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Putthoff Lectures/CIS > Part 4 > Flashcards

Part 4 Flashcards

1
Q

Babies and children with failure to thrive, seizures, or crawling problems (floppy baby syndromes) associated with?

A

Genetic metabolic disorders:Tay Sachs–neuronal storage disorder–neuronal accumulation and neuronal deathLeukodystrophies–AR mutations associated with myelinMitochondrial encephalomyopathies–CNS and muscle; increase lactate levels in tissues, heteroplasmy

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2
Q

Leigh syndrome

A

mitochondrial encephalomyopathies–disease of infancyseizures, hypotonia, lactic acidosis

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3
Q

MELAS

A

mitoch encpmyopathy; lactic acidosis; STROKE LIKE episodes

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4
Q

MERRF

A

MYOCLONIC EPILEPSY and RAGGED RED FIBERS

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5
Q

pyramidal vs extrapyramidal

A

pyramidal: Cst and cbt; umn; motor fxnsextrapyramidal: involuntary reflexes and modulation of movement

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6
Q

tardine dyskinesia associated with what drug

A

benedryll

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7
Q

Vit B1 vs B12 def

A

B1 def: wernickes encephalopathy and korsakoffB12: subacute combined degeneration of spinal cord

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8
Q

Methanol can cause

A

blindness

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9
Q

Most neoplasms in adults in brain are of what lineage

A

GLIAL neoplasms of astrocytic lineage

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10
Q

In adults where are astrocytomas generally foundependymomas?Medulloblastomas?Cerebellar astrocytoma?pilocytic astrocytoma?

A

cerebral cortices 4th ventriclecerebellumposterior fossaposterior fossa

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11
Q

Most malignancies of adults are in what area of the brain?Children?

A

Adults: CEREBRAL CORTEXChildren: posterior fossa!!

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12
Q

Poorly differentiated glioma–is called what?

A

MEDULLOBLASTOMA

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13
Q

Infiltrating astrocytomas–diffuse vs anaplastic

A

diffuse=lower gradeanaplastic=higher grade

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14
Q

Glioblastoma is usually from what lineage?

A

usually astrocytic but can be oligodendrocytic or ependymal

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15
Q

Primary vs secondary glioblastomas

A

primary arises deNovo (cerebrocortical single mass lesion)secondary glioblastomas: arises from antecedent lower grade glial lesions and progress to increasing severity over time

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16
Q

Most common subtype of primary glioblastomas

A

Classic subtype

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17
Q

Most common subtype associated with secondary glioblastomas

A

Proneural type

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18
Q

IDH1 vs IDH2 in glioblastomas (oligodendrogliomas??)

A

IDH2 has better prognosis

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19
Q

Primary brain tumor vs infarction morphology

A

primary brain tumor shows edema but infarction typically does not

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20
Q

Where are glioblastomas found in adults?

A

cerebral cortices

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21
Q

Glioblastoma morphology

A

circumstene (serpentine??) necrosis with pseudopalisading with endothelial proliferation (atypical multiluminal vascular structures), significant atypia, sometimes giant cells and sometimes gemistocytic

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22
Q

Localized glial lesions

A

pleomorphic xanthoastrocytoma (children)–usually temporal lobe; good prognosisbrainstem gliomaspilocytic astrocytomasoligodendrogliomasanaplastic oligodendrogliomasependymomas

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23
Q

Oligodendroglioma morphology

A

coarse calcifications in cerebral cortex

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24
Q

ependymomas found where

A

paraventricular mass lesion

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25
Q

Adults with spinal ependymomas associated with?

A

NF2!!

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26
Q
  1. pilocytic astrocytoma, pleomorphic xanthoastrocytoma and brainstem glioma–what age?2. brainstem glioma where in children vs adults
A
  1. children2. brainstem in children and diffuse in adults and can be a glioblastoma
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27
Q

What is this neoplasm: cystic gelatinous serous mass, neural nodule in posterior fossa in children?

A

can be either a pilocytic astrocytoma or a cerebellar hemangioblastoma

28
Q

Oligodendroglioma grading

A

is from 1-4 with 4/4 being a glioblastomaMost are 2/4; if anaplastic 3/4; if 4/4 its a glioblastoma

29
Q

pilocytic astrocytoma grading

A

1/4

30
Q

Oligodendroglioma morphology and prognosis

A

well circumscribed cyst, focal hemorrhage, calcification, anastomosing capillaries; IDH1 and IDH2 (better prognosis)

31
Q

Ependymoma grading

A

2/4 but can have anaplastic types

32
Q

Myxopapillary ependymomas occurs where? Associated with?

A

cauda equina; other ependymomas usually occur at 4th ventricleassociated with NF2

33
Q

Ependymoma vs medulloblastoma location

A

if from floor of 4th ventricle, its ependymoma and if from roof of 4th ventricle its a medulloblastoma

34
Q

choroid plexus papillomas

A

rare–too much CSF production

35
Q

colloid cyst of 3rd ventricle

A

leads to obstructive hydrocephalus that is episodic

36
Q

medulloblastoma vs ependymoma ages

A

medulloblastoma under 10 yearsependymoma upt0 20 or 30 years of age

37
Q

How to tell bw primary lesion and metastatsis

A

primary lesion only has one lesion; metastasis usually many lesions and primary lesions more likely to have edema!

38
Q

Neuronal tumors

A

Gangliomas: tumor with neuronal differentiation–mostly in adults, presents with seizures, most often in temporal lobeDysembryonic neuroepithelial tumor: present with seizures, most common in children in TEMPORAL LOBEcentral neurocytoma: within ventricular system

39
Q

CNS primitive neuroectodermal tumor (PNET); blue cell tumor; see ROSETTES

A

Medulloblastoma

40
Q

4 groups with differing prognosis in medulloblastoma

A

WNT–older children best prognosisSHH–infants young adultsGroup 3–MYC amplification iscochromosome 17 infants/children–WORST PROGNOSISGroup 4: isochromosome 17 without MYC amplification; SOME MYC amplification–intermediate prognosis

41
Q

Highly aggressive tumor of very young and has similar location to MB but find skeletal muscle in itresponse to chemotheraphy vs MB?

A

Atypical teratoid/rhabdoid tumor (AT/RT)Does not respond as well to chemotherapy as MB

42
Q

MBs and ependymomas must administer chemotherapy how

A

intrathecally bc expands from 4th ventricle into CSF so can get reoccurrence

43
Q

CNS lymphoma vs other lymphomas

A

CNS lymphoma are multifocal, B cell associated; associated with EBV; STAYS in CNS and does not go out of it; other lymphomas do NOT involve the CNS!

44
Q

Intravascular lymphoma

A

in B cell but tends to be infiltrative and often gets misdiagnosed as angiosarcoma

45
Q

Most testicular tumors are what in origin

A

germ cell tumors

46
Q

where are germ cell tumors usually found in brain in who? What are germ cell tumors called in brain vs testicles

A

pineal gland; males; japanesetesticular=seminomas; brain=germinomas

47
Q

Pineal parenchymal tumors–what kind of differentiation; grading?

A

have NEURONAL differentiation; high grade in children but low grade in adults

48
Q

Usually dural based and benign (but can still kill you if it compresses vital structures)

A

Meningiomas

49
Q

Who gets meningiomas?

A

adults

50
Q

characteristic morphologic feature of meningiomas

A

psammoma bodies (round concretions of calcium salts) but not just limited to meningiomas but also found in thyroid tumors and ovarian tumors

51
Q

2 types of meningiomas and clinical features

A

microcstic or secretory; benign but may cause headaches

52
Q

Can meningiomas be anaplastic?

A

YES; if in difficult location to excise; but NEVER metastasizes outside of CNS

53
Q

What kind of neoplasms exhibit accelerated growth during pregnancy

A

meningiomas and pigmented lesions (nevus)

54
Q

Metastatic tumors to the brain are usually primary tumors of what organs?

A

Lung (small cc), breast (infiltrating ductal cc) , skin (melanoma) (BCC and squamous CC–less likely), kidney (RCC), GI tract (adenocarcinoma–colon)

55
Q

How are paraneoplastic syndromes referred to the brain different from other paraneoplastic syndromes?

A

-causes encephalomyelitis–both spinal cord and brain involvement–motor manifestations and bulbar and eye signs-Peripheral NS manifestations–peripheral neuropathies like in alcoholics or diabetics

56
Q

Li-Fraumeni syndrome associated with what mutation?

A

p53

57
Q

brain tubers (malalignments, atypical proliferations of glial cells and neural cells in cerebral cortices that form foci that give rise to seizures), hemartomas (giant cells in ventricles but not true neoplasm), renal angiomyolipomas, pulmonary lymphangioleiumyomatosis (women–grows in preg), cardiac rhabdomyomas all associated with??

A

TUBEROUS SCLEROSIS COMPLEX

58
Q

Von Hippel Lindau disease associated with

A

cysts (including visceral), hemangioblastomas (cerebellar or retinal), RCC, pheochromocytoma, secondary polycythemia (elaborate erythropoetin from cerebellar hemangioblastomas)

59
Q

NF1 and NF2–AD or ARwhich more commoneach associated with?

A

both ADNF1 more common than NF2NF2 associated with schwannomas, meningiomas and ependymomasNF1 associated with malignancy (MPNST)

60
Q

Similarities and differences bw schwannomas and neurofibromas

A

Both have schwann cell lineagebut schwannomas found on pedicle of peripheral nerve sheath while neurofibromas expand nerves

61
Q

Neurofibromas found in NF1 or NF2?

A

both but more common in NF1

62
Q

Loss of merlin, see carney complex; Baracade bodies?–what neoplasm?

A

schwannoma–associated with CPA (acoustic neuroma) also associated with visual/occipital portion invovement

63
Q

Neurofibromas–multiple superficial cutaneous neurofibromas–diffuse vs plexiform

A

plexiform=when sectioning neurofibromas, there are locules (septations that divide substance)–associated with malignant peripher nerve sheath tumors

64
Q

schwannoma vs neurofibroma malignancy

A

shwannoma never malignant but neurofibromas can be esp if plexiform; NO SUCH THING AS MALIGNANT SCHWANNOMA

65
Q

Trinton tumor

A

subtype of MPNST–contains heterologous material like skeletal muscle , glands, etc (can look like teratoma)

Putthoff Lectures/CIS (4 decks)

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