Tumors Flashcards

1
Q

Where are a majority of childhood primary brain tumors located

A

Cerebellum & brainstem

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2
Q

In adults what is the most common tumor of the CNS

A

glioblastoma

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3
Q

In children what is the most common tumor of the CNS

A

Embryonal

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4
Q

Describe the grading of brain tumors

A

Grade I - low proliferative potential
Grade II - Infiltrative (likely to recur)
Grade III - Radiation chemo (anaplasia & mitoses)
Grade IV - Fatal outcome (necrosis & microvascular proliferation)

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5
Q

What are the four histological parameters of gliomas

A

Nuclear atypia
Mitoses
Microvascular proliferation
Necrosis

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6
Q

If someone has an oligodendroglioma what is the grade

A

II/IV unless it is anaplastic then it is III/IV

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7
Q

Where do astrocytomas usually develop

A

White matter

in SC can present as CN VI dysfunction

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8
Q

Describe pilocytic astrocytomas

A

first two decades of life

appear in cerebral hemispheres for adults and cerebellum for children

NF1 predisposes

Well circumscribed often cystic with mural nodule

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9
Q

Describe the histology of pilocytic astrocytomas

A

Biphasic patterns (loose glial with cystic changes & dense piloid tissue)

Hair like cells with long bipolar processes

Rosenthal fibers

Eosinophilic granular bodies

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10
Q

Describe glioblastomas

A

most common primary brain neoplasm

Contrast ring enhancing, with hypodense central necrosis

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11
Q

Differentiate primary vs secondary glioblastoma

A

primary: later in life no precursor lesion

secondary low grade (TP53) lesion. IDH1 better prognosis than IDH2

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12
Q

what are the three histology hallmarks of glioblastoma

A

Necrosis (serpentine)
Pseudopalisading
Vascular/endothelial proliferation

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13
Q

Describe Oligodendroglioma

A

10-15% of all gliomas, primarily in adults
Calcification restricted to cortex

on histo: perinuclear (fried egg halos); chicken wire anastomosing capillaries

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14
Q

Describe ependymomas

A

first two decades of life
fourth ventricle most common site, spinal for adult

on histo looks like scream painting

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15
Q

What are ependymal rosettes

A

true rosette: tubular structure with central canal; more diagnostic than perivascular rosettes

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16
Q

What embryonal tumor makes up 20% of tumors in children

A

medulloblastoma

17
Q

What are the 4 molecular GRPS

A

WNT (older kids, monosomy 6, B-catenin 90% survival 5 yrs)

SHH (infants -young adults)

MYC (infants and children Worst progrosis)

Group 4 17q. no MYC +/- MYCN poor prognosis

18
Q

Describe atypical teratoid/rhabdoid tumor

A

Posterior fossa or supratentorial

Divergent differentiation
Rhabdoid cells: eosinophilic cytoplasm with sharp boarders & eccentric nucleus, resemble Rhabdomyosarcoma

> 90% have chr 22; HSNF5/INI1
<1 year survival

19
Q

Describe the etiology of primary CNS lymphoma

A
Immunosuppressed individuals (AIDS) 
B-cell origin CD20 aggressive with EBV 

Accumulate around vessels = hooping

20
Q

What is the most common pineal tumor

A

Germinoma of the pineal body.

pineocytoma: well differentiated LG in adults
pineoblastoma: high grade necrosis mitoses HG kids (RB gene spreads throughout CNS)

21
Q

Why do meningiomas develop rapidly in pregnant women?

A

70% express progesterone receptors and will ballon during pregnancy

22
Q

What are the most common places to have tumors metastasize from to reach the brain

A
Lung 
Breast
Melanoma 
Kidney 
GI

Meninges is common site of met

23
Q

Describe cowden syndrome

A

Dysplastic gangliocytoma PTEN -> IK3/AKT signaling mutation

24
Q

Describe Li-Fraumeni syndrome

A

Medulloblastomas; mutations in TP53

25
Q

Describe Turcot syndrome

A

MEdulloblastoma or Glioblastoma

Mutations in APC or mismatch repair genes

26
Q

Describe Gorlin Syndrome

A

Medulloblastoma

mutations in PTCH -> upreg of SHH

27
Q

Describe Neurofibromatosis NF, type 1

A

Common

neurofibromas
Optic nerve gliomas
Lisch nodules
Cafe au lait spots

28
Q

Describe NF type 2

A

Less common
BL schwannomas (CN VIII)
increased meningiomas ependymomas