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Nervous System > Tumors of the Nervous System > Flashcards

Tumors of the Nervous System Flashcards

1
Q

Tumor that presents microscopically with necrosis in a serpentine pattern; necrosis bordered by Pseudopalisading pattern; extensive vascular/endothelial cell proliferation; Glomeruloid bodies present

A

Glioblastoma

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2
Q

Double layer of endothelail cells that tufts to form a ball-like structure.

A

Glomeruloid body

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3
Q

What is the condition called when multiple regions of the brain are infiltrated by neoplstic astrocytes?

A

Gliomatosis cerebri

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4
Q

What mutations result in a primary glioblastoma? Secondary glioblastoma?

A

1) EGFR

2) PDGF

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5
Q

Cystic glioma that is benign; typically occurs in children; commonly found in the cerebellum; composed of bipolar cells with long “hair like” processess; GFAP positive; necrosis and mitoses are uncommon; Rosenthal fibers

A

Pilocytic Astrocytoma

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6
Q

Where are pilocytic astrocytomas most commonly found?

A

Cerebellum, third ventricle, or the optic nerve

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7
Q

Where are pleomorphic Xanthoastrocytomas located? What do they appear microscopically?

A

1) Temporal lobe

2) Appears microscopically as a high grade tumor due to atypia

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8
Q

Glioma that presents usually in the 4th to 5th decades of life; often found in the cerebrum, appears well circumscribed often with cysts, focal hemorrhage, and calcifications

A

Oligodendroglioma

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9
Q

What are the mutations found in oligodendrogliomas?

A

THINK: O119odendroglioma

1) Loss of heterozygosity for chromosomes 1 and 19

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10
Q

Ependymoma like tumor that appears in the filum terminale of the spinal cord; contains papillary elements in a myxoid background along with ependymoma like cells

A

Myxopapillary ependymoma

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11
Q

Where are Myxopapillary ependymoma most commonly found?

A

1) Filum terminale of the spinal cord

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12
Q

Neuronal tumor that at first progresses slowly and then rapidly becomes active

A

1) Ganglioma

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13
Q

Poorly differentiated neoplasm exclusively found int he cerebellum; commonly found in children; Composed of cells with small scant cytoplasm and hyperchromatic nuclei

A

Medulloblastoma

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14
Q

What is an important property that influences the treatment of Medulloblastoma?

A

1) It is exquisitely radiosensitive

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15
Q

Cell with eosiniophilic cytoplasm, sharp cell borders, and eccentrically located nuclei

A

Rhabdoid cell

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16
Q

What chromosome mutation are commonly associated with meduloblastoma?

A

1) chromosome 17

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17
Q

Highly aggressive tumor of the very young

A

Atypical teratoid/rhabdoid tumor

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18
Q

What tumor is commonly associated with calcifications?

A

Oligodendrogliomas

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19
Q

Tumor consisting of cells that express Bcl-6 and show “Hooping” on silver stain; multifocal masses

A

Primary CNS lymphoma

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20
Q

Where do germ cell tumors in the CNS usually arise?

A

Occur along the midline, commonly in the pineal and suprasellar regions

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21
Q

What causes an increased risk for meningiomas?

A

1) Radiation therapy

2) Mutation in NF2

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22
Q

Benign tumor consisting of round masses with dural bases that may compress underlying brain

A

Meningioma

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23
Q

What is the common genetic cause of meningiomas?

A

1) Loss of chromosome 22

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24
Q

What does a meningioma arise from?

A

1) Arise from the meningothelial cell of the arachnoid

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25
Q

What is the difference between a schwannoma and a plexiform neurofibroma?

A

1) Schwannomas can be separated from the nerve; plexiform neurofibroma cannot

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26
Q

S-100 positive indicates?

A

Schwannoma

27
Q

Where are Schwannomas most commonly found?

A

1) At the cerebellopontine angle; also known as acoustic neuroma

28
Q

Tumor composed of regular cells and abundant granular chromatin; cells form gland-like round elonggated structures and perivascular pseudorosettes

A

Ependymoma

29
Q

Glomeruloid body and pseudopalisading indicates?

A

Glioblatoma

30
Q

What is the mutation observed in Neurofibromatosis type 1?

A

1) Autosomal dominant mutation of the NF1 gene located on chromosome 17
2) Results in the absence of neurofibromin and uninhibited RAS

31
Q

Neurofibromas, gliomas of the optic nerve, pigmented nodules of the iris, and cutaneous hyper pigmented macules (Cafe Au Lait Spots)

A

Neurofibromatosis Type 1

32
Q

Disease associated with bilateral acoustic neuromas and meningiomas, as well as ependyoma of the spinal cord

A

Neurofibromatosis Type 2

33
Q

What is the cause of Neurofibromatosis type 2?

A

1) Autosomal dominant mutation of the NF2 gene on chromosome 22
2) Results in a mutated merlin protein

34
Q

Disease associated with the development of hamartomas and benign neoplasms of the brain; Ash-Leaf patches, angiofibromas

A

Tuberous Sclerosis complex

35
Q

What is the cause of Tuberous Sclerosis complex?

A

1) Autosomal dominant mutation of the of TSC1 (chromosome 9) or TSC2 (chromosome 16)

36
Q

Disease characterized by hemangioblastomas and cysts involving the pancreas, liver, and kidneys

A

Von Hippel Lindau disease

37
Q

What is increased in a primary glioblastoma?

A

Increased EGFR

38
Q

What is increased in secondary glioblastoma?

A

Increase PDGF

39
Q

Tumor found in the cerebellum of a child composed of bipolar cells with hair like processes. What should you suspect?

A

1) Pilocytic Astrocytoma

40
Q

Well circumscribed mass with cysts, focal hemorrhage, and calcifications; consists of small uniform round cells with perinuclear halos

A

Oligodendroglioma

41
Q

Single ring enhancing mass; may cross the corpus callosum (butterfly lesion); onset is rapid and survival is low

A

Glioblastoma

42
Q

What mutations are commonly found in Ependymomas?

A

1) NF2 mutations - encodes Merlin protein (chromosome 22)

43
Q

What is a cause of a Schwannoma?

A

1) Mutation in NF2

44
Q

What mutation is commonly associated with neurofibromas?

A

1) NF1

45
Q

Tumor present in the dermis and subcutaneous fat that is composed of spindle cells and highly collagenized stroma

A

Cutaneous Neurofibroma

46
Q

Poorly defined tumor that frequently infiltrates along the axis of a parent nerve and invades the tissue

A

Malignant Peripheral Nerve Sheath Tumor

47
Q

Tumor with an irregular ropey tangled mass of musculature

A

Malignant Peripheral Nerve Sheath tumor

48
Q

Characterized by neurofibromas, gliomas of the optic nerve, pigmented nodules of the iris, and cafe au lait spots? What is the syndrome? What is the gene mutated? What is the protein it encodes?

A

1) Neurofibromatosis type 1
2) NF1 gene (chromosome 17)
3) Neurofibromin

49
Q

What is the result of a mutation in NF1?

A

1) Neurofibromin becomes absent and results in the uninhibited RAS pathway

50
Q

Syndrome characterized by bilateral acoustic neuromas and multiple meningiomas, may also have ependymoma. What is the syndrome? What is the gene mutated? What is the protein it encodes?

A

1) Neurofibromatosis Type 2
2) NF2 (chromosome 22)
3) Merlin protein

51
Q

Syndrome characterized by the development of hamartomas and benign neoplasms of the brain; Ash-Leaf patches and angiofibromas are also present. What is the syndrome? What is the gene lost? What does the gene encode?

A

1) Tuberous Sclerosis Complex
2) Mutation in TSC-1 & 2
3) TSC-1 = hamartin; TSC-2 = tuberin

52
Q

Syndrome characterized by hemangioblastomas and cysts involving the pancreas, liver, and kidneys; may also develop renal cell carcinoma or a pheochromocytoma. What is the syndrome? What gene is mutated?

A

1) Von Hippel Lindaue Disease

2) Mutation of VHL gene (chromosome 3)

53
Q

Presence of round, eosinophilic laminar structures composed of dense calcifications with surrounding collagen

A

Meningioma

54
Q

Congenital disorder with port-wine stains, typically in V1 opthalamic distribution; ipsilateral leptomeningeal angioma; pheochromocytomas; causes glaucoma, mental retardation, seizures

A

Sturge- Weber syndrome

55
Q

Lisch nodule

A

Neurofibromatosis type I

56
Q

Butterfly glioma

A

Glioblastoma

57
Q

Infiltrating tumor with perinuclear halos and prominent delicate vasculature; “fried egg appearance”

A

Oligodendroglioma

58
Q

Perivascular Pseudorossetes

A

Ependyoma

59
Q

How do you differentiate between a medulloblastoma and a pilocytic astrocytoma?

A

1) Pilocytic astrocytoma has cystic spaces on CT; medulloblastoma is soley solid tumor

60
Q

Von Recklinghausen’s disease

A

Neurofibromatosis type 1

61
Q

What type of tumors are GFAP positive? Synaptophysin?

A

1) Astrocytoma, Oligodendroglioma, and ependymal

2) Neuroma

62
Q

What causes opsoclonus- myoclonus syndrome (non-rhythmic eye movements with myoclonus)?

A

Neuroblastoma

63
Q

Suprasellar mass consisting of solid tumor, cystic component, and a calcified component

A

Craniopharyngioma

64
Q

Syndrome that predisoposes pt. to the early development of cancer? What mutation is present?

A

1) Li-Fraumeni syndrome

2) p53

Nervous System (5 decks)

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