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Neuro II > CNS neoplasms > Flashcards

CNS neoplasms Flashcards

1
Q

Features of grade I CNS neoplasms

A

Low proliferative potential and cure with surgical resection

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2
Q

Features of grade II CNS neoplasms

A

Diffuse infiltrative
Low mitotic activity
Probability of recurrence
Some tend to progress to high grade

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3
Q

Features of grade III CNS neoplasms

A

Anaplastic tumor
Malignant histologic features
High recurrence rate
Need chemotherapy and radiotherapy

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4
Q

Features of grade IV CNS neoplasms

A

Obviously malignant
Necrosis-prone and fast capability of recurrence
Diffuse spreading in CNS

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5
Q

Typical age range associated with glioblastoma, astrocytoma, and oligodendroglioma

A

Adults

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6
Q

Typical age range associated with pilocytic astrocytoma, medulloblastoma, and pineoloma

A

Children

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7
Q

Types of CNS cancers that are found in both children and adults

A

Craniopharyngioma
Ependymoma

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8
Q

Overexpression of these protooncogenes is associated with CNS tumor

A

EGFR
PDFR

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9
Q

Loss of these tumor suppressor genes is associated with CNS malignancy

A

P53
CDKN2A/B
PTEN

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10
Q

Typical mutation in lower-grade astrocytomas

A

IDH mutation with intact 1p/19q

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11
Q

Typical mutation oligodendrogliomas

A

IDH mutation and codeletion of 1p/19q

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12
Q

IDH type associated with a better prognosis

A

IDH mutant type –> IDH positive

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13
Q

IDH type associated with a poor prognosis

A

IDH wild type –> IDH negative

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14
Q

WHO grade and type of glioblastoma multiforme

A

Grade IV astrocytoma

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15
Q

Most common malignant primary brain tumor

A

Glioblastoma multiforme

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16
Q

Typical IDH type in glioblastoma multiforme

A

Negative

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17
Q

Histology shows atypia, mitosis, and microvascular proliferation with areas of necrosis and hemorrhage surrounded by pseudopalisade arrangement of tumor cells that are EGFR and GFAP positive

A

Glioblastoma multiforme

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18
Q

WHO grade of pilocytic astrocytoma

A

Grade I –> relatively benign

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19
Q

Most common benign tumor of childhood

A

Pilocytic astrocytoma

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20
Q

T1 MRI shows well demarcated cystic lesion with an enhancing mural nodule in the cerebellum, or perhaps the brainstem

A

Pilocytic astrocytoma

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21
Q

Histology shows bipolar cells with hairlike processes, called Rosenthal fibers

A

Pilocytic astrocytoma

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22
Q

Common location of oligodendroglioma

A

Cerebral hemisphere, usually involving the frontal lobe

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23
Q

Grossly a well-circumscribed gray mass with calcification diffusely infiltrating the cortex and white matter

A

Oligodendroglioma

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24
Q

Histology shows sheet of cells with round regular nuclei and perinuclear halo, dense network of branching capillaries, and perineuronal satellites

A

Oligodendroglioma

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25
Q

Tumor that is derived from ependymal cells lining the ventricular surface, frequently arising from wall of fourth ventricle, within the posterior fossa

A

Ependymoma

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26
Q

Histology shows perivascular pseudorosettes and ependymal rosettes, usually GFAP positive

A

Ependymoma

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27
Q

Electron microscopy findings in ependymoma

A

Cilia and microvilli
Junctional complexes

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28
Q

Most common malignant brain tumor of childhood

A

Medulloblastoma

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29
Q

Poorly differentiated tumor of the cerebellum, common in childhood

A

Medulloblastoma

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30
Q

MRI shows densely enhancing mass in the posterior fossa, within the cerebellum

A

Medulloblastoma

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31
Q

Drop metastases

A

Dissemination through CSF to spinal cord

32
Q

Histology shows small blue cells, Homer-Wright/neuroblastic rosettes with central lumen of neutrophils, sheets of primitive cells and high mitotic figures. Maybe synaptophysin positive.

A

Medulloblastoma

33
Q

Characteristics of small blue cells on histology

A

Densely packed cells with hyperchromatic nuclei and scan cytoplasm

34
Q

WHO grade of meningioma

A

Grade I, slow-growing and benign

35
Q

CNS tumor associated with ionizing radiation and NF2, with a female predominance

A

Meningioma

36
Q

Grossly a well-demarcated, firm, rubbery dural-based tumor

A

Meningioma

37
Q

Histology shows whorls of arachnoid cap cells, laminated calcified psammoma bodies. It is EMA, PR, and somatostain positive. It is GFAP and synaptophysin negative.

A

Meningioma

38
Q

Two types of craniopharyngioma and there associated age

A

Adamantinomatous –> children
Papillary –> adults

39
Q

Most common non-neuroepithelial intracranial tumor in children

A

Adamantinomatous craniopharyngioma

40
Q

Mutation associated with adamantinomatous craniopharyngioma

A

Beta-catenin gene

41
Q

Mutation associated with papillary craniopharyngioma

A

BRAF/V600E mutation

42
Q

CNS tumor derived from remnant of Rathke’s pouch

A

Craniopharyngioma

43
Q

Tumor signaling pathway found in adamantinomatous craniopharyngioma

A

WNT pathway

44
Q

Tumor signaling pathway found in papillary craniopharyngioma

A

MAPK pathway

45
Q

Imaging shows displaced midbrain with hydrocephalus and suprasellar cyst mass

A

Cranipharyngioma

46
Q

Grossly a suprasellar, partially cystic, focally calcified mass filled with brownish-yellow viscous fluid rich in protein and cholesterol crystals

A

Craniopharyngioma

47
Q

Histology shows epithelial islands containing amorphous pink areas of keratinization, surrounded by a layer of palisading cells

A

Craniopharyngioma

48
Q

Imaging shows one or more discrete lesions that are usually ring-enhancing. Grossly a well-circumscribed mass(es) along the gray-white junction.

A

Brain metastasis

49
Q

Brain metastasis type that is common in malignancies that spread to bone

A

Epidural metastasis

50
Q

Brain metastasis type that is common in hematologic malignancies and solid tumors

A

Leptomeningeal metastasis

51
Q

Tumors associated with neurofibromatosis 1

A

Neurofibromas
Optic gliomas

52
Q

Mutation in neurofibromatosis 1

A

NF1 on chromosome 17

53
Q

Tumors associated with neurofibromatosis 2

A

Bilateral schwannomas
Meningioma

54
Q

Mutation in neurofibromatosis 2

A

NF2 on chromosome 22 encoding merlin protein

55
Q

Tumors associated with tuberous sclerosis

A

Cortical tubers
Subependymal nodules

56
Q

Mutations associated with tuberous sclerosis

A

TCS1 on chromosome 9 –> hamartin
TCS2 on chromosome 16 –> tuberin

57
Q

Mutation associated with Von Hippel-Lindau syndrome

A

VHL on chromosome 3

58
Q

Tumors associated with Von Hippel-Lindau syndrome

A

Hemangioblastomas - cerebellar and retinal
Pheochromocytomas
Multiple bilateral kidney cysts and clear cell renal carcinoma

59
Q

WHO grade of schwannoma

A

Grade 1 –> benign

60
Q

Benign nerve sheath tumor arising from Schwann cells

A

Schwannoma

61
Q

Merlin protein

A

Cytoskeletal protein that mediates contact inhibition

62
Q

Common location of vestibular schwannomas

A

Internal auditory meatus at the cerebellopontine angle

63
Q

Well-circumscribed, variegated massed with a tan appearance and macro-cysts. Appears as densely enhancing lesion on MRI.

A

Schwannoma

64
Q

Hypercellular and hypocellular regions with Verocay bodies, characterized by distinct palisades with a fibrillary core. With well-formed capsules and hyalinized vessels. S100 strong and diffusely positive, and highly positive for pericellular collagen IV.

A

Schwannoma

65
Q

Hamartomatous neurocutaneous disease

A

Tuberous sclerosis

66
Q

Skin findings in tuberous sclerosis

A

Adenoma sebaceum
Hypomelanotic macule
Shagreen patches

67
Q

Cortical hamartomas, subependymal nodules, and subependymal giant cell astrocytomas

A

Tuberous sclerosis

68
Q

Non-CNS tumors associated with tuberous sclerosis

A

Angiomyolipomas in kidneys
Rhabdomyomas in heart

69
Q

Tumor syndrome associated with impaired down-regulation of mTOR signaling pathways

A

Tuberous sclerosis

70
Q

Tumor syndrome associated with increased HIF-1

A

Von Hippel-Lindau disease

71
Q

Mutation in Sturge-Weber syndrome

A

GNAQ gene somatic mosaic mutation

72
Q

Syndrome associated with ecephalotrigeminal angiomatosis

A

Sturge-Weber syndrome

73
Q

Conditions associated with Sturge-Weber syndrome

A

Mental retardation
Epileptic spasm as infancy
Early-onset glaucoma

74
Q

CT shows tram-track sign of cortical and subcortical calcification

A

Sturge-Weber syndrome

75
Q

T1 MRI, post-gadolinium, shows prominent leptomeningeal enhancement of the affected area (pial angiomatosis)

A

Sturge-Weber syndrome

76
Q

Inheritance of tuberous sclerosis

A

Autosomal dominant

Neuro II (21 decks)

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