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Dr. Cheng neuro review Qs > ch 8 neuro-oncology > Flashcards

ch 8 neuro-oncology Flashcards

1
Q

Patient presents with spontaneous rapid, irregular, and high-amplitude conjugate eye movements that occur in any direction, as well as diffuse myoclonic jerks, and ataxia. What is the syndrome?

A

opsoclonus myoclonus syndrome

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2
Q

opsoclonous myoclonus syndrome presents in adults with what types of cancer (3)?

A

breast, ovarian, and small cell lung cancer

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3
Q

opsoclonus myoclonus syndrome presents in children with what type of cancer (1)?

A

neuroblastoma

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4
Q

In adults with opsoclonus myoclonus syndrome, what antibody can be seen (especially in association with breast cancer?

A

anti-Ri (also known as ANNA-2) antibodies

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5
Q

txt for children with neuroblastoma and opsoclonus myoclonus syndrome?

A

ACTH; may have resolution of neurologic manifestations when neuroblatoma is resected

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6
Q

In opsoclonus myoclonus syndrome, who has worse prognosis: adults or children?

A

adults

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7
Q

Diffuse astrocytomas can be classified according to the histopathologic cell type into the following: (8)?

A

fibrillary, gemistocytic, protoplasmic, small cell, giant cell, epithelioid and granular cell, glioblastoma with oligodendroglioma component

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8
Q

What are fibrillary astrocytomas?

A

prototypical and more common, with elongated hyperchromati nuclei, scant cytoplasm, and presence of a fibrillary background

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9
Q

What are gemistocytic astrocytomas?

A

rounded cells with prominent eosinophilic cytoplasm

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10
Q

What are protoplasmic astrocytomas?

A

cells with oval-shaped nuclei and wispy cobweb-like processes

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11
Q

Clinical manifestations of leptomeningeal disease? (7)

A

headache, backache, polyradiculopathies, multiple cranial neuropathies, AMS, features of increased IP and hydrocephalus

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12
Q

How to diagnose leptomeningeal carcinomatosis?

A

MRI shows leptomeningeal enhancement. CSF cytology and flow cytometry (not sensitive, and sometimes biopsy is required)

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13
Q

Treatment and prognosis of leptomeningeal carcinomatosis?

A

whole-brain radiation plus intraventricular (or intrathecal) chemotherapy; prognosis is poor with survivals of < 6 months

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14
Q

Most common primary brain tumor?

A

Glioma

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15
Q

What is a well established risk factor known to be associated with the development of gliomas?

A

radiation exposure

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16
Q

Describe characteristic MRI findings of a astrocytoma WHO grade 4 (aka glioblastoma)? (4)

A

neoplasm that crosses corpus callosum, with prominent gad enhancement, hypointense areas that correlate with necrosis, edema on FLAIR

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17
Q

where do ependymomas localize to?

A

most commonly infratentorial, typically in the 4th ventricle. Can also occur supratentorial in the periventricular region Can also occur in the spinal cord, more commonly in adults

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18
Q

Where do oligodendrogliomas localize to?

A

hemispheric masses usually arising superficially and with cortical involvement

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19
Q

Histopathologic features of glioblastoma (astrocytoma WHO grade 4)? (5)

A

perinecrotic pseudopalisading, nuclear atypia, mitoses,endothelial hyperplasia, and necrosis

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20
Q

Histopathologic feature of oligodendrogliomas?

A

fried egg

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21
Q

Histopathologic feature of ependymomas?

A

perivascular pseudorosettes

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22
Q

Histopathologic feature of medulloblastoma?

A

Homer-Wright rosettes

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23
Q

Treatment and prognosis for glioblastoma?

A

median survival is about 15 months with radiotherapy plus temozolomide

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24
Q

What tumor does not commonly present with seizures?

A

ependymoma

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25
Q

anti-Yo antibodies are associated with what cancer?

A

ovarian cancer (and in a smaller percentage of patients with breast cancer)

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26
Q

anti-Hu antibodies are associated with what cancer?

A

small cell carcinoma of the lung

27
Q

Anti-Tr antibody is associate with what cancer?

A

lymphomas (and seen more commonly in young men)

28
Q

What is the treatment of glioblastoma?

A

surgical resection, radiation therapy and chemotherapy with temozolomide

29
Q

txt of recurrent glioblastomas? How does it work?

A

Bevacizumab; mab that binds to VEGF –> decreases edema and need for steroids

30
Q

What genetic mutation is seen in low grade gliomas and associated with better prognosis?

A

IDH1 (isocitrate dehydrogenase)

31
Q

What is the predominant antibody in paraneoplastic optic neuropathy? What tumor is it associated with?

A

anti-CRMP-5; lung cancer

32
Q

What is the antibody in paraneoplastic retinal degeneration? Associated cancers?

A

anti-recoverin or anti-CAR; SCLC, thymoma, RCC, melanoma

33
Q

What is the antibody associated with paraneoplastic subacute sensory neuropathy and neuronopathy? Associated cancer?

A

anti-Hu (ANNA-1); SCLC

34
Q

What antibody is associated with paraneoplastic cerebellar degeneration? Associated cancer?

A

anti-Yo; ovarian and breast cancer

35
Q

antibodies associated with paraneoplastic chorea (presenting with bilateral choreoathetosis)? associated cancer?

A

anti-Hu and anti-CRMP-5 ab; lung cancer

36
Q

What WHO grade is anaplastic astrocytomas? Prognosis?

A

WHO grade 3; highly infiltrating; average survival of 2-3 years

37
Q

What genetic mutations are commonly seen in anaplastic astrocytomas?

A

p53 mutation in almost half

38
Q

What is a common genetic mutation in oligodendogliomas? prognosis?

A

1p19q deletion; may be associated with better response to treatment and survival

39
Q

What is the WHO grade of pilocytic astrocytoma?

A

WHO grade I

40
Q

What is the most common glioma in children?

A

pilocystic astrocytoma

41
Q

Where do pilocytic astrocytomas typically appear and what are typical features on MRI?

A

typically appear in the cerebellum, but can also be in other subtentorial areas; In the cerebellum they are well circumscribed, cystic, with gad enhancing mural nodule. (In the hypothalamus and optic nerves, these tumors are solid).

42
Q

What disease is associated with pilocytic astrocytomas, especially in the optic nerve?

A

NF type 1

43
Q

Pleomorphic xanthoastrocytoma (PXA) typically affects what part of the brain?

A

well-demarcated tumor, typically superficial and affecting the cortex, most commonly seen in the temporal lobes

44
Q

What does PXA look like on MRI?

A

cyst with enhancing mural nodule

45
Q

histopathology of PXA?

A

pleomorphic astrocytes in fascicles, intercellular reticulin deposition, eosinophilic granular bodies snd Rosenthal fibers

46
Q

what is the prognosis of PXA?

A

surgically resectable; prognosis is favorable

47
Q

What is the WHO grading of subependymal giant cell astrocytoma (SEGA) and where do they occur?

A

hamartomatous tumor, WHO grade I, located in the intraventricular region, commonly in the 3rd or lateral ventricles

48
Q

What disease is SEGA associated with?

A

tuberous sclerosis

49
Q

Histopathology of SEGA?

A

cells are packed in fascicles and around blood vessels, giving the appearance of pseudorosettes

50
Q

What part of the brain is typically affected by oligodendrogliomas?

A

arises superficially in the cortex, most commonly in the frontal lobes

51
Q

Histopathologic features of pilocytic astrocytoma?

A

biphasic pattern of compact regions along with microcystic components; there are piloid or hair-like astrocytic processes

52
Q

Prognosis of patients < 3 years old with ependymoma?

A

poor/worse

53
Q

What is myxopapillary ependymoma? typical patient demographic? prognosis?

A

WHO grade I variant of ependymoma that occurs almost exclusively in the filum terminale. More common in adults and has a better prognosis?

54
Q

genetically, ependymomas are related to what disease?

A

associated with chromosome 22q deletions; spinal ependymomas may be related to NF2 mutations

55
Q

What are gangliomas? What part of the brain is typically affected? Common presenting symptom?

A

CNS tumor with glial and neuronal components; arises from the temporal lobe, extends superficially and often presents with seizures

56
Q

MRI appearance of ganglioma?

A

cyst with an ehancing mural nodule

57
Q

microscopic features? (5)

A

fibrillary background with dysmorphic ganglion cells; eosinophilic granular bodies, perivascular lymphocytic cuffing, microcystic spaces, collagen deposition

58
Q

What is a reactive marker of neurocytoma?

A

synaptophysin; marker of neuronal differentiation

59
Q

Common location for neurocytomas?

A

intraventricular

60
Q

microscopic features?

A

round cells in a fibrillary background with prominent vasculature +/- calcification

61
Q

neurocytomas resemble oligodendrogliomas; how can you tell the difference microscopically?

A

neurocytomas are immunoreactive for neuronal differentiation but not for glial markers; positive for synaptophysin but negative for GFAP (glial fibrillary acidic protein)

62
Q

WHO grade of neurocytomas and prognosis?

A

WHO grade II, good prognosis

63
Q

Describe the sympathetic pathway to the eye? (Lesion of this pathway causes Horner’s sydrome.

A
64
Q

Patient has Horner’s syndrome? How can you use cocaine drops and hydroyamphetamine drops to further localize the lesion?

A

Dr. Cheng neuro review Qs (9 decks)

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