid, contrast-enhancing midline pineal mass near the 3rd ventricle

197
Q

germinoma: pathology

A

small reactive lymphocytes and large neoplastic germ cells with clear cytoplasm
histologically similar to cells in the ovaries and testes

198
Q

germinoma: male/female

A

males much higher risk than females

199
Q

germinoma: associations

A

should be considered in patients who are young and develop diabetes insipidus

200
Q

sella region tumors

A

craniopharyngioma
pituitary macroadenoma
pituitary microadenoma
hypothalamic hamartoma

201
Q

craniopharyngioma: imaging

A

partially calcified cystic/solid multi-compartmental suprasellar mass

202
Q

craniopharyngioma: cells of origin

A

derived from Rathke pouch epithelium

203
Q

craniopharyngioma: differential diagnosis

A

Rathke cleft cyst (RCC)
suprasellar arachnoid cyst
hypothalamic/chiasmatic astrocytoma
pituitary adenoma
(epi-)dermoid tumors
thrombosed aneurysm
germinoma or mixed germ cell tumor with cystic compionent(s)

204
Q

Rathke cleft cyst (RCC) compared to craniopharyngioma

A

noncalcified, less heterogeneous, look for intracystic nodule, does not enhance, “claw” sign (enhancing pituitary draped around cyst)

205
Q

suprasellar arachnoid cyst compared to craniopharyngioma

A

noncalcified, no enhancement

206
Q

hypothalamic/chiasmatic astrocytoma compared to craniopharyngioma

A

solid or with small cystic/necrotic components, calcification is rare; robust enhancement is common

207
Q

pituitary adenoma compared to craniopharyngioma

A

rare in prepubescent children, isointense with the brain, enhances strongly, can mimic CP when cystic and hemorrhagic

208
Q

(epi-)dermoid tumors compared to craniopharyngioma

A

minimal or no enhancement

209
Q

thrombosed aneurysm compared to craniopharyngioma

A

contains blood products, look for residual patent lumen, phase artifact

210
Q

germinoma or mixed germ cell tumor with cystic component(s) compared to craniopharyngioma

A

CSF spread common, calcification rare

211
Q

pituitary macroadenoma: epidemiology

A

most common suprasellar mass in adults

212
Q

pituitary macroadenoma: presentation

A

can lead to compression of the optic chiasm

213
Q

pituitary macroadenoma: imaging

A

a large, enhancing sellar/suprasellar mass

214
Q

pituitary microadenoma: imaging

A

small focal hypointense sellar mass

215
Q

pituitary microadenoma: secretory

A

prolactinoma is most common secretory pituitary adenoma
bromocriptine and cabergoline can be used to suppress prolactin secretion

216
Q

hypothalamic hamartoma: imaging

A

a round, non-enhancing, iso-intense mass in the hypothalamus, superior to the infundibulum

217
Q

hypothalamic hamartoma: presentation

A

can present with precocious puberty and/or gelastic seizures

218
Q

cysts and other lesions

A

colloid cyst
epidermoid cyst
synovial cyst
rathke cleft cyst
arachnoid cyst
mucocele
lipoma

219
Q

colloid cyst: imaging

A

hyperdense non-enhancing epithelial-lined cyst of the foramen of Monro

220
Q

colloid cyst: presentation

A

at risk of developing obstructive hydrocephalus
- presents with intermittent or persistent headaches due to the increased intracranial pressure from obstruction of CSF outflow

221
Q

colloid cyst: typical age

A

peak age of onset is 3rd or 4th decade of life

222
Q

colloid cyst: treatment

A

microsurgically, endoscopically, or with biventricular shunts in nonsurgical candidates

223
Q

epidermoid cyst: imaging

A

an extra-axial mass with a cystic appearance
- will be low density and similar to CSF on CT scan, like an arachnoid cyst
- on MRI, both arachnoid and epidermoid cysts are hyperintense on T2, but with FLAIR arachnoid cysts become hypointense (like CSF) while epidermoid cysts usually stay hyperintense
- epidermoid cysts also have hyperintensity on DWI which helps to distinguish from an arachnoid cyst as well

224
Q

epidermoid cyst: compared to arachnoid cyst

A
  • on MRI, both arachnoid and epidermoid cysts are hyperintense on T2, but with FLAIR arachnoid cysts become hypointense (like CSF) while epidermoid cysts usually stay hyperintense
  • epidermoid cysts also have hyperintensity on DWI which helps to distinguish from an arachnoid cyst as well
225
Q

synovial cyst: location

A

can form adjacent to any joint throughout the body
if they occur in the spine, they can occur in the cervical, thoracic, or lumbar regions

226
Q

synovial cyst: imaging

A

well-circumscribed, smooth, extra-dural lesion adjacent to facet joints
calcification within the cyst can also occur

227
Q

rathke cleft cyst: imaging

A

cystic lesion of the pituitary stalk

228
Q

synovial cyst: presentation

A

radicular pain and symptoms related to compression of the adjacent root

229
Q

rathke cleft cyst: presentation

A

commonly present with pituitary dysfunction

230
Q

arachnoid cyst: location

A

extra-axial, congenital, space-occupying lesions that displace parenchymal brain tissue and are often asymptomatic

231
Q

arachnoid cyst: imaging

A

fluid collection that is isodense/isointense to CSF on all sequences (CT, MRI, FLAIR, DWI)

232
Q

mucocele: imaging

A

opacification of the sinus

233
Q

mucocele: at risk

A

at risk of abscess formation

234
Q

lipoma: imaging

A

an intradural extramedullary sharply circumscribed homogenous mass that suppresses on STIR fat-suppressed sequences

235
Q

lipoma: location

A

if found intracranially, they may be near the corpus callosum

236
Q

milial tumor syndromes of the CNS

A

neurofibromatosis type 1
neurofibromatosis type 2
tuberous sclerosis
sturge-weber syndomre
von-hippel lindau
li-fraumeni
cowden syndrome
turcot syndrome

237
Q

NF1: aka

A

von Recklinghausen disease

238
Q

NF1: genetics

A

autosomal dominant disease related to the NF1 gene, which codes for neurofibromin

239
Q

NF1: non-neurologic findings

A

cafe-au-lai spots, Lisch nodules, skeletal abnormalities, and axillary freckling

240
Q

NF1: neurologic lesions

A

optic nerve gliomas
neurofibromas

241
Q

NF1: optic nerve gliomas

A

patients should have yearly eye exams
typically found in the first 6 years of life
small asymptomatic gliomas can be observed, as they may regress
larger, symptomatic lesions are treated with chemotherapy

242
Q

NF1: neurofibromas

A

can occur on cutaneous branches of peripheral nerves, including neuronal roots and plexuses
they tend to be slow-growing and can remodel the surrounding bone
lesions encase the nerve and are contrast enhancing

243
Q

plexiform neurofibromas

A

seen in NF1 but not seen in NF2

244
Q

NF2: aka

A

central neurofibromatosis

245
Q

NF2: genetics

A

autosomal dominant disorder related to the NF2 gene, which encodes for merlin on chromosome 22

246
Q

NF2: presentation

A

vestibular schwannomas and multiple meningiomas

247
Q

NF2: presentation

A

can have bilateral acoustic neuromas, patients usually present in their 20s with tinnitus

248
Q

NF2: of note

A

these patients do not form neurofibromas

249
Q

NF2: mnemonic

A

NF2 is a story of 2s: 2 nerves affected (typically CN VII or CN VIII), often on 2 sides (bilateral), 2 tumors (schwannomas, meningiomas), chromosome 22

250
Q

tuberous sclerosis: genetics

A

autosomal dominant neurocutaneous disorder related to two tumor-suppressor genes: TSC1 (9q34) which encodes hamartin and TSC2 (16q13) which encodes tuberin

251
Q

TSC1 genetics

A

9q36 which encodes hamartin

252
Q

TSC2 genetics

A

16q13 which encodes tuberin

253
Q

TS: imaging

A

cortical tubers which can be foci for seizures, which can be foci for seizures

254
Q

TS: non-neurologic

A

ash-leaf spots, facial angiofibromas, angiomyolipomas, and retinal hamartomas

255
Q

TS: association

A

intellectual disability occurs in several cases

256
Q

TS: subependymal giant cell astrocytomas

A

5-10% of patients develop SEGAs

257
Q

TS: mnemonic

A

TUBERRRS: Tsc2, U(ventricular), Bulk removal, Eosinophilic Rhabdomyosarcoma/Renal angiomyolipoma/Retinal hamartomas, Seizures

258
Q

sturge weber syndrome: aka

A

encephalotrigeminal angiomatosis

259
Q

sturge weber syndrome: gene

A

neurocutaneous disorder caused by a spontaneous somatic GNAQ gene mutation on chromosome 9 that manifests in a mosaic pattern
because this mutation is somatic (occurs after conception) it is not inherited

260
Q

sturge weber syndrome: presentation

A

classic “port-wine stain” (cutaneous angioma) on the face with ipsilateral angiomatosis

261
Q

sturge weber syndrome: imaging

A

CT head: parenchymal calcification adjacent to the angiomatosis
MRI: contrast enhancing angiomatosis of the leptomeninges and cortex (seen as “tram track sign” on MRI)

262
Q

sturge weber syndrome: presentation

A

variable presentation from asymptomatic to seizures, weakness, etc

263
Q

Cobb’s syndrome

A

sturge weber syndrome with spinal dural angioma in the spinal cord with a port-wine stain in the corresponding dermotome

264
Q

Von Hippel Lindau (VHL): genetics

A

autosomal dominant disorder that leads to the upregulation of vascular endothelial growth factor (VEGF)

265
Q

Von Hippel Lindau (VHL): presentation

A

infratentorial hemangioblastomas and retinal angiomas

266
Q

Von Hippel Lindau (VHL): non-CNS manifestations

A

renal cell carcinoma, pheochromocytomas, and pancreatic and renal cysts

267
Q

Von Hippel Lindau (VHL): mnenoic

A

VHL: Visceral cysts (kidney, pancreas, genitals), Hemangioblastomas, Light-sensitive (i.e. retinal involvement)

268
Q

Li-Fraumeni: genetics

A

autosomal dominant disorder due to mutations of the p53 gene

269
Q

Li-Fraumeni: presentation

A

tumors at various sites including breast, bone, blood, and adrenal glands

270
Q

Li-Fraumeni: CNS

A

astrocytomas, medulloblastomas, and less commonly primitive neuroextodermal tumors (PNETs)

271
Q

Cowden syndrome: genetics

A

autosomal dominant disease secondary to the PTEN tumor suppressor gene

272
Q

Cowden syndrome: presentation

A

multiple hamartomas in various tissues, especially the skin and colon
young adults with cerebellar dysfunction or obstructive hydrocephalus

273
Q

Cowden syndrome: association

A

associated with Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma)

274
Q

Cowden syndrome: pathology

A

abnormal ganglion cells

275
Q

Turcot syndrome: aka

A

mismatch repair cancer syndrome

276
Q

Turcot syndrome: presentation

A

multiple colon polyps and an increased risk of colorectal and brain cancers (glioblastoma, medulloblastoma, SGCA)

277
Q

metastatic cancer

A

intraparenchymal metastasis
leptomaningeal carcinomatosis

278
Q

intraparenchymal metastasis: imaging

A

lesions of intense contrast enhancement surrounded by mild to moderate edema usually at the grey-white junction

279
Q

intraparenchymal metastasis: common sources

A

lung (~40-50%)&raquo_space; breast (~15-20%) > melanoma and GI (~10-% each)

280
Q

intraparenchymal metastasis: causing hemorrhage

A

melanoma and renal cell carcinoma most likely to cause hemorrhage

281
Q

intraparenchymal metastasis: meningeal infiltration

A

breast cancer

282
Q

tip for which organs metastasize to the brain

A

when in doubt, paired organs (lungs, breast, and kidneys) and surface organs (skin, GI tract) typically metastasize to the brain

283
Q

leptomeningeal carcinomatosis: imaging

A

contrast enhancement along the leptomeninges

284
Q

leptomeningeal carcinomatosis: work up

A

when on differential, patients should have the whole neuraxis imaged to look for additional lesions as well as CSF analysis

285
Q

leptomeningeal carcinomatosis: presentation

A

obstruction of CSF re-absorption from the malignancy can lead to communicating hydrocephalus
severe headaches, nausea, and vomiting
can improve with large volume lumbar puncture or EVD placement

286
Q

paraneoplastic disorders: presentation

A

symptoms often precede cancer diagnosis and thus patients require cancer surveillance for a minimum of 2 years after symptom onset

287
Q

paraneoplastic disorders

A

limbic encephalitis
paraneoplastic cerebellar degeneration
lambert-eaton myasthenic syndrome (LEMS)
others (myasthenia gravis, subacute sensory neuronopathy, opsoclonus-myoclonus syndrome, stiff-person syndrome, neuro-myotonia, dysautonomia, myelopathy)

288
Q

limbic encephalitis: associated

A

most associated with SCLC, testicular germ cell tumors, or ovarian teratomas

289
Q

limbic encephalitis: antibodies

A

several are related including anti-Hu, voltage-gated potassium channels, NMDA receptors, etc

290
Q

limbic encephalitis: CSF

A

slightly more than half of patients with autoimmune limbic encephalitis have detectable CNS antibodies

291
Q

limbic encephalitis: presentation

A

psychiatric/cognitive abnormalities, seizures, facial and limb dyskinesias, and autonomic instability

292
Q

limbic encephalitis: imaging

A

abnormalities in cortical and subcortical regions

293
Q

limbic encephalitis: EEG

A

NMDA-related encephalitis can have delta brush pattern

294
Q

limbic encephalitis: young women

A

should be evaluated for ovarian teratoma

295
Q

paraneoplastic cerebellar degeneration: presentation

A

subacute progression of ataxia, nystagmus, and dysarthria

296
Q

paraneoplastic cerebellar degeneration: common causes

A

gynecological (ovarian, uterine, breast) and small cell lung cancers

297
Q

paraneoplastic cerebellar degeneration: pathology

A

profound loss of Purkinje cells

298
Q

paraneoplastic cerebellar degeneration: antibody

A

anti-Yo is the anti-Purkinje cell antibody

299
Q

paraneoplastic cerebellar degeneration: MRI

A

often normal, may have some enhancement near the cerebellum, and may have cerebellar atrophy

300
Q

paraneoplastic cerebellar degeneration: recovery

A

slow or partial (postmortem studies show permanent loss of Purkinje cells)

301
Q

LEMS: presentation

A

proximal weakness and fatigability, impotence, and ptosis

302
Q

LEMS: association

A

paraneoplastic encephalomyelitis and cerebellar degeneration

303
Q

LEMS: cancer

A

most often SCLC, 70% have cancer

304
Q

myasthenia gravis

A

thymoma

305
Q

opsoclonus-myoclonus syndrome: presentation

A

rapid, high-amplitude conjugate eye movements (opsoclonus), myoclonus, ataxia

306
Q

opsoclonus-myoclonus syndrome: related to

A

neuroblastoma in children and breast, ovarian, or small cell lung cancer in adults

307
Q

opsoclonus-myoclonus syndrome: antibody

A

anti-Ri/ANNA-2 or anti-Hu

308
Q

stiff person syndrome: antibody

A

anti-amphiphysin (paraneoplastic) or anti-GAD (more often autoimmune than paraneoplastic)

309
Q

acetylcholine receptor, MUSK: tumor

A

thymoma

310
Q

acetylcholine receptor, MUSK: neurologic symptoms

A

myasthenia gravis

311
Q

voltage-gated calcium channel (VGCC) antibody: tumor

A

small cell lung cancer

312
Q

voltage-gated calcium channel (VGCC) antibody: neurologic symptoms

A

LEMS

313
Q

neuronal ganglionic acetylcholine receptor antibody: tumor

A

adenocarcinoma
thymoma

314
Q

neuronal ganglionic acetylcholine receptor antibody: neurologic symptoms

A

autonomic neuropathy

315
Q

voltage-gated potassium channel (VGKC) complex (LGI1 and CASPR2): tumor

A

variable

316
Q

voltage-gated potassium channel (VGKC) complex (LGI1 and CASPR2): neurologic symptoms

A

limbic encephalitis
faciobrachial dystonic seizures (LGI1)

317
Q

LGI1

A

faciobrachial dystonic seizures

318
Q

NMDA receptor antibody: tumor

A

ovarian teratoma

319
Q

NMDA receptor antibody: neurologic symptoims

A

limbic encephalitis

320
Q

ANNA-1 (anti-Hu): tumor

A

small cell lung cancer

321
Q

ANNA-1 (anti-Hu): neurologic symptoms

A

limbic encephalitis, opsoclonus-myoclonus

322
Q

ANNA-2 (anti-Ri): tumor

A

small cell lung cancer, breast cancer

323
Q

ANNA-2 (anti-Ri): neurologic symptoms

A

opsoclonus-myoclonus, encephlomyelitis, limbic encephalitis

324
Q

ANNA-3 antibody: tumor

A

small cell lung cancer

325
Q

ANNA-3 antibody: neurologic symptoms

A

limbic encephalitis, myelopathy, peripheral neuropathy

326
Q

PNMA-1 (anti-Ma1): tumor

A

testicular cancer, breast cancer, colon cancer

327
Q

PNMA-1 (anti-Ma1): neurologic symptoms

A

limbic encephalitis, brainstem encephalitis

328
Q

PNMA-2 (anti-Ma2): tumors

A

testicular

329
Q

PNMA-2 (anti-Ma2): neurologic symptoms

A

limbic encephalitis, brainstem encephalitis

330
Q

PCA-1 (anti-Yo): tumor

A

gynecologic cancers, breast cancer

331
Q

PCA-1 (anti-Yo): neurologic symptoms

A

cerebellar degeneration

332
Q

CRMP-5 (anti CV2): tumor

A

small cell lung cancer, thymoma

333
Q

CRMP-5 (anti CV2): neurologic symptoms

A

chorea, myelopathy, peripheral neuropathy

334
Q

anti-GAD: tumor

A

small cell lung cancer, thymoma

335
Q

anti-GAD: neurologic symptoms

A

Stiff-person syndrome, cerebellar ataxia, limbic encephalitis

336
Q

anti-amphiphysin: tumor

A

breast cancer

337
Q

anti-amphiphysin: neurologic symptoms

A

stiff-person syndrome

338
Q

intraventricular tumors: lateral ventricle

A

subependymoma, choroid plexus tumors

339
Q

intraventricular tumors: third ventricle

A

colloid cyst, SGCA/SEGA, central neurocytoma

340
Q

intraventricular tumors: fourth ventricle

A

ependymoma, medulloblastoma

341
Q

dural based tumors

A

meningioma, metastases

342
Q

sellar tumors

A

pituitary adenoma, craniopharyngioma, rathke cleft cyst

343
Q

pineal tumors

A

germinoma, pineal tumors

344
Q

intramedullary/intraparenchymal tumors

A

astrocytoma, ependymoma

345
Q

intradural extramedullary/extraparenchymal tumors

A

meningioma, neurofibroma, lipoma

346
Q

extradural extramedullary/extraparenchymal tumors

A

metastasis, bone-related tumors (lagnerhans cell histiocytosis, multiple myeloma, chordoma)

347
Q

chordoma

A

notochord-derived tumor
imaging: a heterogeneous enhancing lesion usually located with the clivus or sacrum

348
Q

cyst and mural nodule

A

hemangioblastoma, pilocytic astrocytoma

349
Q

calcified

A

meningioma, oligodendroglioma, low-grade astrocytoma, ependymoma, craniopharyngioma

350
Q

contrast

A

metastasis, meningioma, astrocytoma, schwannoma

351
Q

hemorrhagic

A

metastasis, high-grade astrocytoma, lymphoma, hemangioblastoma

352
Q

transthyretin

A

choroid plexus papilloma

353
Q

TSC1

A

hamartoma

354
Q

TSC2

A

tuber

355
Q

p53

A

astrocytoma

356
Q

CDKN2A

A

astrocytoma

357
Q

PTEN

A

astrocytoma

358
Q

gain ch7, loss of 9p

A

astrocytoma

359
Q

glial fibrillary acidic protein (GFAP) which is expressed in astrocytes and ependymal cells

A

astrocytoma, oligodendroglioma, ependymoma, gangliocytoma, hemangioblastoma