Tumors

Question 1

• circumscribed
• no/rare mitotic figures, vascular proliferation, no focal necrosis

rosenthal fibers, microcysts

Answer 1

grade 1 astrocytoma=Pilocytic

Question 2

Inc cellularity and diffuse infiltration, inc nuclear atypia, incr mitotic activity

Answer 2

grade 3 astrocytoma=anaplastic

Question 3

moderately hypercellular, monotonous cells, mild nuclear atypia, no/minimal mitotic activity, no necrosis

Answer 3

grade 2 astrocytoma=diffuse

Question 4

vascular proliferation, necrosis,crowded anaplastic cells, marked nuclear atypia, brisk mitotic activity, butterfly pattern

Answer 4

grade 4 astrocytoma=glioblastoma

Question 5

what mutation is seen in pilocytic

Answer 5

braf serine/threonine kinase

Question 6

mutation seen in grade 2 and 3?

Answer 6

p53 loss of function and isocitrate dh genes 1 or 2

Question 7

differentiated resembling oligodendrocytes

poorly circumscribed, variable cellularity, egg appearance

Loss of 1p and 19q

little or no GFAP

Answer 7

oligodendroglioma

Question 8

dural based, well circumscribed, slow growing, whorles, psammoma bodies, grade 1

Answer 8

meningiomas

Question 9

slow growing
affects children: 4th ventricle->hydrocephalus
affects adults: spinal cord
rosettes/psuedorosettes
grade 1-3

Answer 9

ependymomas

Question 10

arise in cerebellum, sheet of densely packed small cells, most common grade 4 pediatric tumor

myc amp=poor outcome
wnt pathway mutation=good outcome
sonic hedgehog pathqay

homer-wright rosettes

Answer 10

medulloblastoma

Question 11

single or multiple intraparenchymal lesions, b-cell malignancies, a/w EBV, treated with chemo/radiation

poor prognosis

Answer 11

primary cns lymphoma

Question 12

mutation of neurofibromin on 17q
a/w neurofibromas
malignant peripheral nerve sheath tumors
a/w pilocytic astrocytomas in optic chiasm (optic glioma) and diffuse astrocytoma and glioblastoma

Answer 12

NF-1 or von recklinghausens dz

Question 13

mutation of merlin on 22q

vestibular schwannomas, ependymomas, meningiomas, diffuse astrocytoma

Answer 13

NF-2

Question 14

mutation of VHL gene on 3p

cerebellar hemangioblastoma, renal cell carcinoma, pheochromocytoma

Answer 14

von hippel-lindau dz

Question 15

mutation of hamartin on 9q and tuberin on 16p

-subependymal giant cell astrocytoma, renal angiomyolipoma, cardiac rhabdomyoma, skin changes

cortical hamartomas, supependymal nodules

Answer 15

tuberous sclerosis