BMB 2 - CNS Neoplasms; Paraneoplastic & Autoimmune Encephalitis Flashcards

1
Q

Metastatic malignancies to the brain are ____x more likely than primary malignancies.

A

Metastatic malignancies to the brain are 10 x more likely than primary malignancies.

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2
Q

20% of metastases to the adult brain come from what organ system?

A

The lungs

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3
Q

What are the four most common origin sites for metastases ending up in the adult brain?

A

Lung > melanoma > breast > renal cell carcinomas

(>> colorectal)

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4
Q

What are the three most common origin sites for metastases ending up in the pediatric brain?

A

Sarcomas

Neuroblastomas

Germ cell tumors

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5
Q

What are the most common origin sites for hemorrhagic metastases ending up in the adult brain?

A

Melanoma,

renal,

thyroid,

choriocarcinoma

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6
Q

What is the most common type of primary brain cancer?

A

Diffusely infiltrating gliomas

(e.g. glioblastoma multiforme)

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7
Q

Name some of the various S/Sy sometimes seen in patients with primary brain tumors?

A

Weakness and numbness

Aphasia

Seizures

Ataxia and gait impairment

Headaches

Brainstem symptoms

Blindness

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8
Q

Primary brain tumors are generally more associated with epilepsy in ________er patient populations and due to the less common, _______-grade gliomas.

A

Primary brain tumors are generally more associated with epilepsy in younger patient populations and due to the less common, high-grade gliomas.

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9
Q

Why are high-grade gliomas more likely to result in epilepsy than low-grade?

A

Due to an IDH 1/2 mutation which results in production of 2-hydroxyglutarate, which is both cytotoxic and stimulatory

(i.e. they produce glutamate, which is excitatory)

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10
Q

Brain metastases do not typically cause seizures; they are only more likely to cause them in what circumstances?

A

Unless (1) the cortex is involved or (2) the metastases are hemorrhagic

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11
Q

Brain metastases are widely associated with _________ edema and ____toxicity.

A

Brain metastases are widely associated with vasogenic edema and cytotoxicity.

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12
Q

True/False.

MRI is the main imaging technique used in identifying brain metastases.

A

True.

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13
Q

How do brain metastases typically present on MRI?

A

Multiple, well-circumscribed lesions at the gray-white junction

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14
Q

The large majority (80%) of brain metastases are in what location?

Options: cerebrum, cerebellum, or brainstem

A

Cerebrum

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15
Q

What would be your first step in working up the source of a patient’s brain metastases?

A

CT scan of the chest/abdomen/pelvis

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16
Q

What are the three options for radiation of brain metastases?

A
  1. Whole brain radiation
  2. Hippocampal-avoidant radiation
  3. Stereotactic radiosurgery
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17
Q

What term refers to the condition in which a solid tumor (typically lung, melanoma, or breast tumors) diffusely spreads to the leptomeninges?

A

Leptomeningeal carcinomatosis

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18
Q

A patient with untreated leptomeningeal carcinomatosis can expect to live how long?

And with treated leptomeningeal carcinomatosis?

A

4 weeks

4 months

(rapidly fatal)

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19
Q

Give the four names for the four grades of astrocytoma:

A

Grade 1 — Pilocytic

Grade 2 — Diffuse

Grade 3 — Anaplastic

Grade 4 — Glioblastoma

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20
Q

Glioblastoma multiforme is actually a very aggressive form of __________oma.

A

Glioblastoma multiforme is actually a very aggressive form of astrocytoma.

21
Q

Are glioblastomas benign or malignant?

22
Q

What are the features of oligodendroglioma growth and IDH genetics?

A

Slow-growing with calcification;

1p/19q codeletion

23
Q

Which primary brain tumor is typically found in either the spinal cord, brainstem, or thalamus and involves histone mutations?

A

Diffuse midline glioma

(associated with K27M and G34R mutations)

24
Q

Which primary brain tumor is associated with HIV and EBV?

A

Primary CNS lymphoma

25
What is the most common pediatric malignancy of the brain?
**Medulloblastoma** | (slight predominance for males)
26
What primary brain tumor is seen in children and is associated with a variety of familial cancer predisposition syndromes (e.g. Li-Fraumeni, Turcot, Gorlin, etc.)?
Medulloblastoma
27
What tumor type is described as a “sausage shaped” tumor in the conus medullaris?
Ependymoma
28
What is the characteristic histology of an ependyoma?
Perivascular psuedorossettes
29
Which size of pituitary adenoma is typically non-secreting?
Macroadenomas
30
Pituitary adenomas can either be pituitary macroadenomas (\> ___ cm) or pituitary microadenomas (\< ___ cm).
Pituitary adenomas can either be pituitary macroadenomas (\> **_1_** cm) or pituitary microadenomas (\< **_1_** cm).
31
Mengiomas arise from what tissue layer(s)?
The **arachnoid** mater (and can thus arise anywhere in the CNS where there's arachnoid)
32
How does a meningioma appear on imaging?
An extra-axial mass with dural tail
33
**True/False**. _~80% of meningiomas are benign_ (grade I; surgery only); _~20% are atypical_ (grade II; surgery and maybe radiation); _\<2% are malignant_ (grade III; surgery and radiation)
True.
34
Name a few malignancies associated with **paraneoplastic subacute cerebellar degeneration**.
**Gynecological** (ovarian, endometrial, and breast cancers) ## Footnote **Small cell carcinoma of the lung** **Hodgkin's lymphoma**
35
What are the presenting features of paraneoplastic subacute cerebellar degeneration?
**_Progressive cerebellar dysfunction over a period of several weeks_** in a patient with an underlying malignancy; dysarthria, vertigo, gait instability, limb and ocular ataxia, nystagmus, diplopia *(results due to loss of **Purkinje fibers**)*
36
Which auto-antibodies are associated with paraneoplastic subacute cerebellar degeneration in patients with breast cancer?
Anti-**Yo** antibodies; anti-**Ri** antibodies
37
Which auto-antibodies are associated with paraneoplastic subacute cerebellar degeneration in patients with small cell lung cancer?
Anti-**Hu** antibodies
38
Which auto-antibodies are associated with paraneoplastic subacute cerebellar degeneration in patients with neuroblastoma?
Anti-**Hu** antibodies
39
A patient with breast cancer develops opsoclonus and ataxia. She likely has anti-\_\_\_\_ antibodies targeting her \_\_\_\_\_\_\_\_\_\_\_\_\_.
A patient with breast cancer develops opsoclonus and ataxia. She likely has anti-**_Ri_** antibodies targeting her **_cerebellum_**.
40
An infant presenting with opsoclonus-myoclonus syndrome likely has anti-\_\_\_\_\_ antibodies and an underlying \_\_\_\_\_\_\_\_\_\_\_\_.
A baby presenting with opsoclonus-myoclonus syndrome likely has anti-_**Ri** (**Anna 2**)_ antibodies and an underlying **_neuroblastoma_**.
41
What is the colloquial term for opsolonus-myoclonus syndrome?
'Dancing eyes, dancing feet' syndrome
42
A patient with a previous _ovarian teratoma_ presents with S/Sy of encephalitis. MRI shows hyperintensities in her temporal lobes. You suspect her body may have developed antibodies against what?
**NMDA-receptors** *(There were present on the teratoma; there is now cross-reactivity with the CNS.)*
43
A patient with a previous _thymoma_ presents with S/Sy of encephalitis. MRI shows hyperintensities in his medial temporal lobes and basal ganglia. You suspect his body may have developed antibodies against what?
**LGI1** (leading to LGI1-associated encephalitis)
44
What medication used in managing thymomas is associated with severe fatigue, acute kidney injury, respiratory failure, sepsis, and multiple organ failure due to _myasthenic crisis_?
Pembrolizumab
45
What medication type is useful in treating some malignancies by 'taking the foot off the break' of T cells (via a mechanism of action in which an inhibitory signal is purposefully blocked)?
PD-1 inhibitors and PD-L1 inhibitors
46
A 10 year old presents with chorea one week after an upper respiratory infection. What do you suspect?
_Autoimmune issue of the basal ganglia_ following a ***S. pyogenes infection***
47
A 22-year-old presets with a new onset movement disorder, psychoses, slow writhing movements, and dystonia. What do you suspect?
Anti-NMDA-receptor antibodies
48
A 35-year-old presents with a new-onset movement disorder, altered mental status, and abnormal liver function tests. What do you suspect?
Wilson's disease | (hepatolenticular degeneration)
49
Following treatment for Hashimoto's thyroiditis, a female patient presents with neuropsychiatric symptoms and seizures, anti-TPO/thyroglobulin antibodies. How do you treat her?
**Steroids** (Hashimoto's encephalitis, aka steroid-responsive encephalopathy)