CNS neoplasms Flashcards

(76 cards)

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
Tumor that is derived from ependymal cells lining the ventricular surface, frequently arising from wall of fourth ventricle, within the posterior fossa
Ependymoma
26
Histology shows perivascular pseudorosettes and ependymal rosettes, usually GFAP positive
Ependymoma
27
Electron microscopy findings in ependymoma
Cilia and microvilli Junctional complexes
28
Most common malignant brain tumor of childhood
Medulloblastoma
29
Poorly differentiated tumor of the cerebellum, common in childhood
Medulloblastoma
30
MRI shows densely enhancing mass in the posterior fossa, within the cerebellum
Medulloblastoma
31
Drop metastases
Dissemination through CSF to spinal cord
32
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.
Medulloblastoma
33
Characteristics of small blue cells on histology
Densely packed cells with hyperchromatic nuclei and scan cytoplasm
34
WHO grade of meningioma
Grade I, slow-growing and benign
35
CNS tumor associated with ionizing radiation and NF2, with a female predominance
Meningioma
36
Grossly a well-demarcated, firm, rubbery dural-based tumor
Meningioma
37
Histology shows whorls of arachnoid cap cells, laminated calcified psammoma bodies. It is EMA, PR, and somatostain positive. It is GFAP and synaptophysin negative.
Meningioma
38
Two types of craniopharyngioma and there associated age
Adamantinomatous --> children Papillary --> adults
39
Most common non-neuroepithelial intracranial tumor in children
Adamantinomatous craniopharyngioma
40
Mutation associated with adamantinomatous craniopharyngioma
Beta-catenin gene
41
Mutation associated with papillary craniopharyngioma
BRAF/V600E mutation
42
CNS tumor derived from remnant of Rathke's pouch
Craniopharyngioma
43
Tumor signaling pathway found in adamantinomatous craniopharyngioma
WNT pathway
44
Tumor signaling pathway found in papillary craniopharyngioma
MAPK pathway
45
Imaging shows displaced midbrain with hydrocephalus and suprasellar cyst mass
Cranipharyngioma
46
Grossly a suprasellar, partially cystic, focally calcified mass filled with brownish-yellow viscous fluid rich in protein and cholesterol crystals
Craniopharyngioma
47
Histology shows epithelial islands containing amorphous pink areas of keratinization, surrounded by a layer of palisading cells
Craniopharyngioma
48
Imaging shows one or more discrete lesions that are usually ring-enhancing. Grossly a well-circumscribed mass(es) along the gray-white junction.
Brain metastasis
49
Brain metastasis type that is common in malignancies that spread to bone
Epidural metastasis
50
Brain metastasis type that is common in hematologic malignancies and solid tumors
Leptomeningeal metastasis
51
Tumors associated with neurofibromatosis 1
Neurofibromas Optic gliomas
52
Mutation in neurofibromatosis 1
NF1 on chromosome 17
53
Tumors associated with neurofibromatosis 2
Bilateral schwannomas Meningioma
54
Mutation in neurofibromatosis 2
NF2 on chromosome 22 encoding merlin protein
55
Tumors associated with tuberous sclerosis
Cortical tubers Subependymal nodules
56
Mutations associated with tuberous sclerosis
TCS1 on chromosome 9 --> hamartin TCS2 on chromosome 16 --> tuberin
57
Mutation associated with Von Hippel-Lindau syndrome
VHL on chromosome 3
58
Tumors associated with Von Hippel-Lindau syndrome
Hemangioblastomas - cerebellar and retinal Pheochromocytomas Multiple bilateral kidney cysts and clear cell renal carcinoma
59
WHO grade of schwannoma
Grade 1 --> benign
60
Benign nerve sheath tumor arising from Schwann cells
Schwannoma
61
Merlin protein
Cytoskeletal protein that mediates contact inhibition
62
Common location of vestibular schwannomas
Internal auditory meatus at the cerebellopontine angle
63
Well-circumscribed, variegated massed with a tan appearance and macro-cysts. Appears as densely enhancing lesion on MRI.
Schwannoma
64
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.
Schwannoma
65
Hamartomatous neurocutaneous disease
Tuberous sclerosis
66
Skin findings in tuberous sclerosis
Adenoma sebaceum Hypomelanotic macule Shagreen patches
67
Cortical hamartomas, subependymal nodules, and subependymal giant cell astrocytomas
Tuberous sclerosis
68
Non-CNS tumors associated with tuberous sclerosis
Angiomyolipomas in kidneys Rhabdomyomas in heart
69
Tumor syndrome associated with impaired down-regulation of mTOR signaling pathways
Tuberous sclerosis
70
Tumor syndrome associated with increased HIF-1
Von Hippel-Lindau disease
71
Mutation in Sturge-Weber syndrome
GNAQ gene somatic mosaic mutation
72
Syndrome associated with ecephalotrigeminal angiomatosis
Sturge-Weber syndrome
73
Conditions associated with Sturge-Weber syndrome
Mental retardation Epileptic spasm as infancy Early-onset glaucoma
74
CT shows tram-track sign of cortical and subcortical calcification
Sturge-Weber syndrome
75
T1 MRI, post-gadolinium, shows prominent leptomeningeal enhancement of the affected area (pial angiomatosis)
Sturge-Weber syndrome
76
Inheritance of tuberous sclerosis
Autosomal dominant