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OS9.2 - Neuro > Tumors > Flashcards

Tumors Flashcards

1
Q

Types of gliomas

A
  1. astrocytoma (juv. PA, low-grade, anaplastic)
  2. ependymoma
  3. oligodendroglioma
    + GBM
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2
Q

What’s a tumor of neurons called?

A

Neuronal tumor

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3
Q

What’s a mixed cell tumor of neuronal and glial cells called?

A

Ganglioglioma

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4
Q

Nerve tumors

A

Schwannoma and Neurofibroma

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5
Q

Other tumors that can occur intracranially

A
  • meningioma
  • hemangioma
  • choroid plexus tumors
  • pineal tumor
  • germinoma (from nests of germ cell tumors)
  • primary CNS lymphoma
  • sellar tumors (ie pituitary)
  • suprasellar tumors (ie craniopharyngioma)
  • cystic lesions
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6
Q

How to distinguish primary vs. metastatic cancer in brain?

A
  • find extracranial cancer (whole body scan)
  • metastatic will often be multiple lesions (ring-enhancing)
  • mets will have significant edema, out of proportion to the nodule size
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7
Q

Signs and symptoms of brain tumors

A
  • tumor HA

- focal signs: papilledema; seizures; focal neuro deficits

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8
Q

What labs to do when a patient has a brain tumor?

A

x

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9
Q

Treatment plan for patients with brain tumor

A 
treating symptoms:
- steroids
- if seizures: anticonvulsants (*use non-CYP inducers; don't use prophylactically)
definitive treatment:
- observation
- surgery
- radiotherapy
- chemo
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10
Q

Most common primary brain neoplasia

A
  1. Infiltrative Astrocytoma - 42%
  2. GBM - 40%
  3. all the rest are
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11
Q

EBV is linked to

A

CNS lymphoma in transplant patients

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12
Q

Brain lesions in Tuberous sclerosis (TSC on 9/16)

A
  1. Subependymal giant cell astrocytoma
  2. cortical tubers
  3. glioma
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13
Q

NF1 vs. NF2 tumors

A

NF1 (17) - optic nerve gliomas, glioblastoma, neurofibromas in skin derived from NCCs
NF2 (22) - meningioma, schwannoma (acoustic neuroma), ependymoma

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14
Q

Li Fraumeni tumors

A
  • glioma (MC astro), medulloblastoma

- p53 mutation

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15
Q

Feature of tumor headache

A
  • 2/2 increased ICP or local irritation
  • may show laterality, resembles migraine
  • suspect tumor especially if it’s worse in the AM because overnight the veins get congested from lying down
  • suggests tumor if vomiting immediately follows acute onset HA (suggesting ICP)
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16
Q

Types of herniation

A
  • subfalcine - ACA
  • diencephalic - drowsiness, impaired gaze, Horner’s
  • uncal - ipsi CN3, hemiparesis
  • upward through tentorium - ipsi CN3, contra hemiparesis
  • tonsillar - BP changes, weakness, respiratory disturbance, Horner’s
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17
Q

What brain tumor type can be diagnosed with lumbar puncture?

A

Primary CNS lymphoma - malignant cells can be in CSF

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18
Q

T1 vs. T2 weighted imaging

A 
T1 = water is dark (so is tumor)
T2 = water/tumor is bright
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19
Q

Normally, edematous contrast enhancing tumors are fast-growing and malignant. What are 2 contrast-enhancing BENIGN tumors?

A
  1. Pilocytic astrocytoma

2. Meningioma

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20
Q

Gliomas Grades 1-4

A

Grade 1: JPA; indolent, usually non-infiltrative;
Grade 2: low-grade astro
Grade 3: anaplastic astro
Grade 4: GBM

21
Q

Temozolamide

A
  • alkylating agent, causes DNA damage (that is repaired by MGMT)
  • SOC for malignant glioma
  • given during and after radiation
  • prolongs median survival by 3mo
22
Q

Meningioma

A
  • diffuse enhancement
  • slow-growing
  • dural tail
  • benign tumors, but may want to remove them +/- radiotherapy (higher grade would need radiotherapy too)
23
Q

Pituitary tumor

A
  • mostly derived from anterior pituitary
  • micros are usually functional; macros are usually non-functional
  • sx: HA, vision, endo
  • Tx: observe, surgery, RT, chemo
24
Q

Acoustic neuroma

A
  • Schwann cells; most common site is CN8, also CN5 or others
  • also commonly present at the cerebellopontine angle
  • may present with hearing loss, ataxia, trigeminal neuralgia/neuropathy, and ataxia
  • if bilateral –> NF2
25
Q

PCNSL

A
  • steroids are oncolytic; avoid
  • from trafficking B-LCs
  • prognosis: mo for steroids, 1yr for RT, 3-4yr for chemo+RT (MTX works well)
  • recall link between EBV and immunosuppression
26
Q

Brain metatasases

A
  • as devastating as high grade glioma but more common
  • mostly from the LUNG; also breast, melanoma, colon, RCC
  • will see single/multiple discrete enhancing nodules, edema out of proportion to nodules
  • tx: WBRT with goal to prolong life w/o incurring neuro deficit; untx’d survival is ~4wk, tx may prolong to 12mo
27
Q

Ring-enhancing lesion in the brain - what could it be?

A
  • metastasis
  • abscess
  • CVA
  • high grade glioma
  • lymphoma
  • demyelination (open ring enhancement)
28
Q

Spinal cord tumors

A
  • can be extradural or intradural; if intradural can be intramedullary (astro, ependymo, hemangio) or extramedullary (schwanno, neurofibro, menigio)
  • present as pain, weakness (UMN/LMN findings), paresthesias
  • tx: surgery, RT, chemo
29
Q

Risk factors for pediatric brain tumors

A
  1. ionizing radiation
  2. other tumors (esp. kidney, RB)
  3. immune suppression (Wiskott-Aldrich, Ataxia-telan; acquired ID)
  4. familial conditions
30
Q

Clinical presentation of PBTs

A

Depends on location:

  • supratentorial –> localized sx, seizures, hemiparesis
  • midline –> endocrinopathies, VF defect, signs of inc. ICP/hydrocephalus
  • infratentorial –> inc. ICP
  • brainstem –> CN deficits, hydrocephalus
31
Q

How to diagnose PBT

A
  • H&P
  • imaging
  • biopsy
  • CSF cytology/look for mets (+/- bone scan)
32
Q

How to treat PBT

A
  • surgery (histo dx and reduce tumor burden; won’t work for brainstem gliomas/DIPG)
  • radiation: >7yo, min. >3yo
  • chemo: for high-grade tumors or optic
33
Q

Types of PBTs

A
  • astrocytoma (LG is MC; JPA can be anywhere)
  • medulloblastoma
  • supratentorial PNETs
  • pineoblastomas
  • brainstem glioma
  • diffuse intrinsic pontine glioma
  • ependymoma
  • neurofibroma

*high grade gliomas and GBM are RARE in kids

34
Q

Most common anatomic sites of PBTs (in order)

A
  1. Infratentorial
  2. Supratentorial
  3. Midline
35
Q

Ependymoma

A
  • from epithelial cells of ventricular system

- in kids it’s usually IC

36
Q

Medulloblastoma

A
  • MC malignant PBT (primary)
  • invasive, rapidly growing tumors of primitive nerve cells (or cerebellar stem cells)
  • occurs infratentorial/posterior fossa
  • metastasize via CSF
  • heterogenous enhancement on MRI
  • Homer-Wright rosettes
  • if WNT mutation good prog; if Shh may be good/intermediate
37
Q

Craniopharyngioma

A
  • rare, usually suprasellar neoplasm, which may be cystic, that develops from nests of epithelium derived from Rathke’s pouch
  • Rathke’s pouch is an embryonic precursor of the anterior pituitary
38
Q

If the kid is

A

supratentorial

  • glioma
  • teratoma
  • PNET
  • choroid plexus
39
Q

If the kid is 1-11yo, the tumor is most likely where and what type?

A

infratentorial

  • medulloblastoma
  • ependymoma
  • brainstem glioma
40
Q

If the kid is >11yo, the tumor is most likely where and what type?

A

Infra/Supratentorial

  • glioma
  • PNET
  • medulloblastoma
  • germinoma
41
Q

Turcot syndrome

A
  • medulloblast-omas
  • GBM
  • associated with germline APC mutation
42
Q

Cowden syndrome

A
  • dysplastic gangliocytoma of the cerebellum

- PTEN mutation leads to hyperactivity of mTOR

43
Q

Classic triad for symptoms of inc. ICP

A
  • morning headache
  • N/V
  • lethargy
  • infants: bulging fontanel and sundowning
44
Q

How to histologically recognize PA?

A
  • long red strands
  • eosinophilic Rosenthal fibers
  • hyalinization of blood vessels
  • pilocytic refers to the hairlike projections from neoplastic cells
45
Q

Mutation in the MAPK path and what tumors they cause

A
  • BRAF activating mutation –> JPA in cerebrum
  • RAF fusion/activation –> JPA in cerebellum
  • loss of NF1 inhibition on KRAS –> NF
  • BRAF v600e GOF mutation –> extracerebellar
46
Q

Brainstem glioma

A
  • uniformly fatal in ~2yr
  • non operable
  • radiation, supportive care helps
47
Q

Diffuse intrinsic pontine glioma

A
  • dismal prognosis
  • CST signs (hemiparesis), ataxia, CN6-8 deficits
  • on MRI will see obscuration of pons w/o enhancement and engulfing basilar artery - characteristic MRI signs so no biopsy needed
  • non-operable; radiation helps
48
Q

Macro vs. Microadenoma of the Pituitary

A

Macro: >10mm; Micro:

49
Q

Pituicytoma

A
  • rare, low-grade glioma derived from pituicytes of the posterior pituitary
  • non-secreting
  • present as mass lesion similarly to meningioma

OS9.2 - Neuro (10 decks)

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