CNS Tumors Flashcards

1
Q

Where do most adult CNS tumors occur?

A

in cerebral hemispheres, most freq in frontal lobes

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2
Q

Where do most childhood CNS tumors occur?

A

posterior fossa

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3
Q

List some key general features of astrocytomas

A

most common glial tumor
80% are glioblastomas
present with seiuzres, focal neuro deficits (gradual), headaches
diffuse tend to become anaplastic over time

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4
Q

What are the various grades/names for astrocytomas?

A

grade 1-pilocytic astrocytoma
grade 2- diffuse astrocytoma
grade 3-anaplastic astrocytoma
grade 4-glioblastoma multiforme

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5
Q

Describe diffuse astrocytoma histology

A

cellularity moderately increased and occasional nuclear atypia

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6
Q

Describe anaplastic astrocytoma histology

A

increased cellularity distinct nuclear atypia, marked mitotic activity

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7
Q

Describe glioblastoma multiforme histology

A

pleomorphic astrocytic cells, brisk mitotic activity, prominent microvascular proliferation
has necrosis with pseudopalisading

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8
Q

Describe pilocytic astrocytoma histology

A

biphasic pattern: densely fibrillary (pilocytic) areas alternating with microcystic component
rosenthal fibers

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9
Q

What are key clinical features of pilocytic astrocytoma?

A

cerebellum, also optic nerve, 3rd ventricle, hypothalamus, brainstem and occasionally cerebral hemispheres
1st 2 decades
presents with focal neurologic deficits, seizures, or S/S of increased intracranial pressure

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10
Q

What does pilocytic astrocytoma look like on imaging?

A

well demarcated, often cystic contrast-enhancing tumor

mural nodule

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11
Q

What is a significant mutation realted to oligodendromglioma?

A

loss of 1p and 19q are predictors of prolonged survival and susceptibility to chemotherapy in anaplastic oligodendrogliomas

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12
Q

What are some key clinical features of oligodendroglioma?

A

5th to 6th decade
long history of progressive neuro sx (seizures, headache, focal signs)
better survival than astrocytomas (5-10 yrs for grade II)

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13
Q

What does an oligodendroglioma look like on imaging?

A

well defined hypodense/hypointense mass, may see calcification

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14
Q

What does oligodendroglioma histology look like?

A

round nuclei, freid egg cell and calcifications

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15
Q

What are key features of ependymomas?

A

children and young adults
occurs along ventricular system, usually posterior fossa (4th ventricle)
hydrocephalus, occasonially seizures
avg survival 4 yrs
may invade brain parenchyma
may disseminate through subarachnoid space

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16
Q

What does ependymoma look like on imaging?

A

well circumscribed mass

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17
Q

What is seen on histology for ependymoma?

A

true rosettes-columnar cells arranged around central lumen
perivascular pseudorosettes
anaplastic ependymoma-increased mitoses & vascular proliferation

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18
Q

What are key features of choroid plexus papillomas?

A

1st 2 decades
4th ventricle, lateral ventricle, 3rd ventricle, cerebello-pontine angle
presents with hydrocephalus
can progress to choroid plexus carcinoma (usually in children)
good prog unless progresses to carcinoma

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19
Q

How can you distinguish a choroid plexus papilloma from a choroid plexus carcinoma/

A

papillomas do not invade adjacent parenchyma but carcinomas do

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20
Q

What are the key features of colloid cysts?

A

usually attached to roof of 3rd ventricle
intermittent obstruction of foramen of monro (positional)
positional headache, thin walled cyst lined by cuboid/columnar epithelium

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21
Q

What are the eky features of ganglioglioma?

A

first 3 decades
long standing history of seizures is common
usually supretentorial and in temporal lobe
surgery usually curative

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22
Q

What does a ganglioglioma look like on imaging?

A

solid or cycstic well circumcribed mass, mural nodule, calcification

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23
Q

What does histology of a ganglioglioma look like?

A

atypical ganglion cells (neurons) and neoplastic glial component

24
Q

What are the key clinical features of medulloblastoma?

A

primitive neuroectodermal neoplasm of posterior fossa
first decade
cerebellar dysfunction
incrased intracranial pressure
solid, well defined homogenous mass, tendency to spread through subarachnoid space and for drop metastases

25
What does a medulloblastoma look like on imaging?
well defined, contrast enhancing mass, may see leptomeningeal spread
26
What does medulloblastoma look like on histology?
homer wright rosettes synaptophysin immunoreactivity highly cellular and composed of undifferentiated cells
27
What are the key features of primary CNS lymphomas?
``` 40-60 yo typically immunocompirmised more common, EBV assoc non specific symptoms, referable to mass lesion usually Bcell poor prognosis usually supratentorial ```
28
What are key features of meningiomas?
most common extraparenchymal neoplasm of CNS middle to late adult life usually women sx due to enlarging mass
29
How do meningiomas look on imaging?
dural based, vascular, contrast enhancing, well defined
30
What are 2 associations with meningiomas?
radiation tx | NF2
31
Describe the 3 grades of meningiomas
1(typical): excellent 5 yr survival 2 (atypical): high mitotic index or architectural loss, hypercellularity, macronucleoli, small cell differentiation or brain invasion 3 (anaplastic): excessive mitotic activity or loss of meningothelial differentiation
32
What does meningioma histology look like?
sheets of tumor cells with indistinct borders whorls round to oval nuclei, dispersed chromatin, wispy eosinophilic cytoplasm psammomma bodies necrosis, brain invasion
33
What are common clinical signs/sx of metastatic turmos to the CNS?
headaches focal neurologic signs altered mental status
34
How mets to the CNS usually look on imaging?
distinct, contrast enhancing mass with surrounding edema, usually multiple
35
Where to mets to the CNS usually come from?
lung or breast | sometimes kidney, GI, skin (malignant melanoma)
36
Hwat are key features of peripheral nerve sheath tumors (schwannomas)?
``` benign tumor composed of schwann cells 4th to 6th decades peripheral nerves, usaully in head and neck (ie vestibulocochlear) spinal tumors-radicular pain hearing loss, facial numbness well definted contrast enhancing mass associated w/NF2 ```
37
What does schwannoma histology look like?
antoni A: compact spindle cells | antoni B: loose spongy areas, small cells with round nuclei
38
What is a neurofibroma?
benign tumor of schwann cells, fibroblasts and perineural cells associated with NF1 cutaneous neorfibroma or peripheral nerve forms
39
What does neurofibroma histology look like?
hypocellular, elongated spindle cells with wavy nuclei | diffusely infiltrate adjacent nerve and soft tissue
40
What are key features of plexiform neurofibroma?
almost exclusively in NF1 transformation of mutiple fascicles of nerves into neurofibroma with preservation of anatomic configuration typically affects larger nerves or a plexus high likelihood of malignant transformation
41
What are key features of malignant peripheral nerve sheath tumors?
``` usually in extremities in CNS assoc w/CN V strong assoc w NF1 high grade, agressive infiltrative non-encapsulated fleshy masses ```
42
What does malignant peripheral nerve sheath tumor histology look like?
highly cellular, moderate to marked nuclear pleomorphism | high mitotic rate
43
What are key features of neurofibromatosis type 1? (von Recklinghausen disease)
AD 1/3000 neurfibromas, cafe au lai spots, lisch nodules (pigmented hamartomas in iris), optic glioma, osseous lesions, axillary freckling, family hx
44
Where is the neurofibromatosis 1 gene found?
chromosome 17 | gene product: neurofibromin
45
What are key features of NF2?
``` AD 1/40,000-50,000 gene on chromosome 22 gene product is merlin bilateral vestibular schwannomas, fam hx, meningiomas, schwannomas, gliomas, neurofibromas, lens opacity, cerebral calcifications ```
46
What chromosome is the VHL gene found on?
3
47
What are key features of von hippl lindau disease?
hemangioblastomas of CNS and retina renal cell carcinoma pheochromocytoma visceral cysts
48
What do hemangioblastomas look like on imaging?
well-defined, contrast enhancing cystic mass with mural nodule
49
What do hemangioblastomas look like on histology?
numerous vessels interspersed wit hstromas cells | stromal cells have abundant foamy cytoplasm (contains fat)
50
What are key features fo tuberous sclerosis?
AD casued by mutations in TSC1 (chr 9)-hamartin & TSC2 (chr 16)-tuberin cortical hamartomas subcortical glioneuronal hamrtomas subependymal giant cell astrocytomas cutaneous angiofibromas, subungual fibromas, cardiac rhabdomyomas, intestinal polyps, etc
51
What do tubers look like on histology?
neuronas haphazardly arranged in cortex | often have glial as well as neuronal features
52
What are key features of subependymal giant cell astrocytoma?
cause obstructive hydrocephalus exophytic solid well-defined mass arising in the wall of lateral ventricle large pleomrophic multinucleated tumor cells w/eosinophilic cytoplasm may be of astrocytic or glioneuronal origin no malignant transformation, local invasion reported
53
What malignancies most commonly cause paraneoplastic syndromes?
``` small cell carcinoma gynecologic malignancies hodgkin's and non-hodgkins lymphoma testicular cancer neuroblastoma ```
54
What are the 2 main categories or paraneoplastic syndromes?
1. related to ectopic hormone production | 2. neurologic syndromes (rare)
55
Describe subacute cerebellar ataxia
a paraneoplastic neurologic syndrome progressive ataxia, dysarthria, nystagmus, vertigo, diplopia, titubation associated w/ ovarian and breast cancer ab to purkinje cells
56
Describe Lambert Eaton Myasthenic syndrome
a paraneoplastic neurologic syndrome muscle weakness, esp in the legs that improves with testing on exam (extraocular muscle are spared) Ab to P/Q type voltage gated calcium channels, leads to decreased ACh release most commonly assoc w/SCLC