Tumors of the Nervous System

Question 1

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

Answer 1

Glioblastoma

Question 2

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

Answer 2

Glomeruloid body

Question 3

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

Answer 3

Gliomatosis cerebri

Question 4

What mutations result in a primary glioblastoma? Secondary glioblastoma?

Answer 4

1) EGFR

2) PDGF

Question 5

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

Answer 5

Pilocytic Astrocytoma

Question 6

Where are pilocytic astrocytomas most commonly found?

Answer 6

Cerebellum, third ventricle, or the optic nerve

Question 7

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

Answer 7

1) Temporal lobe

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

Question 8

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

Answer 8

Oligodendroglioma

Question 9

What are the mutations found in oligodendrogliomas?

Answer 9

THINK: O119odendroglioma

1) Loss of heterozygosity for chromosomes 1 and 19

Question 10

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

Answer 10

Myxopapillary ependymoma

Question 11

Where are Myxopapillary ependymoma most commonly found?

Answer 11

1) Filum terminale of the spinal cord

Question 12

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

Answer 12

1) Ganglioma

Question 13

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

Answer 13

Medulloblastoma

Question 14

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

Answer 14

1) It is exquisitely radiosensitive

Question 15

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

Answer 15

Rhabdoid cell

Question 16

What chromosome mutation are commonly associated with meduloblastoma?

Answer 16

1) chromosome 17

Question 17

Highly aggressive tumor of the very young

Answer 17

Atypical teratoid/rhabdoid tumor

Question 18

What tumor is commonly associated with calcifications?

Answer 18

Oligodendrogliomas

Question 19

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

Answer 19

Primary CNS lymphoma

Question 20

Where do germ cell tumors in the CNS usually arise?

Answer 20

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

Question 21

What causes an increased risk for meningiomas?

Answer 21

1) Radiation therapy

2) Mutation in NF2

Question 22

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

Answer 22

Meningioma

Question 23

What is the common genetic cause of meningiomas?

Answer 23

1) Loss of chromosome 22

Question 24

What does a meningioma arise from?

Answer 24

1) Arise from the meningothelial cell of the arachnoid

Question 25

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

Answer 25

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

Question 26

S-100 positive indicates?

Answer 26

Schwannoma

Question 27

Where are Schwannomas most commonly found?

Answer 27

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

Question 28

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

Answer 28

Ependymoma

Question 29

Glomeruloid body and pseudopalisading indicates?

Answer 29

Glioblatoma

Question 30

What is the mutation observed in Neurofibromatosis type 1?

Answer 30

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

Question 31

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

Answer 31

Neurofibromatosis Type 1

Question 32

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

Answer 32

Neurofibromatosis Type 2

Question 33

What is the cause of Neurofibromatosis type 2?

Answer 33

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

Question 34

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

Answer 34

Tuberous Sclerosis complex

Question 35

What is the cause of Tuberous Sclerosis complex?

Answer 35

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

Question 36

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

Answer 36

Von Hippel Lindau disease

Question 37

What is increased in a primary glioblastoma?

Answer 37

Increased EGFR

Question 38

What is increased in secondary glioblastoma?

Answer 38

Increase PDGF

Question 39

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

Answer 39

1) Pilocytic Astrocytoma

Question 40

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

Answer 40

Oligodendroglioma

Question 41

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

Answer 41

Glioblastoma

Question 42

What mutations are commonly found in Ependymomas?

Answer 42

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

Question 43

What is a cause of a Schwannoma?

Answer 43

1) Mutation in NF2

Question 44

What mutation is commonly associated with neurofibromas?

Answer 44

1) NF1

Question 45

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

Answer 45

Cutaneous Neurofibroma

Question 46

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

Answer 46

Malignant Peripheral Nerve Sheath Tumor

Question 47

Tumor with an irregular ropey tangled mass of musculature

Answer 47

Malignant Peripheral Nerve Sheath tumor

Question 48

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?

Answer 48

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

Question 49

What is the result of a mutation in NF1?

Answer 49

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

Question 50

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?

Answer 50

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

Question 51

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?

Answer 51

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

Question 52

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?

Answer 52

1) Von Hippel Lindaue Disease | 2) Mutation of VHL gene (chromosome 3)

Question 53

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

Answer 53

Meningioma

Question 54

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

Answer 54

Sturge- Weber syndrome

Question 55

Lisch nodule

Answer 55

Neurofibromatosis type I

Question 56

Butterfly glioma

Answer 56

Glioblastoma

Question 57

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

Answer 57

Oligodendroglioma

Question 58

Perivascular Pseudorossetes

Answer 58

Ependyoma

Question 59

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

Answer 59

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

Question 60

Von Recklinghausen's disease

Answer 60

Neurofibromatosis type 1

Question 61

What type of tumors are GFAP positive? Synaptophysin?

Answer 61

1) Astrocytoma, Oligodendroglioma, and ependymal | 2) Neuroma

Question 62

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

Answer 62

Neuroblastoma

Question 63

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

Answer 63

Craniopharyngioma

Question 64

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

Answer 64

1) Li-Fraumeni syndrome | 2) p53