Path- 2: Tumors

Question 1

50-75% of all tumors are either primary or secondary?

Answer 1

Primary

Question 2

What are the 3 gliomas?

Answer 2

Astrocytomas, oligodendrogliomas, ependymomas

the 3 glia of the CNS lol

Question 3

What comprises ~80% of adult primary tumors?

Answer 3

Fibrillary astrocytoma

Question 4

Where are the fibrillary astrocytomas located in the CNS?

Answer 4

Cerebal hemispheres

Question 5

When do fibrillary astrocytomas occur during oens life?

Answer 5

3rd or 4th decade of life

Question 6

What are the clinical Sx of fibrillary astrocytomas?

Answer 6

szrs, headaches, no focal neurological deficits

Question 7

True or False: Fibrillary astrocytomas have focal dense cellularity, proliferation of vascular elements, mitotic activity, and zones of necrosis.

Answer 7

FALSE

Question 8

What is the life spans of pt’s with Glioblastomas following Dx?

Answer 8

8-10 months.

Question 9

Does this occur in younger or older pt’s with a diagnosed glioblastoma: short, rapidly progressive, arising without preexisting low grade tumors.

Answer 9

Older

Question 10

Does this occur in younger or older pt’s with a diagnosed glioblastoma: prveiously diagnosed low grade astrocytoma with p53 mutations.

Answer 10

Younger

Question 11

What is the hallmark of GBM’s (grade IV glioblastomas)?

Answer 11

Necrosis

Question 12

What age groups does pilocytic astrocytomas effect?

Answer 12

young adults and kids

Question 13

Are pilocytic astrocytomas benign or malignant?

Answer 13

Relatively benign

Question 14

Where in the brain are pilocytic astrocytomas?

Answer 14

CEREBELLUM

Sometimes can be in the floor of the 3rd vent and press on the ant optic pathway

Question 15

What types of tests can you run to Dx a pilocytic astrocytoma?

Answer 15

B-crytallin and HSP’s, immunoreactive for alpha-1-antitrypsin

comes up as a bright-white cyst.

Question 16

Where in the brain do Pleomorphic Xanthoastrocytoma’s occur?

Answer 16

Superficial temporal sides of the hemispheres

Question 17

Pt’s have what main Sx with PX’s?

Answer 17

Seizures

Question 18

Whgat age group does PX’x occur?

Answer 18

Kids and young adults

Question 19

What is the composition of the tumors in PX’s?

Answer 19

mix of pleomorphic cells, raning from fibrillary to giant multinucleated cells with intracellilar lipid vacuoles (xanthomas)

Question 20

What is the prognosis of PX’s?

Answer 20

may progress to grade III or Grade (IV)

Question 21

What pt’s have brainstem gliomas?

Answer 21

first 2 decades of life

Question 22

What are intial pontine gliomas?

Answer 22

most common pediatric, aggressive, short survival

Question 23

What are exophylic tumors?

Answer 23

Happen at the cervicomedullary jxn, less aggressive than initial pontine gliomas

Question 24

Are tectal gliomas more or less benign that exophylic tumors?

Answer 24

More benign

Question 25

In adults, what types of brainstem gliomas can occur?

Answer 25

Rare intrinsic pontine gliomas

Question 26

Define: 5-15% of gliomas, happen in young and middle aged adults, in cerebral hemispheres white matter.

Answer 26

Oligodendrogliomas

Question 27

What is the characteristics of oligodendrogliomas that could differentiate from say fibrillary astrocytomas (which also happen in the hemispheres)?

Answer 27

Oligodendrogliomas represent foci of intratumoral ***calcification***

Question 28

What do you see on cross-sections of the brain of oligodendrogliomas?

Answer 28

Chicken wire-like blood vessels and fried egg-like cells with clear cytoplasm and well defined borders with H&E stain

Question 29

What is the mutation to cause oligodendrogliomas?

Answer 29

loss of chromosome 1p and 19q

Question 30

What is the prognosis of oligodendrogliomas?

Answer 30

better than astrocytomas, survival of 5-10 years

Question 31

Define: these are tumors that arise next to CSF-lined structures.

Answer 31

Ependymomas

Question 32

If the ependymoma occurs with people in their first 2 decades of life, where do they usually occur in the CNS?

Answer 32

near the 4th vent

Question 33

If the ependymoma occurs with people in adulthood, where do they usually occur in the CNS?

Answer 33

Spinal cord

Question 34

What is the clinical Sx/prognosis of ependymomas?

Answer 34

psoterior fossa ependymomas occur with hydrocephalus secodnary to obstruction, often with a poor prognosis (~4 years)

Question 35

What shows on histology of ependymomas?

Answer 35

Rosettes or perivascular pseudorosettes

Question 36

Define: non-neoplasic lesion, occurs in young adutls, attahced to roof of 3rd vent --> noncommunicating hydrocephalus, headache.

Answer 36

Colloid cyst

Question 37

Where do central neurocytomas occur?

Answer 37

Intraventicular (usually lateral)

Question 38

What ages affect central neurocytomas?

Answer 38

Young adults (25-30)

Question 39

Sx of central neurocytomas?

Answer 39

nausea, vomiting, headache due to obstructive hydrocephalus

Question 40

Tx of central neurocytomas?

Answer 40

Resection (these are just benign balls of shit)

Question 41

What are gangliogliomas?

Answer 41

Mixture of glial and ganglial cells, most common CNS tumor, contain mature appearing ganglion cells

Question 42

Sx of gangliogliomas?

Answer 42

Seizures

Question 43

Where in the brain are gangliogliomas?

Answer 43

In temporal (often with cyst component)

Question 44

What ages does Dysembryoplastic neuroepithelial tumors effect?

Answer 44

Kids | think "embryo" --> kids

Question 45

What are the Sx and prognosis of Dysembryoplastic neuroepithelial tumors?

Answer 45

rare, low grade, good prognosis, happen in superficial temporal lobe

Question 46

What is the most common poorly defined differentiated neoplasm?

Answer 46

Medullablastoma

Question 47

Which ages doe smedullablastomas occur in?

Answer 47

Kids (~20% of brain tumors)

Question 48

What are the clinical features of medullablastomas?

Answer 48

highly malignant, VERY RADIOSENSTITIVE, loss of 17p --> poor prognosis (5 yr survival)

Question 49

Which ages does atypical rhabdoid/teratoid tumors affect?

Answer 49

kids < 5 yrs

Question 50

What is the mutation to cause rhabdoid/teratoid tumors?

Answer 50

chromosme 22 alterations

Question 51

What are the clinical features of rhabdoid/teratoid tumors?

Answer 51

Death within a year

Question 52

Which ages get primary CNS lymphomas?

Answer 52

Old people.

Question 53

What types of pts get primary CNS lymphomas?

Answer 53

immunosupressed people

Question 54

Where do primary CNS lymphomas come from?

Answer 54

from B-cells

Question 55

What is the prognosis of primary CNS lymphomas?

Answer 55

poor because it's very aggressive.

Question 56

What are the clinical features of pirmary CNS lymphomas?

Answer 56

perivascular localization, may involve neuroparenchyma if systemic

Question 57

What are the 2 age groups that get craniopharyngiomas?

Answer 57

10-14 years and the second peak is in the 7th decade

Question 58

Sx of craniopharyngiomas?

Answer 58

headaches and increased ICP (from obstructive hydrocephalus), visual problems

Question 59

Clinical presentation of craniopharyngiomas?

Answer 59

80-87% are calcified

Question 60

Tx of craniopharyngiomas?

Answer 60

CUT IT OUT

Question 61

Why are there bilateral temporal hemianopnia with craniopharyngiomas?

Answer 61

COmpression of the optic chiasm

Question 62

Who gets meningiomas?

Answer 62

Adults

Question 63

What are the clinical feastures of meningiomas?

Answer 63

benign, attached to dura, slow growing

Question 64

What are the freuqent sites of metastatic tumors?

Answer 64

Meningies (present as mass lesions)

Question 65

Define: these happen when central and peripheral immune responses to tumor antigens cross-react in the CNS or PNS.

Answer 65

Paraneoplastic syndromes

Question 66

What are the clinical features of encephalomyelitis?

Answer 66

- eye movement disorders - limbic system - cerebellar degeneration - Lambert-Eaton - sensory neuropathy

Question 67

Where can peripheral nerve sheath tumors arise?

Answer 67

in both brain and PNS

Question 68

What are the 3 characteristics of Peripheral nerve sheath tumors?

Answer 68

Schwann cells (express S-100 Ag), perineural cells, fibroblasts

Question 69

What are the clinical featueres of schwannomas?

Answer 69

benign, arise from neural crest cells, space occupying lesions

Question 70

What is the mutation to cause schwannomas?

Answer 70

mutations of chromosome 22, loss of **merlin**

Question 71

What does Merlin do?

Answer 71

restricts actions of cell surface growth factor EGFR

Question 72

Where do msot schwannomas occur?

Answer 72

VIII (cerebellopontine angle) --> tinnitus and hearing loss (acoustic neuroma)

Question 73

What is the inheritance of von Hipple Lindau disease?

Answer 73

AD

Question 74

Pt's with vHLD develop hemangioblastomas in which 2 locations?

Answer 74

Cerebellum and retina

Question 75

vHL pt's have an increased risk of developing what 2 other tumors?

Answer 75

Renal cell carcinoma and pheochromocytomas

Question 76

What is the inheritance of tuberous sclerosis?

Answer 76

AD

Question 77

What are the clinical features of tuberous sclerosis?

Answer 77

Harmatomous + benign neoplasms of the brain and other tissues. Can have cortical tuberous and subependymal giant cell astrocutomas in the Cx of the brain.

Question 78

What happens if the tubers in the brain grow and invade the ventricular system?

Answer 78

Seizures

Question 79

What are the clinical findings of tuberous sclerosis?

Answer 79

tubers/potatoes on CT of the brain, may also form in heart, liver and kidneys. skin and fingerenails may show ungual fibromas

Question 80

What is the inheritance of neurofibromatosis type 1 (NF1)?

Answer 80

AD

Question 81

Incidence of NF1?

Answer 81

1/3000

Question 82

What tumors form in NF1?

Answer 82

plexiform and solitary neurofibromas (plexiform is more malignant), gliomas of the optic nerve, Lisch nodules

Question 83

If there is a mutation of the NF1 gene, what is activated to cause proliferation of the cancer cells?

Answer 83

RAS (normally inhibited by NF1 on chromosome 17)

Question 84

Where are Lisch nodules?

Answer 84

They're yellow elevations on the iris

Question 85

What is the inheritance of NF2?

Answer 85

AD

Question 86

What are the tumors assocated with NF2?

Answer 86

VIII schwannomas, multiple meningiomas and ependymomas of the spinal cord

Question 87

Incidence of NF2?

Answer 87

1/40,000

Question 88

Is there any malignant transformation of NF2?

Answer 88

No

Question 89

What are the Sx of NF2?

Answer 89

heaing loss, facial weakness, dizziness, ataxia, cataracts at an early age