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Peds neuro boards, V2 > Neuro Oncology > Flashcards

Neuro Oncology Flashcards

1
Q

Most common location for metastatic brain tumors

A

Gray/white matter junction

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2
Q

Most common mets to brain:

Adult (4)

Pediatric (3)

A

Adult

  1. Lung
  2. breast
  3. renal
  4. melanoma

Pediatric

  1. leukemia
  2. germ cell tumors
  3. neuroblastoma
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3
Q

Most common malignant tumor in brain (1) and two additional specifications (2)

A

Most common malignant tumor in brain: Lung Cancer

Specifications:

  1. NSCLC is most common (80% of lung CA)
  2. SCLC has higher incidence of CNS mets, but is a less common cancer overall
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4
Q

Brain Mets most likely to bleed

A

My Cranium Really Bleeds Terribly

  1. Melanoma
  2. Choriocarcinoma
  3. Renal cell Carcinoma
  4. Bronchogenic (lung)
  5. Thyroid
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5
Q

Treatment for Metastatic Brain tumors:

Single Met (3)

Multiple Brain mets (2)

A

Single Brain Met

  1. Surgery followed by RT
  2. Stereotactic raiosurgery
  3. Whole brain radiation therapy

Multiple Brain metastesis

  1. Whole brain radiotherapy
  2. stereotactic radiosurgery to multiple lesions
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6
Q

Variant of whole brain radiation therapy used to decrease cognitive decline (1), and caveat (1)

A

Hippocampal avoidance WBRT + memantine

No defined role for up-front systemic therapy

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7
Q

Leptomeningeal Metastases
1. other names (3)

  1. relative prognosis to other mets
  2. Presenting sign / symptoms
A
  1. other names
    1. Leptomeningeal carcinomatosis
    2. carcinomatous meningitis
    3. leptomeningeal disease
  2. much poorer prognosis compared to other brain mets
  3. Presenting sings
    1. Symptoms of lesions “all over”
      1. Multiple cranial neuropathies
      2. asymmetric radiculopathies
    2. sx of increased ICP
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8
Q

Lemtomeningeal Metastesis: Workup

Imaging
CSF

A
  1. MRI Brain and Spine (i.e. whole neuroaxis)
  2. CSF
    1. Cytology
    2. Pathology review
    3. Circulating tumor DNA (ctDNA)
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9
Q

Leptomeningeal metastesis:

Treatment

A
  1. Hospice
  2. Radiotherapy
    1. Focal vs WBRT vs Craniospinal irradiation (CSI)
  3. systemic treatment
  4. intrathecal treatment
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10
Q

Treatment for Metastatic cord compression

A

High-dose steroids

Surgery > RT (better than Radiotherapy alone)

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11
Q

Integrated diagnosis of infiltrating Gliomas

A
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12
Q
  1. What is this?
  2. MRI features
  3. how would you treat it (3 + if progression)?
A

Glioblastoma (IDHwt)

MRI features

  1. heterogenously enhancing lesion

Treatment:

  1. maximal surgical resection
  2. Daily temozolomide + RT (no stereotactic radiosurgery)
  3. Temozolomide cycles (5/28 day) +/- tumor-treating fields (TTF)
  4. (if progression) consider one of the following
    1. bevacizumab (anit-VEGF ab)
    2. CCNU (nitrosurea)
    3. TTF
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13
Q
  1. What is this?
  2. MRI features (2)
  3. how would you treat it?
A
  1. Astrocytoma IDH-mutated
  2. increased T2/FLAIR; rarely enhances
  3. Treatment:
    1.
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14
Q
  1. What is this?
  2. Pathology features (2)
A

Glioblastoma (IDH WT)

  1. Endothelial proliferation
  2. pseudopalisading necrosis
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15
Q

Difference and significance between two types of GBM

A
  1. Wild type (worse prognosis)
  2. IDH mutated (better prognosis)
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16
Q

Oligodendroglioma:

  1. Grade
  2. WHO requirement for diagnosis
  3. Buzz-word clinical feature
  4. Treatment (3)
A
  1. Grade 2 or 3
  2. IDH mutation AND 1p19q co-deletion
  3. highest incidence of seizures among infiltrating glioma
  4. Treatment:
    1. Responsive to radiation
    2. Procarbazine / CCU / Vincristine (PCV)
    3. Temozolomide (TMZ)
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17
Q

Pilocytic Astrocytoma

  1. Grade
  2. Onset
  3. Imaging
  4. Pathology (4)
  5. locations (3)
  6. Treatment
A
  1. Grade 1
  2. Onset: childhood (juvenile pilocytic astrocytoma or JPA)
  3. Imaging
    1. enhancing
    2. well circumscribed
  4. Pathology
    1. circumscribed
    2. rosenthal fibers
    3. esoinophilic granular bodies
    4. Highly vascular
  5. Locations
    1. COmmon in posterior fossa
    2. Can be found elsewhere
    3. If in optic nerve, think NF1
  6. Treatment: resection
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18
Q

Ependymal Tumors

  1. Typically arise from ____
  2. Age group
  3. Imaging (3)
  4. Pathology (3)
  5. Key clinical feature
  6. Treatment (2)
A
  1. Typically arise from lining of ventricals (most commonly posterior fossa)
  2. Typically in the young
  3. Imaging:
    1. relatively circumscribed
    2. variable enhancement
    3. often cystic
  4. Pathology
    1. Perivascular pseudorosettes (around blood vessels)
    2. ependymal rosettes (around central lumen)
    3. + GFAP
  5. Clinical features
    1. spread through CSF > make sure to evaluate spine and CSF
  6. Treatment
    1. Complete surgical resection
    2. +/- RT (focal vs craniospinal)
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19
Q

What are these and what are they characteristic of?

A

Ependymal tumors

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20
Q

What is this a picture of?

A

Dysembryoplatic neuroepithelial tumor (DNET)

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21
Q

GFAP + is a marker for…

A

Astrocytic or Ependymal tumors

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22
Q

Tumor markers for Lymphoma

A

CD20

BCL6

EBV

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23
Q

Embyonal Tumors:

  1. Histopathology Buzzword
  2. Examples (6)
A
  1. Histopathology Buzzword: “small round blue cell tumor”
  2. Examples
    1. Medulloblastoma
    2. Embyonal tumor with multilayered rosettes
    3. AT/RT
    4. CNS neuroblastoma
    5. CNS ganglioneuroblastoma
    6. medulloepithelioma
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24
Q

Medulloblastoma

  1. Grade
  2. tumor type
  3. location
  4. age group
  5. _____ is associated with poorer prognosis
  6. pathology (2)
  7. Subtypes (4)
A
  1. Grade IV
  2. most common embryonal tumor
  3. Typically cerebellar (not in medulla)
  4. Age group: children
  5. if < 3 years = pooer prognositic (also more likely to suffer long-term neurologic sequelae in survivors)
  6. Pathology:
    1. dense round blue cells
    2. neuroblastic rosettes
  7. Subtypes:
    1. WNT
    2. SHH
    3. C
    4. D
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25
Q
  1. What is this?
  2. What is the workup?
  3. How is it treated (3)
A
  1. (recurrent) medulloblastoma
  2. MRI Brain and spine
  3. LP (disseminates via CSF) - if safe to do so
  4. Treatment
    1. surgery (Goal = gross total resection)
    2. Craniospinal irradiatin (CSI)
    3. Chemotherapy: CCNU / Cisplatin / vincristine (packer regimen)
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26
Q
  1. What is this?
  2. MRI features
  3. workup
  4. treatment (3)
A
  1. CNS lymphoma
  2. MRI features
    1. Diffusely enhancing
    2. restricted diffusion (particularly if in a non-vascular distribution)
    3. may cross corpus callosum
  3. Treatment:
    1. high dose methotrexate (HD-MTX)
    2. (rarely) WBRT
    3. NO surgery
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27
Q

CNS lymphoma

  1. What type?
  2. types (2)
  3. patient population
  4. important diagnostic note
A
  1. Typically B-cell
  2. types
    1. primary
    2. assocaited with systemic lymphoma
  3. often in immunocompromised population (HIV, post-transplant, elderly)
  4. steroids are lymphocytic: ideally give after biopsy
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28
Q

Tumors that tend to cross corpus callosum

A
  1. CNS lymphoma
  2. high-grade gliomas
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29
Q
A

CNS lymphoma

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30
Q
  1. What is this?
  2. Common mutation
  3. Gross histologic finding
A
  1. Adamantinous craniopharyngioma
  2. CTTNB1
  3. cystic “motor oil”
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31
Q

Pituitary tumors and key points for each

  1. A (3)
  2. B (2)
  3. C (3)
A
  1. Pituitary Adenoma
    1. Benign, micro/macroadenoma
    2. may present with hormonal abnormalities (adenoma)
    3. Bitemporal hemianopsia
  2. Pituitary Carcinoma
    1. Can spread outside of CNS
    2. Rare
  3. Craniopharyngioma
    1. Arise from rathke’s cyst remnants
    2. Ademantinous: CTNNB1 muation, cystic / “motor oil” appearance
    3. Papillary: BRAF mutation, solid
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32
Q

What is this?

A

Pituitary Adenoma

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33
Q

Treatment: Pituitary tumors

A
  1. Surgery
  2. Radiotherapy
  3. hormonal therapy
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34
Q
  1. What is this?
  2. epidemiology notes (3)
  3. origin
  4. Grades and subtypes
  5. pathology
  6. imaging
  7. treatment
A
  1. Meningioma
  2. Epidemiology
    1. 33% of primary CNS tumors
    2. Women > Men
    3. increase markedly with age
  3. arise from arachnoid cells in Dura
  4. Grades
    1. I (most common; 90%)
    2. II (atypical menigioma)
    3. III (anaplatic / malignant meningioma)
  5. Pathology: various subtypes, require molecular subtyping (unlikely to be tested)
  6. Imaging: homogenous enhancement with dural tail
  7. Treatment: resection + radiation (stereotactic > fractionated)
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35
Q

Pineal tumor most sensitive to radiation / chemotherapy

A

Germinoma

(think, radiation kills Germs)

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36
Q

Pineoblastoma

  1. Grade
  2. pathology buzz word
  3. clinical notes (2)
A
  1. Pineoblatsoma
  2. Grade IV
  3. Pathology: +/- homer-write rosettes
  4. Clinical notes:
    1. Can spread through CSF
    2. can occur with bilateral retinoblastoma: if so think (trilateral retinoblatoma, Rb gene mutation)
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37
Q

Pineocytoma

  1. Grade
  2. Pathology buzz words (2)
A
  1. Pineocytoma
  2. Grade I
  3. Pathology = neuronal markers
    1. synaptophysin
    2. neuron-specific enolase (NSE)
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38
Q

What is this?

A

Either Pineocytoma (Grade I) or Pineoblastoma (Grade IV)

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39
Q

Germ - cell tumors

  1. Location (2)
  2. Category 1
  3. Category 2
  4. spread via…
  5. Biomarkers (4)
  6. treatment
A
  1. Typically a pituitary tumor, but can occur elsewhere in CNS and extra-CNS
  2. Germ cell tumor (sensitive to RT and chemo)
  3. Non-germinomatous germ cell tumor
    1. Teratoma
    2. yolk sac tumor
    3. embryonal carcinoma
    4. choriocarcinoma
  4. Sprea via local extension or through CSF
  5. Biomarkers
    1. AFP
    2. BhCG
    3. placental alkaline phosphatase
    4. carcinoembryonic antigen (CEA)
  6. Treatment
    1. biopsy vs surgery
    2. RT
    3. Chemo
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40
Q

3 main categories of peripheral nerve tumors

A
  1. Schwannoma
  2. neurofibroma
  3. malignant peripheral nerve sheath tumor
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41
Q

Schwannomas

  1. Grade
  2. Location (2)
  3. if ____ think ___
  4. histopathology classification
A
  1. Grade I
  2. Location
    1. Peripheral nervous system (including spinal nerve roots)
    2. cranial nerves
  3. if bilateral acoustic schwannoma think NF2
  4. Histopathology classification
    1. Antoni A (closely packed)
    2. Antoni B (loosely packed)
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42
Q

Neurofibroma

  1. Grade
  2. Location and association
  3. types
  4. pathology (2)
  5. clinical note
A
  1. Grade I
  2. Peripheral nerves (associated with NF 1)
  3. types
    1. single nodule
    2. plexiform
  4. Pathology
    1. schwann cell
    2. perineurial-like cell
  5. may transform into malignant peripheral nerve-sheath tumor
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43
Q

Malignant Peripheral nerve sheath tumors (MPNST)

  1. Grade
  2. Association
  3. Most common location
  4. Pathology (2)
A
  1. Grade II, III, or IV
  2. assocaited with NF 1 (50%) but also sporadic
  3. most commonly in sciatic nerve (think about if an NF patient presents wtih sciatica)
  4. Pathology
    1. sarcoma-like
    2. +/- S100
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44
Q

Neurofibromatosis Type I

  1. Gene mutation
  2. Chromosome
  3. Inheritance
  4. Diagnostic criteria
A
  1. Neurofibromin Gene (GTPase activator)
  2. Chromosome 17
  3. Autosomal Dominant
  4. Diagnostic criteria (need 2+)
    1. cafe au lait spots (>/= 6)
    2. lisch nodules (>/= 2)
    3. axillary frecklinkg
    4. neurofibromas (>/= 2 or 1 plexiform)
    5. optic glioma
    6. bony dysplasia
    7. 1st degree relative with NF1
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45
Q

Neurofibromatosis Type 2

  1. Gene
  2. chromosome
  3. inheritance
  4. assiciated tumors (4)
A
  1. Merlin gene (cytoskeleton protein)
  2. Chromosome 22
  3. Autosomal Dominant
  4. assoicated tumors
    1. bilareal acoustic schwannomas
    2. neurofibromas
    3. meningiomas
    4. ependymomas
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46
Q

Most common tumors prsenting with Seizures

A
  1. DNET
  2. Astrocytoma
  3. Ganglioglioma
  4. Oligodendroglioma
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47
Q

Tuberous Sclerosis

  1. Assocciated mutations
  2. inheritance
  3. Associated tumors
  4. Treatment
A
  1. TSC 1 and TSC 2 (mTOR pathway)
  2. Autosomal Dominant
  3. associated tumors
    1. CNS “tubers”
    2. SEGA
    3. renal hamartomas
    4. cardiac rhabdomyomas
  4. Everolimus (mTOR inhibitor)
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48
Q

Von Hippel-Lindau Syndrome

  1. Mutation and associated pathogenesis
  2. Tumors
    1. Most notable
      1. location
      2. Clinical note
    2. Others (5)
A
  1. Mutation in VHL gene > increased HIF1 > incrased angiogenesis
  2. Tumors
    1. Most notable = CNS hemangioblatoma (grade I)
      1. Infratentorial / spinal
      2. Saltatory growth pattern (grows…stops…grows…)
    2. Others
      1. Endolymphatic sac tumors (ELST) - associated with decreased hearing
      2. renal cell carcinoma
      3. Pheochromocytoma
      4. retinal angiomas
      5. pancreatic cysts
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49
Q

Cowden Syndrome

  1. Mutation and pathogenesis
  2. biggest buzz-word tumor
  3. other associated tumors
  4. Non-tumor features
A
  1. Mutation in PTEN > phosphatylinositol 3-kinase (PI3K)/Akt pathway
  2. Lhermitte-duclos
    1. Dysplastic gangiocytoma of cerebellum (grade I)
    2. “tiger stripe” appearance
  3. Other associated tumors
    1. breat cancer
    2. follicular thyroid cancer
    3. endometrial cancer
  4. Non-tumor features
    1. macrocephaly
    2. intestinal hamartomas
    3. trichilemmomas of skin
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50
Q

MEN syndrome

  1. Mutation
  2. Tumors
    1. CNS (3)
    2. non-CNS (5)
A
  1. MEN1 mutation
  2. CNS tumors
    1. meningiomas
    2. ependymomas
    3. pituitary tumors
  3. non-CNS tumors
    1. parathyroid
    2. pancreatic islet cell
    3. adrenal cortex
    4. carcinoid
    5. pheochromocytoma
51
Q

Post-chemotherapy Peripheral neuropahty:

  1. Most common agents (4)
  2. Type of neuropathy
  3. comorbidiites
A
  1. Most common agents
    1. vincristine and other vinca alkaloids (vinblastine, vinesine)
    2. taxanes (paclitaxel, docetaxel)
    3. platinum agents (cisplatin, oxaliplatin)
    4. bortezomib
  2. Type: axonal
  3. Comorobidities: increased risk in patients wiht underlying neuropathy
52
Q

Chemotherapeutic agent associated with cerebral Edema

53
Q

Temozolamide:

  1. used to treat
  2. typical adverse effects
A
  1. Used to treat GBM
  2. Adverse effects (timzLOWlamide)
    1. thrombocytopenia
    2. leukopenia
    3. anemia
    4. nausea / vomiting
    5. constipation
54
Q

Bevacizumab

  1. Used to treat
  2. adverse effects (4)
A
  1. Used to treat: recurrent GBM
  2. Adverse effects
    1. increased bleeding
    2. incrased clotting
    3. increased blood pressure (PRES)
    4. increased urine protein
55
Q

Vincristine

  1. Used to treat
  2. adverse effects (3)
A
  1. Used to treat oligodendroglioma
  2. adverse effects
    1. neuropathy
    2. constipation
    3. myelosupprsssion
56
Q

Methotrexate

  1. used to treat
  2. Most notable adverse effects
A
  1. used to treat primary CNS lymphoma
  2. leukocencephalopathy (similar to Normal pressure hydrocephalus picture)
57
Q

How to differentiate Infarct edema from tumor Edema

A

Tumor Edema involves only white matter

58
Q

High-grade oligodendrogliomas, if they metastasize, do so to …..

A

Bone (particularly bone marrow)

59
Q

Features of headache arising from pheochromocytoma (3) and exacerbated by (4)

A
  1. Bilateral and throbbing
  2. Typically nocturnal attacks
  3. relatively short (50% < 15 minutes, 70% <60 minutes)
  4. Provoked by:
    1. sleep
    2. valsalva
    3. exertion
    4. micturation
60
Q

Limbic encephalitis:

  1. Symptoms
  2. Antibodies
    1. Most common (2)
    2. Others (4)
  3. Most common associated tumor
A
  1. Symptoms
    1. amnesia (anterograde)
    2. aggression, paranoid delusions
    3. hyperphagia
  2. Antibody:
    1. Most common: anti-Hu and CV2
    2. Others
      1. Anti-Yo
      2. Anti-ANNA
      3. anti-VGKC
      4. anti-GABA
  3. Most common associated tumor: Lung
61
Q

Infusion-related symptoms associated with Oxaliplatin and what it looks like

A

Pharyngeolaryngeal dysthesias

Symptoms

  1. Cold sensation of throat
  2. discomfort in breathing without respiratory distress or desaturation
  3. Tightness in jaw
62
Q
  1. potentially fatal adverse effect of Cytarabine arabinoside
  2. what causes it?
  3. what does it look like?
A
  1. acute cerebellar toxicity
  2. selective loss of cerebellar Purkinje cells
  3. Symptoms
    1. dysarthria
    2. dysmetria
    3. ataxia
    4. multidirectional nystagmus
63
Q

Exam finding common to nearly all patients receiving Vincristine therapy

A
  1. Loss of deep tendon reflexes
64
Q

Specific localizable symptoms from Meningioma:

  1. Foramen Magnum (3)
  2. Parasagittal (1)
  3. subfrontal (4)
  4. occipital (1)
  5. cerebellopotine angle (2)
A
  1. Foramen Magnum
    1. Paraparesis
    2. sphincteric troubles
    3. tongue atrophy + fasciculation
  2. Parasagittal
    1. Contralateral leg monoparesis
  3. Subfrontal
    1. change in mentation
    2. apathy
    3. disinhibited behavior
    4. urinary incontinence
  4. Occipital lobe
    1. Contralateral hemianopsia
  5. Cerebellopontine angle
    1. decreased hearing
    2. Possible facial weakness / numbness
65
Q

Best imaging study to differentiate progression versus pseudoprogression of tumor

A

MR spectroscopy with perfusion imaging

66
Q

Most common mutation in astrocytoma

67
Q

Paraneoplastic Cerebellar Degeneration

  1. Symptoms (5)
  2. Antibodies
  3. Tumors (4)
A
  1. Symptoms
    1. dysarthria
    2. ataxia (midline and all limbs)
    3. nystagmus
    4. Diplopia
    5. nausea / vomiting
  2. Antibodies
    1. Many (Yo, Hu, VgCC, CV2/CRMP5, MA2, Ri, Tr, GAD, mGluR1)
  3. Tumors
    1. Lung
    2. ovary
    3. breast
    4. Hodgkin’s lymphoma
68
Q

Histolopathology Buzzwords:

  1. Fried Egg
  2. Pseudopallisading
  3. perivascular pseudorosettes
  4. Homer-Wright Rosettes (4)
  5. Rosenthal fibers (4)
A
  1. Fried Egg: oligodendroglioma
  2. Glioblastoma
  3. ependymoma
  4. Homer-wright rosettes
    1. Medulloblastoma (classic; pathognomonic if in posterior fossa)
    2. pineoblastoma
    3. primintive neuroectodermal tumors
    4. olfactory neuroblastoma
  5. Rosenthal fibers
    1. Alexander’s disease
    2. pilocytic astrocytoma
    3. pleomorphic xanthroastrocytoma
    4. Chronic reactive Gliosis
69
Q

MRI (3) and histologic (2)features of DNET

A

MRI

  1. 30% of tumors enhance
  2. “bubbly” appeareance on T2
  3. Calcification

Histology

  1. specific glioneuronal elements
    1. bundles of axons surrounded by oligodendrocyte-like cells
    2. Floating neurons
70
Q

Tumors presenting with enhancing mural nodule (5)

A
  1. Central neurocytoma (pictured)
  2. pleomorphic xanthroastrocytoma
  3. ganglioglioma
  4. DNET
  5. Hemangioblastoma
71
Q

Medulloblastoma:

Factors suggestive of “higher” risk (7)

Factors suggestive of “lower” risk (4+ 30)

Medulloblastoma Genes associated with good (1) and poor (3) prognosis

A
  1. High
    1. Younger age (<3 years)
    2. dissemninated disease
    3. MYC amplification
    4. Anaplasia
    5. Subtotal resection (1.5 cm of residual tumor)
    6. P53 mutation
    7. GL1 zinc finger mutation
  2. Lower
    1. WNT
    2. monosomy 6
      • staining for B catenin
    4. total or near-total resection
72
Q

Histopathologic features of pleomorphic xanthrastrocytoma

A
  1. Intracellular reticulin deposition
  2. Xanthomatous cells with Foamy cytoplasm
  3. Eosinophilic granular bodies
  4. rosenthal fibers
73
Q

Diagnostic Critereon for Li Fraumeni syndrome

Chlassic Li Fraumeni Syndrome Diagnostic criteria

Comphret diagnostic criteria for li Fraumeni syndrome

A

Classic Li Fraumeni Syndrome (need all of the following)

  1. sarcoma diagnosed < 45
  2. 1st degree relative with any cancer before 45
  3. 1st or 2nd degree relative with any cancer <45 OR with Sarcoma diagnosed at any time

Comphret (made in 1 of four circumnstances)

  1. first criteria
    1. 1 tumor typical of LFS prior to 46
    2. (>/=) 1st or 2nd degree relative with typical tumor of LFS before 56
    3. presence of multiple tumors
  2. 2nd criteria
    1. many tumors (>/=) 2 with disease spectrum
    2. (>/=) 1 prior to 46
  3. Adrenocortical carcinoma or tumor of choroid plexus
74
Q

Typical tumors of Li Fraumeni Syndrome (7)

A
  1. brain tumor
  2. adrenocortical carcinoma
  3. leukemia
  4. bronchoalveolar cancer
  5. soft tissue sarcoma
  6. osteosarcoma
  7. premenopausal breast cancer
75
Q

Testing for TP53 should be performed in (5)

A
  1. individuals meeting criteria for Li Fraumani Syndrome
  2. women with breast cancer < 30 with no BRCA1 or BRCA 2 mutations
  3. adrenocortical carcinoma
  4. people with choroid plexus carcinoma
  5. people with childhood sarcoma (exception being Ewings sarcoma)
76
Q

antibody associated with MGUS

77
Q

MRI features of Epidermoid cyst

A
  1. Cerebellopontine angle diffusion-restricting lesions with otherwise largely CSF signal on CT and MRI
78
Q

Most common CP angle tumors (3) and how you can differentiate among them

A
  1. Acoustic schwannoma
    1. NO restriction
  2. Meningioma
    1. may be associated with isolated CSF protein elevation (>200 mg/dL)
    2. NO chemical meningitis
    3. NO diffusion restriction
  3. Epidermoid cyst
    1. Diffusion restriction
    2. Chemical meningitis
79
Q

Malignancies associated with:

NF- 1
NF - 2

A

NF-1: “william, Leuk at me, phocus, youre rhabbing on my last nerve Fiber (fibrosarcoma)”

  1. Fibrosarcoma
  2. Leukemia
  3. Pheochromocytoma
  4. Rhabdomyosarcoma
  5. wilms tumor

NF-2

  1. Acoustic Schwannoma
  2. Meningioma
  3. Glioma
80
Q

Tumors associated with Von Hippel-Lindau Disease (5)

A

“HP makes C3PO

  1. Hemabgioblastoma of CNS
    1. Retina
    2. Brain
  2. Pheochromocytoma
  3. Clear cell cancer of Kidney
  4. Chromosome 3 (where mutation is)
  5. Neuroendocrine tumors of pancreas
  6. Others
81
Q

Most common congenital brain tumors

A

remember a “TACHy PeRM”

  1. MC = Intracranial teratomas (50%)
  2. astrocytomas
  3. choroid plexus papilloma
  4. primitive neuroectodermal tumor
  5. rhabdoid tumors
  6. medullowblastomas
82
Q

what is this?

A

Psammoma body (meningioma)

calcium organized in lamellated concentric structure

“onion skin”

83
Q

Spinal cord tumors:

Most common in adults
Most common in children
MC with VHL

A

MC overall: Spinal ependymoma
MC in children: Astrocytoma
MC with VHL: Hemangioblastoma

84
Q

Patient With _____ tumor has. RELA fusion

what does this mean?

A

Patients with ependymomas and Rela fusions:

  1. Poor prognostic factor
  2. more common in children and young adults
  3. more common in supratentorial locations
85
Q

What type and grade of meningioma is this?

A

Meningothelial meningioma (Grade 1)

86
Q

What type and grade of meningioma is this?

A

Fibrous meningioma

(Grade 1)

87
Q

What type of meningioma is this?

A

Transitional

Features of both Fibrous and meningothelial
Characteristic whorls

(grade 1)

88
Q

What type and grade is this meningioma?

A

Psammamomatous

(grade 1)

89
Q

patient presents with newly diagnosed malignancy on MRI

What features can help differentiate metastesis from primary tumors?

A
  1. Metastatic lesions are often located at the gray-white junction or arterial border zones
  2. though metastesis are most often solitary, multifocal lesions should prompt worry about metastesis
  3. known history of cancer elsewhere in the brain
    4.
90
Q

Brain cancer MRI buzzwords:

Central Necrosis (3)
crossing corpus callosum (2)
Vasogenic Edema (2)
A
  • Central necrosis:
    • GBM
    • Metastatic lesiosn
    • CNS lymphoma
  • Crossing corpus callosum
    • GBM
    • CNS lymphoma
  • Vasogenic edema
    • Metastatic
    • High-grde gliomas
91
Q

Patient with asceptic meningitis, hearing loss, and headaches, subsequently found to have tumor in cerebellopontine angle

What are some features you would expect to see on MRI?
What cell type would you see on histology?
How would you treat it?

A

Epidermoid cyst

MRI features:

  • mildy enhancing
  • cystic
  • Restricted diffusion

Histology:

  • Desquamated epilethelial cells

Treatment:

  • Surgical resection
92
Q

Spinal Cord tumors:

MC overall

  • Typical location
  • MRI findings (3)

MC Children

  • Typical location
  • MRI findings (3)

MC of conus medullaris / filum terminale

*

A

MC overall: Spinal epyndoma

  • Typically cervical
  • MRI = unencapsulated but well circumscribed mass, often with cysts
  • Syringomyelia (50%)
  • average length: 4 vertebral setments

MC children: Astrocytoma

  • Typically thoracic, then cervical
  • Ill defined
  • eccentrically located
  • patchy irregular contrast enhancement

MC of conus medullaris / filum terminale: myxopapillary ependymoma

  • heterogenious enhancement
93
Q

How to differentiate the two tumors that can produce “Fried egg” appearance

A

Oligodendroglioma (most common):

  • GFAP+
  • IDH mutation
  • 1p/19q co-deletion

Neurocytoma

  • Synaptophysin
  • Neuron-specific Enolase
94
Q

Tumors associated with:

Anti-Ma1 and Anti-Ma2

A
  • testicular germ cell tumors
  • lung cancer
95
Q

Patient undergoing chemotherapy for cancer presents with multiple cranial neuropathies, radiculopathies, and increased ICP

What is this?

A

Meningeal carcinomatosis (aka carcinomatous meningitis, leptomeningeal carcinomatosis)

96
Q

Potential trip up:

“rosenthal fibers” make you think of what two conditions?

A
  1. Juvenile pilocytic astrocytoma
  2. Alexander’s disease
97
Q

What do you think if you see a Mural nodule:

Adult + cerebellar location

Child and cerebellar location

Temporal location

A

Cerebellar + adult: Hemangioblastoma (likely in VHL disease)

Cerebellar + child: juvenile pilocytic astrocytoma

Temporal: pleomorphic xanthroastrocytoma

98
Q

“intracellular eosinophilic granular bodies”

(+) Biphasic: ____

(-) biphasic: ____

99
Q

What is the most common Primary brain tumor to bleed?

A

Oligodendroglioma

100
Q

fetaures of Meningiomas

A

“whipped”

(w)horls
(H)omogeneous enhancement
(P)sammoma bodies
(P)ositive staining for…
(E)pithelial membrane antigen and
(D)esmosomes, (D)ural Tail

101
Q

name 4 relatively aggressive meningiomas

A

ones that present a “Clear Cut, Real Problem”

Clear cell (grade II)
Choroid (Grade II)
Rhabdoid (Grade III)
Papillary (Grade III)

102
Q

Child presenting with macrocephaly, hypocalcemia, and diplopia, Subsequently found to have a supratentorial tumor

What is it?
Where did it come from?

A

Craniopharyngioma

Thought to arise from rathke’s pouch remnant

103
Q

What is a key feature needed for a tumor to be classified as a pituitary carcinoma

A

Metastesis

104
Q

Cancer syndromes with primary brain mets:
Associated tumors with:

Cowden’s disease
Gorlin Syndrome
Li-Fraumeni Syndrome
Von Hippel-Lindau disease
Tuberous Sclerosis
Turcot’s Syndrome

A
  • Cowden’s : Lhermitte Duclos (Dysplsatic Gangliocytoma of Cerebellum)
  • Gorlin syndrome: Medulloblastoma
  • Li-Fraumeni Syndrome: Diffuse Astrocytoma
  • VHL: Hemangioblastoma
  • TS: SEGA
  • Turcot’s: GBM or Medulloblastoma
105
Q

What does this show Above and Below the mass lesion?

A

Above: normal
Below: fatty replacement of bone marrow suggsetive of radiation therapy

106
Q

What’s this?

A

Colloid cyst (homogenous enhancement)

107
Q

Pineal tumors and Prognosis:

Embryonal Carcinoma
Mixed Germ Cell Tumor
Yolk Sac tumor
Choriocarcinoma
Germinoma
Mature Teratoma

A

Embryonal Carcinoma: Poor
Mixed Germ cell tumor: Intermediate
Yolk-Sac tumor: Poor
Choriocarcinoma: Intermediate
Germinoma: Excellent
Mature Teratoma: Excellent

108
Q

Urine tests for Cancer

24 hour fractionated metanephrines:
urine Histamine levels

A

Fractionated metenephrines: pheochromocytoma

Histamine: carcinoid tumor (does not exclude, however)

109
Q

Pituitary Masses (mnemonic)

A

SATCHMO

(S)arcoid
(A)neurysm
(T)eratoma, (T)uberculosis
(C)raniopharyngioma, (C)hordoma, (C)left cyst
(H)ypothalamic Glioma, (H)ypothalamic Hammartoma, (H)istiocytosis
(M)eningiomas, (M)ets
(O)ptic tract glioma

110
Q

Histopathologic feature of Paraneoplastic cerebellar degeneration

A

Loss of Purkinje cells

111
Q

How would you treat this?

A

Pilocytic astrocytoma

Resection

112
Q

Tumor most likely to disseminate in CSF

A

Posterior fossa ependymoma

113
Q

Tumors associated with:
AFP

bhCG

CEA

A

Germ cell tumors

(germinoma, Teratoma, yolk sac tumor, embryonal carcinoma, choriocarcinoma)

114
Q

Cancer patient presents with peripheral neuropathy

What chemo treatments could he have been given?

A
  • Vincristine
  • Taxanes
  • Platinum agents
  • Bortezumib
115
Q

Cancer patient presents with cerebral edema

What chemo treatments could he have been given?

116
Q

Cancer patient presents with pancytopenia

What chemo treatments could he have been given?

what kind of tumor does he have?

A

Temozolomide

GBM

117
Q

Cancer patient presents with intracranial bleed, increased clotting, PRES, and increased urine protein

What chemo treatments could he have been given?

What type of cancer does he have?

A

Bevacizumab

Recurrent GBM

118
Q

Cancer patient presents with Neuropathy, constipation, and myelosuppression

What chemo treatments could he have been given?

What kind of tumor does he have?

A

Vincristine

Oligodendroglioma

119
Q

Cancer patient presents with peripheral NPH like picture

What is this called?

What chemo treatments could he have been given?

What type of cancer does he have?

A

Leukoencephalopathy

Methotrexate

Primary CNS lymphoma (most common in elderly population

120
Q

Trip-up:

Tumors associated with:

Pleomorphic xanthroastrocytoma

Pilocytic astrocytoma

A

Pleomorphic xanthroastrocytoma:
Intracellular reticulin deposits

Pilocytic astrocytoma:
bipolar “hair like” astrocytes

121
Q

Patient presents with diagnosis of Medulloblastoma:

What are 3 features suggestive of “high risk” tumor?

A

Age <3

>1.5 cm2 residual tumor after maximal safe total resection

metastatsis at initial presntation

122
Q

patient presents iwth vertical gaze palsy but no headaches, nausea, or vomiting

pupils normal

Horizonal and inferior gaze normal

Where is the leasion?

A

Pineal gland (pineal tumor)

123
Q

“high risk” status for children with medulloblastoma

(3)

A

Metastatsis at initial diagnosis

Under age 3

over 1.5 cm2 residual tumor after maximal resection

Peds neuro boards, V2 (29 decks)

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