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Neuro Exam 2 > Nervous System Malignancies > Flashcards

Nervous System Malignancies Flashcards

1
Q

3 characteristics of neoplasms

A

*autonomous - growth is independent of physiologic growth signals
*clonal - acquired mutations affect cell and its progeny
*proliferative - mutations confer cell survival and growth advantage

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2
Q

benign vs. malignant neoplasms

A

*differentiation - how well do neoplastic cells resemble normal?
*invasion - is there infiltration/destruction of surrounding tissues?
*metastasis - has neoplasm spread to physically discontinuous sites?

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3
Q

behavior of neuro neoplasms

A

*benign vs. malignant distinction less evident in the brain:
-confined space in skull
-widely infiltrate the brain parenchyma
-may be lethal if situated in critical brain region, even if “benign”

*patterns of spread differs from other organ systems
-may spread within CNS through the CSF
-rarely metastasize to other organ systems

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4
Q

primary neuro neoplasms

A

*from cells intrinsic to the nervous system
*locally infiltrative and destructive
*approx 66% of neuro neoplasms

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5
Q

metastatic neuro neoplasms

A

*from other organ systems (LUNG, breast, kidney, skin)
*multiple, well-circumscribed lesions at gray-white matter junction
*approx 33% of neuro neoplasms

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6
Q

typical adult neuro neoplasms

A

*SUPRAtentorial/cerebral hemispheres
*types: glioblastoma, meningioma, schwannoma

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7
Q

typical childhood neuro neoplasms

A

*INFRAtentorial/posterior fossa
*account for nearly 20% of all cancers in childhood
*types: pilocytic astrocytoma, ependymoma, medulloblastoma

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8
Q

risk factors of neuro neoplasms

A

*most CNS tumors are sporadic
*about 1% are hereditary
*immunosuppression - strongly associated with primary CNS lymphoma
*RADIATION is a strong risk factor, esp for gliomas and meningiomas

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9
Q

neurofibromatosis type 1

A

*inherited neoplastic syndrome
*gene = NF1 (17q11)
*tumor types = neurofibroma, pilocytic astrocytoma

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10
Q

neurofibromatosis type 2

A

*inherited neoplastic syndrome
*gene = NF2 (22q12)
*tumor types = schwannoma, meningioma, ependymoma

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11
Q

tuberous sclerosis

A

*inherited neoplastic syndrome
*gene = TSC1/TSC2 (9q34/16p13)
*tumor types = subependymal giant cell astrocytoma

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12
Q

Von Hippel-Lindau

A

*inherited neoplastic syndrome
*gene = VHL (3p25)
*tumor type = hemangioblastoma

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13
Q

Li-Fraumeni

A

*inherited neoplastic syndrome
*gene = TP53 (17p13)
*tumor types = gliomas

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14
Q

primary neuro neoplasms - classification

A

*glial tumors/gliomas (astrocytomas, oligodendrogliomas, ependymomas)
*embryonal tumors (medulloblastomas)
*meningiomas
*nerve sheath tumors (schwannomas)

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15
Q

what types of things cause ring-enhancing lesions on MRI of brain

A

*high-grade gliomas (ex. glioblastomas)
*abscesses
*central nervous system toxoplasmosis
*organizing infarcts

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16
Q

gliomas - overview

A

*neoplasms of glial cell origin (non-specific)
*types: astrocytomas, oligodendrogliomas, ependymomas
*tissue biopsy required to confirm dx

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17
Q

gliomas - general characteristics

A

-no recognizable premalignant or in situ stages
-produce major clinical abnormalities
-rarely spread outside CNS

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18
Q

gliomas - signs and symptoms

A

*focal OR generalized signs/symptoms:
-headaches, seizures, aphasia, vomiting, visual disturbances
-cognitive dysfunction (memory + mood disorders)
-intracranial pressure may be increased
-suspected on basis of imaging studies (MRI)

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19
Q

grade I glioma

A

*slow growing and unlikely to spread
*survival > 5 years; curable if excised
*general category = benign (pilocytic astrocytoma)

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20
Q

grade II glioma

A

*unlikely to grow and spread, but more likely to come back after treatment
*survival > 5 years; tendency to recur
*general category = low grade malignant (oligodendroglioma, diffuse astrocytoma)

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21
Q

grade III glioma

A

*grow quickly with rapidly dividing cells, but no necrosis
*survival 2-3 years
*general category = high grade malignant (anaplastic astrocytoma)

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22
Q

grade IV glioma

A

*grow and spread quickly with rapidly dividing cells; has vascular proliferation and necrosis
*survival < 1 year
*general category = high grade malignant (glioblastoma)

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23
Q

astrocytoma - pathogenesis

A

*mutation of IDH1 (or IDH2)
*mutated genes encode forms of isocitrate dehydrogenase with new enzymatic activity
*presence of IDH1/2 mutations predicts better prognosis in all grades of astrocytic tumors

24
Q

astrocytomas - overview

A

*neoplasms of astrocytes
*specific type of glioma

25
Q

classifications of astrocytomas

A

*non-infiltrative/localized: pilocytic astrocytoma (grade I)
*infiltrative:
-diffuse astrocytoma (grade II)
-anaplastic astrocytoma (grade III)
-glioblastoma (grade IV)

26
Q

pilocytic astrocytoma - overview

A

*BENIGN neoplasm of astrocytes (grade I); non-infiltrative
-most common benign CNS tumor in children
-very slow growing, usually arises in cerebellum

27
Q

pilocytic astrocytoma - pathogenesis

A

*mutations/translocations involving BRAF (functions in RAS signaling pathway)
*IDH1 mutations NOT seen

28
Q

pilocytic astrocytoma - appearance

A

*discrete CYSTIC lesion with mural nodules
*biphasic architecture: loose “microcystic” and compact, densely fibrillar areas
*ROSENTHAL FIBERS: bright-red corkscrew-shaped astrocyte inclusion

29
Q

infiltrative astrocytomas - overview

A

*MALIGNANT neoplasms of astrocytes (grade II-IV)
*account for 80% of adult gliomas, usually in the 40s-60s
*low-grade tumors progress to high-grade tumors
*on imaging, low grade is non-enhancing, and high-grade is enhancing

30
Q

diffuse astrocytoma

A

*grade II (infiltrative) astrocytoma
*most common in young adults
*IDH1 mutations
*transition between neoplastic and normal tissues not distinct:
-infiltrate diffusely
-increased cellular astrocyte proliferation with atypia
-mitoses RARE
-microvascular proliferation + necrosis ABSENT

31
Q

anaplastic astrocytoma

A

*grade III (infiltrative) astrocytoma
*IDH1 mutations
*transition between neoplastic and normal tissues not distinct:
-mitoses increased
-microvascular proliferation
-necrosis ABSENT

32
Q

glioblastoma

A

*grade IV (infiltrative) astrocytoma
*most common and most malignant primary brain tumor or adults (commonly crosses corpus callosum - “butterfly” lesion)
*NOT associated with IDH1, unless progression from lower grade
*transition between neoplastic and normal tissues not distinct:
-highly pleomorphic
-mitoses numerous
-extensive endothelial proliferation and areas of necrosis

33
Q

oligodendrogliomas

A

*malignant neoplasm of oligodendrocytes
*constitute 5-15% of gliomas
*best prognosis among glial tumors (survival of 10-20 years, depending on grading)

34
Q

oligodendrogliomas - gene defect

A

*IDH1/2 mutation
AND
*deletion of 1p and 19q

35
Q

oligodendrogliomas - clinical features

A

patients may have had several years of neurologic complaints, often including seizures

36
Q

oligodendrogliomas - gross features

A

*usually frontal lobe
*predilection for white matter
*gelatinous, gray masses, often cystic, focal hemorrhages, and calcifications

37
Q

oligodendrogliomas - histological features

A

*regular cells with spherical nuclei
*clear halo cytoplasm, “fried eggs”
*grade II = low mitotic activity
*grade III = anaplastic, higher cell density, anaplasia, increased mitoses, necrosis

38
Q

ependymomas - overview

A

*malignant neoplasm of ependymal cells
*10% of pediatric brain tumors (typically posterior fossa)
*in adults, typically spinal cord (22q deletion ; NF2 gene mutation)

39
Q

ependymomas - clinical features

A

*posterior fossa -> non communicating hydrocephalus (progressive obstruction of 4th ventricle)
*CSF dissemination uncommon

40
Q

ependymomas - pathology

A

*cells with fibrillary processes contain regular nuclei
*canals = glandlike elongated structures with lumen (resembling embryologic ependymal canal)
*pseudorosettes = perivascular nuclear-free zones

41
Q

embryonal tumor (medulloblastoma) - overview

A

*malignant neoplasm of cerebellar stem cells
*accounts for almost 20% of pediatric tumors
*most tumors located cerebellum midline
*molecular groups vary in terms of age of onset, tumor location, and prognosis

42
Q

embryonal tumor (medulloblastoma) - clinical features

A

*posterior fossa -> non communicating hydrocephalus (progressive obstruction of 4th ventricle)
*CSF dissemination: meningeal “icing” or spinal “drop mets”
*highly malignant; however, exquisitely radiosensitive

43
Q

embryonal tumor (medulloblastoma) - pathology

A

*densely cellular, undifferentiated small blue cells (embryonal)
*Homer-Wright rosettes surrounding central neuropil

44
Q

meningioma - overview

A

*predominantly benign neoplasms of arachnoid meningothelial cells
*found along any of the external surfaces of the brain (usually attached to dura)
*most common benign CNS tumor in adults (rare in children)

45
Q

meningioma - pathogenesis

A

*loss of long arm chromosome 22 (22q deletion)
-including NF2 gene, which encodes the protein merlin
-about 50% of sporadic meningiomas have loss-of-function mutations of NF2
*increased risk with prior radiation therapy to head/neck

46
Q

meningioma - clinical features

A

*slow-growing, solitary tumors
*vague non-localizing, or focal referable symptoms to adjacent brain compression
*may express variety of hormones (grow rapidly during pregnancy)

47
Q

meningioma - gross features

A

*round mass attached to dura (may compress underlying brain)
*common sites:
-parasagittal convexity
-lateral convexity
-sphenoid wing, olfactory groove, sella turcica

48
Q

meningioma - histologic features

A

*push, rather than infiltrate
*compact clusters/whorls of cells
*psammomatous calcifications
*malignant variants = high grade)

49
Q

CNS metastatic disease

A

*metastatic meningeal or parenchymal brain lesions
*account for 25-50% of intracranial tumors

50
Q

CNS metastatic disease - clinical features

A

*may be first manifestation of systemic disease
*present as mass lesions: may be multiple; meningeal carcinomatosis
*common primary sites = LUNG, breast, skin, kidney, GI

51
Q

CNS metastatic disease - pathology

A

*intraparenchymal sharply demarcated masses (usually at gray-white matter junction)
*typically resemble parent tumor type (phenotyping helpful for identifying the primary neoplastic site)

52
Q

schwannoma - overview

A

*benign neoplasm of Schwann cells
*nerve sheath tumors
*arise directly from peripheral or cranial nerves (may arise within dura)
*inactivating mutations of NF2 gene on chromosome 22

53
Q

schwannoma - clinical features

A

*local compression of involved nerve or adjacent structures
*dural: acoustic neuroma (attached VIII: vestibular branch; present with tinnitus and hearing loss)
*extradural
*surgical removal is curative

54
Q

schwannoma - gross features

A

*well-circumscribed, firm gray encapsulated masses
*attached loosely to associated nerve without invading
*lesion displaces nerve of origin as it grows
*can often be resected without sacrificing nerve function

55
Q

schwannoma - histologic features

A

*Schwann cells with spindled/wavy, elongated nuclei
*a mixture of dense and loose zones:
-Antoni A = dense cellular areas
-Antoni B = loose cellular areas

Neuro Exam 2 (24 decks)

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