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Tumors Flashcards

1
Q

Are CNS tumors metastatic or primary?

A

50-50 both

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2
Q

What type of tumors present as multiple, well-circumscribed lesions at the gray-white junction?

A

Metastatic tumors

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3
Q

What are common sources of metastatic tumors in the brain?

A

lung, breast, kidney

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4
Q

How are primary tumors classified?

A

cell type origin

–> astrocytes, meningothelial, ependymal, oligodendrocytes, etc

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5
Q

Are adult CNS tumors supratentorial or infratentorial>

A

supratentorial (meninges and cerebrum)

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6
Q

What are the most common CNS tumors in adults?

A

glioblastoma
schwannoma
meningioma

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7
Q

Are children CNS tumors supratentorial or infratentorial?

A

infratentorial (cerebellum and brainstem)

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8
Q

What are the most common CNS tumors in children?

A

pilocystic astrocytoma
medulloblastoma
ependymoma

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9
Q

Do primary CNS tumors metastasize?

A

No, locally destructive

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10
Q

What tumor is a common primary malignant, high-grade tumor of astrocytes in adults?

A

GBM/ glioblastoma

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11
Q

What tumor arises in the cerebral hemispheres and characteristically crosses the corpus callosum (butterfly lesion)?

A

glioblastoma

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12
Q

Does glioblastoma have a good prognosis?

A

no–> grade 4/4 astrocytoma

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13
Q

What tumors are GFAP +?

A

ASTROCYTOMAS:
glioblastoma
pilocytic astrocytoma

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14
Q

Briefly describe the histology of glioblastoma

A

regions of necrosis surrounded by pseudo-palisading tumor cells and endothelial cell proliferation

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15
Q

What should you immediately think of if you have an adult patient with new onset of seizures?

A

brain tumor!

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16
Q

What is a benign tumor of astrocytes and is the most common CNS tumor in children?

A

pilocytic astrocytoma (1/4)

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17
Q

Where are pilocytic astrocytomas usually found?

A

cerebellum (kids)

rare adult–>cerebrum

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18
Q

What tumor is characteristically described as a well-circumscribed, cystic lesion with a mural nodule?

A

pilocytic astrocytoma

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19
Q

What does biopsy of a pilocytic astrocytoma show?

A

Rosenthal fibers, eosinophilic granular bodies

biphasic pattern (loose and dense) with hair-like cells that have bipolar processes (AKA pilocyte)

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20
Q

What tumor grade has low proliferative potential and can be cured by resection (AKA benign)?

A

grade 1/4

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21
Q

What tumor grade is infiltrative with low proliferative potential and likely to recur?

A

grade 2/4

–>cytological atypia

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22
Q

What tumor grade is anaplastic with mitosis and requires radiation and chemo for treatment?

A

grade 3/4

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23
Q

What tumor grade is rapid evolution with fatal outcome and microvascular proliferation and/or necrosis?

A

grade 4/4

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24
Q

What are the 4 histological parameters of gliomas?

A

nuclear atypia
mitosis (mitotic figures)
microvascular proliferation
necrosis

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25
Q

What tumor grade has 1 glioma parameter?

A

2

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26
Q

What tumor grade has 2 glioma parameters?

A

3

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27
Q

What tumor grade has 3-4 glioma parameters?

A

4

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28
Q

What tumor grade is a diffuse astrocytoma?

A

2

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29
Q

What tumor grade is a anaplastic astrocytoma?

A

3

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30
Q

What is the general rule of age versus astrocytoma tumor grade?

A

lower grade–>young
high grade–>old

2–>30-40s
3–>50s
4–>60+

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31
Q

Are astroctyomas grey or white matter lesions?

A

white matter–>surround neurons (center of brain, outside of spinal cord)

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32
Q

If there was a brainstem astrocytoma, what CN dysfunction would you see?

A

CN VI, also long tract and brainstem signs

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33
Q

What does a diffuse astrocytoma look like?

A

raw scallop (vs cooked scallop being nml white matter)

grade 2

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34
Q

How can you differentiate diffuse vs anaplastic astrocytomas?

A

2 vs 3

pearly look vs atypia and numerous cells

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35
Q

What age does pilocytic astrocytoma affect?

A

under 20

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36
Q

What gene predisposes you to pilocytic astrocytoma, especially on optic nerve fibers?

A

NF1

–> functional loss of neuofibromin

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37
Q

What genes are involved in primary glioblastomas?

A

later in life without precursor lesion

EGFR and PTEN

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38
Q

What mutations confer a better prognosis in glioblastoma?

A

IDH1 and IDH2

–> R132H mutation of IDH1

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39
Q

Secondary glioblastomas occur…

A

in younger patients that are preceded by a lower grade lesion
—> TP53

typically treated as kid, years later glioblastoma forms

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40
Q

Where can you find glioblastomas?

A

throughout the brain, crosses corpus callosum to spread

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41
Q

What does glioblastoma look like?

A

contrast ring enhancing

central necrosis

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42
Q

What are the 3 histology hallmarks of glioblastoma?

A

1) serpentine necrosis

2) pattern
pseudo-palisading cells around necrosis (perpendicular walls around necrosis)

3) vascular or endothelial proliferation

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43
Q

What tumor makes up 10-15% of gliomas and occurs primarily in adults in the cerebrum?

A

oligodendroglioma

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44
Q

What is the hallmark term for oligodendrogliomas?

A

calcification

–>restricted to cortex with curvilinear or gyriform distribution

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45
Q

Describe histology of oligodendroglioma

A

Fried egg (perinuclear halos)

Chicken wire (anastomosing capillaries)

surround neurons, vasculature and pia mater

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46
Q

What grade are most oligodendrogliomas?

A

2/4, well differentiated

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47
Q

What mutated genes are the most common in oligodendrogliomas?

A

IDH1 and IDH2

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48
Q

Do IDH1 and 2 confer a favorable prognosis in oligodendroglioma?

A

yes

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49
Q

What gene loss is favorable in oligodendrogliomas?

A

1P19Q loss

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50
Q

What other CNS tumor is similar to the prognosis of glioblastoma (bad)?

A

anaplastic oligodendroglioma (3/4)

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51
Q

What tumor has vascular hypertrophy and necrosis, retains geometric vascularity, increases with increased N:C ratio and cellularity, and is found as nodules in 2/4 tumors?

A

anaplastic oligodendroglioma (3/4)

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52
Q

What tumor is sown as a calcified tumor in the white matter, usually along the frontal lobe, that may present with seizures?

A

oligodendroglioma

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53
Q

When does an ependymoma occur?

A

under 20 years

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54
Q

Where is the most common site of a ependymoma?

A

supratentorial in the 4th ventricle–> discrete, exophytic (stick out), enhancing

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55
Q

What grade is ependymoma typically?

A

3/4

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56
Q

Histology of ependymoma

A

ependymal rosettes more diagnostic than perivascular rosettes (pathoma says vice versa)

57
Q

What tumor is most common in children in lateral ventricles and causes obstructive hydrocephalus d/t increased CSF production?

A

choroid plexus papilloma

58
Q

What tumor is:
-in young adults and attached to the roof of the 3rd ventricle

  • causes obstructive (non-comm) hydrocephalus
  • rapidly fatal
  • positional
A

colloid cyst of 3rd ventricle

59
Q

What is the main malignant embryonal tumor in children?

A

medulloblastoma (4/4)

60
Q

What are the 4 molecular group associated with medulloblastoma?

A

WNT–> older kids, monosomy Chr 6 with 90% 5 year survival

SHH–> MYCN amp with intermediate prognosis

Group 3: MYC amp and 17(I17Q) in infants and kids, WORST PROGNOSIS

Group 4: I17Q, no MYC, poor prognosis

61
Q

Where can you find medulloblastoma?

A

cerebellum in midline, occludes CSF

62
Q

Histology of medulloblastoma

A

anaplastic cell sheets (small, round, blue cells)

abundant mitoses

Homer-Wright rosettes

63
Q

How does medulloblastoma spread?

A

CSF to cauda equina

64
Q

What is medulloblastoma derived from?

A

neuroectoderm of cerebellum

65
Q

What are differential dx for medulloblastoma?

A

ependymoma (perivascular pseudorosettes but less cellular and more fibrillar)

pilocytic astrocytoma (cystic, biphasic, low cellularity)

high grade infiltrating astrocytoma (more pleomorphic)

atypical teratoid/rhabdoid tumor (AT/RT less than 2yo, large cells)

metastatic carcinoma (adult patients)

66
Q

Where is AT/RT found?

A

atypical teratoid/rhabdoid tumor

posterior fossa or supratentorial

67
Q

What components are found in AT/RT?

A

EMA

Vimentin

68
Q

What cells are found in AT/RT?

A

Rhabdoid cells–>eosinophilic cytoplasm with sharp borders and eccentric nucleus

—>resemble rhabdomyosarcoma

69
Q

What gene/chromosome do 90% of people with AT/RT have?

A

Chr 22, hSNF5/INI1

70
Q

What is the prognosis of AT/RT?

A

less than 1 year survival, highly aggressive

4/4

71
Q

Who would get a primary CNS lymphoma?

A

immunocompromised (AIDS) or transplant patient

frequency increases over 60

72
Q

How do primary CNS lymphomas spread?

A

multifocal periventricular spread

involvement outside CNS is late and rare

73
Q

What is the origin of primary CNS lymphoma?

A

B cell (CD20+)

aggressive with latent EBV or organ transplant

74
Q

Histology of primary CNS lymphoma

A

HOOPING accumulate around vessels

–>cells separated by reticulin and silver stain

75
Q

Where do primary germ cell tumors in the brain occur?

A

midline

76
Q

What are some CNS germ cell tumors?

A

pineal and suprasellar

77
Q

M or F predominance of pineal germ cell tumor?

A

Male

78
Q

When do germ cell tumors occur?

A

90% under 20

10% Japanese

79
Q

Can gonadal germ cells metastasize to CNS?

A

yes and common

80
Q

Does germinoma respond to radiation and chemo?

A

yes, good response

81
Q

What is the most common pineal tumor?

A

germinoma

82
Q

What pineal tumor is well differentiated (neuronal), low grade and in adults?

A

pinecytoma

83
Q

What pineal tumor is high grade with necrosis and mitosis, in kids, spreads throughout CNS and has RB gene?

A

pineblastoma

84
Q

What is the most common benign CNS tumor in adults?

A

meningioma

85
Q

When does meningioma occur?

A

after 30 years

–>slow growing but compresses brain d/t attached to dura (can penetrate bone)

86
Q

What tumor is radiation induced?

A

meningioma (1/4)

87
Q

What mutation should you consider if multiple meningiomas?

A

NF2 (22q12)

higher grade tumor

88
Q

Do sporadic meningiomas have the NF2 gene?

A

yes, 50-60%

89
Q

What is the most common gene in meningioma besides NF2?

A

TRAF7

lower histo grade and mora stable than NF2

90
Q

What is the origin of meningioma?

A

benign tumor of arachnoid cells in dura

EMA+

91
Q

Gross appearance of meningioma

A

calcified psammoma bodies

en plaque (sheet like spread of tumor along surface of dura)

92
Q

Histology of meningioma

A

whorled cluster of monotonous cells

psammoma bodies

93
Q

What meningioma subtype has PAS+ droplets, TRAF7 and KFL4 mutations?

A

secretory

94
Q

Describe atypical 2/4 meningioma

A

more aggressive with increased recurrence

XRT mutation, increased cellularity and mitoses

more prominent nucleoli and necrosis

clear and chordoid 2/4 more aggressive

95
Q

Describe anaplastic/malignant 2/4 meningioma

A

resembles high grade sarcoma

high mitosis

papillary and rhabdoid subtypes

96
Q

M or F predominance of meningiomas

A

Women, specifically pregnancy (increases frequency of presentation, not incidence)

97
Q

What do 70% of meningiomas express?

A

PR (progesterone receptor)

98
Q

What accounts for 1/2 intracranial tumors in hospitalized patients?

A

metastatic carcinoma (metastatic brain tumors)

99
Q

What are common sources of metastatic brain tumors?

A

lung>breast>melanoma>kidney>GI

100
Q

What rare tumor frequently mets to brain?

A

choriocarcinoma

101
Q

What is a common malignant tumor but rarely mets to brain?

A

prostate

102
Q

What causes hemorrhage in met tumor of brain?

A

melanoma
choriocarcinoma
renal cell carcinoma
lung

103
Q

What is a common sit of mets in metastatic brain tumors?

A

meninges

–>gray/white jxn or intraperenchymal

104
Q

Metastatic brain tumor in epidural or subdural space can cause…

A

spinal cord compression

105
Q

What is carcinomatosis

A

tumor nodules spread along surface of brain and spinal cord

–> originate from lung and breast

106
Q

What paraneoplastic syndrome is associated with an ovarian teratoma and the NMDA receptor?

A

anti-NMDA receptor encephalitis in hippocampal neurons

sx: psych, memory -, seizures, dyskinesia, language dysfxn

107
Q

What paraneoplastic syndrome is associated with anti-HU antibody in neurons?

A

encephalomyelitis d/t small cell lung cancer

AKA ANNA-1 Ab

108
Q

What paraneoplastic syndrome is associated with antibodies against voltage-gated potassium channels?

A

encephalitis d/t peripheral neuropathy

109
Q

What paraneoplastic syndrome is associated with opsoclonus-myoclonus ataxia syndrome?

A

dancing eyes, dancing feet

NEUROBLASTOMA in kids
small cell lung ca in adults

110
Q

What paraneoplastic syndrome is associated with antibodies to voltage-gated calcium cannels?

A

Lambert-Eaton myasthenic syndrome

small cell lung cancer

111
Q

What is the treatment of paraneoplastic manifestations?

A

immunotherapy to remove circulating Ab

removal of primary tumor

112
Q

What antibody circulates in subacute cerebellar degeneration (paraneoplastic syndrome)?

A

PCA-1 in women with ovarian, uterine or breast carcinoma

destroys purkinje cells, gliosis and mild inflammatory infiltrate

113
Q

What peripheral nerve sheath tumor is S100+?

A

schwannoma

114
Q

Where do schwannomas occur?

A

in cranium: CN VIII @ cerebellar pontine angle

also involves cranial and spinal nerves

115
Q

What condition shows bilateral schwannomas?

A

NF2 (neurofibromatosis type 2)

116
Q

What gene is implicated in schwannoma?

A

NFR d/t loss of merlin

117
Q

Histology of schwannomas

A

mix of Antoni A and B

A: spindle cells with verocay bodies (palisading nuclei around nuclear free zones)

B: hypocellular, myxoid ECM

118
Q

What do schwannoma patients present with?

A

acoustic neuroma (misnomer): tinnitus and hearing loss d/t CN VIII

119
Q

What tumor can transform into malignant peripheral nerve sheath tumors (MPNST)?

A

plexiform neurofibroma (NF1 or sporadic)

AKA bag of worms

120
Q

Describe neurofibromas

A

benign nerve sheath tumors

121
Q

Are MPNST high or low grade?

A

85% HIGH grade, half arise in NF1 patients

122
Q

What is the inheritance pattern of neurofibromatosis type 1?

A

AD

123
Q

Describe neurofibromatosis type 1 (NF1)

A

neurofibromas of peripheral nerves

optic nerve gliomas

lisch nodules (pigmented nodules of iris)

cafe au lait spots (hyperpig cutaneous nodules)

124
Q

Is NF1 or 2 more common?

A

NF1

125
Q

What is the inheritance of NF2?

A

AD (Same as type 1)

126
Q

Describe neurofibromatosis type 2

A

BL schwannomas (CN VIII) @ cerebellopontine angle

increased meningiomas and ependymomas (have NF2 gene mutation)

127
Q

What inheritance is von hippel-lindaud disease?

A

AD

128
Q

Describe von hippel-lindaud disease

A

hemangioblastomas of CNS (benign tumor of vessels in cerebellum and retina)

cysts of pancreas, liver and kidney

renal cell carcinoma

pheochromocytoma

129
Q

What gene is implicated in von hippel-lindaud disease?

A

VHL on chromosome 3

nml downregulates hypoxia induced factor and VEGF, erythropoietin, etc

130
Q

What do you get in von hippel-lindaud disease due to the deletion of VHL?

A

polycythemia (too many RBC)

VHL normally regulates erythropoietin–>since deleted, continuously made

131
Q

What inheritance pattern is tuberous sclerosis?

A

AD

132
Q

What are sx of tuberous sclerosis?

A

seizures, autism, mental retardation

133
Q

Describe tuberous sclerosis

A

Harmatomas (beingn tumors)

cortical tubers (seizure-inducing)

renal angiomyolipomas

cardiac rhabdomyomas

subungal fibromas

shagreen patches (localized cutaneous thickening)

ash-lead patches (hypopigmented areas)

134
Q

What genes are implicated in tuberous sclerosis?

A

TSC1 (chr 9, hamartin)

TSC2 (chr 16, tuberin)

135
Q

What familiar tumor has a mutation in PTEN–>IK3/AKT signaling pathway that leads to dysplastic gangliocytoma?

A

cowden syndrome (AD)

136
Q

What familiar tumor has mutations in TP53 and medulloblastomas?

A

li-fraumeni syndrome (AD)

137
Q

What familial tumor has mutations in APC with medulloblastoma or glioblastoma?

A

turcot syndrome (AD)

138
Q

What familial tumor has mutations in PTCH gene–>increase in sonic hedgehog signaling pathway with medullobalstoma?

A

gorlin syndrome (AD)

Neuro (18 decks)

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