Neoplastic Disease

Question 1

Where is the pterion?

Answer 1

located at the junction of the frontal, parietal, temporal, and greater wing of sphenoid bones It is located two fingerbreadths above the zygomatic arch and a thumb’s breadth behind the frontal process of the zygomatic bone

Question 2

Where is the asterion? What does it signify?

Answer 2

located at the junction of the lambdoid, occipitomastoid, and parietomastoid sutures lies on top of the lower half of the transverse/ sigmoid sinus junction

Question 3

What is the bregma?

Answer 3

located at the junction of the coronal and sagittal sutures

Question 4

What genetic mutations are characteristic for IDH mutant diffuse astrocytomas?

Answer 4

ATRX loss P53 mutation

Question 5

Pilocytic astrocytomas on MRI imaging generally are ________ but when in the optic nerve, are generally _____.

Answer 5

cystic with mural nodule solid and enhancing

Question 6

What disease is associated with pilocytic astrocytoma?

Answer 6

NF-1

Question 7

Describe the epidemiology and symptomology of pilocytic astrocytomas based on location.

Answer 7

Question 8

What is the 5 and 10 yr survival for PXAs?

Answer 8

80 and 70%

Question 9

What is the most common location for oligodendrogliomas?

Answer 9

frontal lobe (50%)

Question 10

Most GBMs that develop from other astrocytomas are _____.

Answer 10

IDH mutants

Question 11

What is the Stupp protocol?

Answer 11

maximal safe resection followed by adjuvant chemotherapy/radiation

fractionated radiation of 2 Gy 5 days/week for six weeks total 60 Gy, plus daily temozolomide at 75 mg/m^2, 7 days a week for 60 days followed by six cycles of adjuvant temozolomide 150-200 mg/m^2 for 5 of 28 days

Question 12

In the fourth ventricle, ependymomas usually arise from the _____.

Answer 12

floor

Question 13

Spinal ependymomas are associated with a syrinx in what percent of cases?

Answer 13

90%

Question 14

“Tiger stripes” in unilaterally in the cerebellum are pathognomonic for _____.

Answer 14

Lhermitte Duclos disease

Question 15

What is the treatment for Lhermitte Duclos disease?

Answer 15

surgical resection is curative

Question 16

What is the chance for seizure freedom after DNET resection?

Answer 16

80% at 5 years, 60% at 10 years

Question 17

What’s the difference between a gangliocytoma and ganglioglioma?

Answer 17

Gangloicytoma: WHO grade I growth of mature neurons

Ganglioglioma: WHO grade I and II mixed population of ganglion and glial cells

Question 18

What is the semiology of hypothalamic hamartomas? What other symptoms can they classically present with?

Answer 18

gelastic seizures

precocious puberty

Question 19

What are the different types of paraganglioms? Provide name and location.

Answer 19

Question 20

What is the overall 5 year survival rate for paragangliomas?

Answer 20

90%

Question 21

What percent of pineoblastomas have CSF seeding?

Answer 21

50%

Question 22

What is the treatment for pineoblastoma?

Answer 22

surgical resection + cranial/spinal radiation and chemotherapy

Question 23

What is the survival rate in pineoblastoma?

Answer 23

Fifteen percent 10-year survival in case of residual tumor versus 100% if no residual left

Question 24

What chromosome abnormality can be seen with medulloblastoma?

Answer 24

loss of 17p in 50% of patients

Question 25

What is the 5 year survival for medulloblastoma in standrard risk patients (no mets, older than 3 years, Gross total resection)?

Answer 25

100% if ERBB-2 tumor protein negative, 54% if positive

Question 26

There are many types of meningiomas. What are they and what WHO grade system do they fall under?

Answer 26

WHO Grade I: meningothelial, fibrous, transitional, psammomatous, angiomatous, microcystic, secretory, metaplastic, lymphoplasmacyte-rich.

WHO Grade II: atypical, clear cell (predilection for spinal cord and posterior fossa), chordoid (“chordoma-like”), brain invasive.

WHO Grade III: anaplastic, rhabdoid, papillary.

Question 27

Describe the Simpson Grading system.

Answer 27

Question 28

What is the most common primary CNS sarcome (malignant mesenchymal tumor)?

Answer 28

fibrosarcoma

Question 29

Soitary fibrous tumors are distinguished form hemangiopericytomas and meningiomas by _____.

Answer 29

CD 34 +

Question 30

Leiomyosarcomas are associated with what virus?

Answer 30

EBV

Question 31

What is the median survival in primary CNS lymphoma?

Answer 31

Median survival 1–4 months without treatment, 1–4 years when treated (2–6 months in AIDS)

Question 32

What is the treatment for germinomas?

Answer 32

biopsy followed by chemo/radiation

Question 33

Klinefelter’s syndrome is associated with what CNS tumor?

Answer 33

embryonal carcnomas

Question 34

What percent of pituitary adenomas are micor vs macor?

Answer 34

50-50 split

Question 35

“Stalk effect” elevated prolactin levels fall into what range?

Answer 35

25 - 150 ng/mL

Question 36

For adrenocorticotrophin releasing pituitary adenomas, what medication can be used as a second line treatment?

Answer 36

ketoconazole

Question 37

Summarize the the most common mets, percent with cerebral mets, and general survival with treatment.

Answer 37

Question 38

What were the results for the Radiation therapy Oncology Group brain metastases trial?

Answer 38

Class 1: KPS > or = 70, < 65 years old, controlled primary with no extra cranial metastases— median survival 7.1 months.

Class 3: KPS < 70—median survival 2.3 months.

Class 2: all others—medial survival 4.2 months

Question 39

What is the difference in possible meningitis complications in dermoids vs epidermoids?

Answer 39

Epidermoid: aseptic Mollaret recurrent meningitis

Dermoid: septic meningitis

Question 40

What is the most common location for chrodomas?

Answer 40

Sacrum (50%) and clival (35%)

Question 41

What type of radiation is most effective for chordoma?

Answer 41

proton beam