030415 brain tumors

Question 1

types of primary brain tumors

Answer 1

meningiomas

gliomas: astrocytomas, glioblastoma, ependymoma, oligodendroglioma, embryonal tumors
others: pituitary, nerve sheath tumors, lymphoma, craniopharyngioma, germ cell tumors, etc

Question 2

morbidity and mortality in low and high grade neoplasms in CNS

Answer 2

both low and high have significant morbidity and mortality (diffusely infiltrative, involvement of critical anatomic areas, inability to resect critical anatomic areas)

Question 3

astrocytomas

Answer 3

most common glial tumor

diffuse astrocytomas (grade 2) have tendency to progress to higher grades (anaplastic astrocytoma, glioblastoma) over time

80% are glioblastomas

Question 4

clinical features of astrocytomas

Answer 4

seizures
focal neurologic deficits (GRADUAL onset)
headaches

Question 5

median survival for glioblastoma multiforme

Answer 5

1 yr

Question 6

WHO grading scheme for astrocytomas

Answer 6

1: pilocytic astrocytoma (no tendency to become higher grade)
2: astrocytoma (diffuse)–CELLULARITY is moderately increased and occasional NUCLEAR ATYPIA
3: anaplastic astrocytoma–increased cellularity, distinct nuclear atypia, marked MITOTIC ACTIVITY
4: glioblastoma multiforme–pleomorphic astrocytic cells, brisk mitotic activity, prominent MICROVASCULAR PROLIFERATION AND NECROSIS

Question 7

grade 2 astrocytoma-gross morphoogy

Answer 7

flattened gyri, ill-defined

Question 8

necrosis w pseudopalisading

Answer 8

distinct for grade 4 astrocytoma

Question 9

pilocytic astrocytoma occurs where

Answer 9

most commonly cerebellum

Question 10

imaging of pilocytic astrocytoma

Answer 10

well demarcated

cystic tumor w/ enhancing mural nodule

Question 11

histology of pilocytic astrocytoma

Answer 11

densely fibrillary (pilocytic/hair like) areas alternating w microcystic component

Rosenthal fibers (eosinophilic astrocyte processes)

Question 12

oligodendroglioma occurs in whom

Answer 12

adults

Question 13

imaging of oligodendroglioma

Answer 13

well defined hypodense mass, may see CALCIFICATION

Question 14

prognosis for oligodendroglioma

Answer 14

5-10 yrs for grade II

better than for astrocytomas

Question 15

allelic loss of chromosome 1p and 19q are predictors of prolonged survival and susceptiblity to chemo

Answer 15

anaplastic oligodendrogliomas

Question 16

histology of oligodendroglioma

Answer 16

calcifications

round nuclei, “FRIED EGG” cells

Question 17

ependymoma occurs in whom

Answer 17

usually children

Question 18

prognosis for ependymoma

Answer 18

4 yrs

Question 19

histology of ependymoma

Answer 19

true rosettes (mini ventricle)
perivascular pseudorosettes

Question 20

choroid plexus papilloma occurs in whom

Answer 20

kids

Question 21

where does choroid plexus papilloma occur

Answer 21

4th ventricle, lateral ventricle, 3rd ventricle, cerebellopontine angle

Question 22

prognosis for choroid plexus papilloma

Answer 22

very good w surgical resection if papilloma

if carcinoma, poor prognosis

Question 23

colloid cyst

Answer 23

benign
usually attached to roof of 3rd ventricle
can obstruct foramen of Munro intermittently
positional headache

Question 24

ganglioglioma occurs in whom

Answer 24

young (under 30)

Question 25

where does ganglioma occur

Answer 25

typically supratentorial and in temporal lobe

Question 26

how is ganglioglioma like or unlike pilocytic astrocytoma

Answer 26

it also has cyst w mural nodule, but it's in cerebrum

Question 27

medulloblastoma

Answer 27

embryonal neuroepithelial neoplasm of POSTERIOR FOSSA, of primitive neuroectoderm

Question 28

characteristic feature of medulloblastoma

Answer 28

tendency to spread through CSF pathways

Question 29

tx for medulloblastoma

Answer 29

surgical resection followed by radiation (usually cranio-spinal)

Question 30

histology for medulloblastoma

Answer 30

small blue cells, highly cellular | Homer Wright rosettes

Question 31

primary CNS lymphoma

Answer 31

occur btwn 40-60 poor prognosis-most die within a year periventricular lesions

Question 32

meningioma

Answer 32

extra parenchymal tumor of CNS | women more than men

Question 33

imaging of meningioma

Answer 33

DURAL BASED | WELL DEFINED

Question 34

meningioma-associations

Answer 34

progesterone receptors | NF2, radiotherapy

Question 35

outlook for meningioma

Answer 35

if grade I, excellent 5 yr survival

Question 36

histology for meningioma

Answer 36

sheets of tumor cells w indistinct borders whorls Psammoma bodies

Question 37

where are schwannomas located usually

Answer 37

peripheral nerves in head and neck and flexor surfaces or extremities schwannomas-intracranial tumors most often in cerebellopontine angle and attached to 8th nerve (symptoms of hearing loss, tinnitis, facial numbness)

Question 38

neurofibroma

Answer 38

benign tumor composed of Schwann cells, fibroblasts, perineural cells assoc w NF1 cutaneous form and peripheral nerve form

Question 39

familial tumor syndromes assoc w increased risk of nervous system tumors-four types

Answer 39

NF1, NF2, Von Hippel LIndau dis, tuberous sclerosis each has cutneaous or eye abnormalities most linked to loss of tumor suppressor genes

Question 40

NF1

Answer 40

much more common than NF2 neurofibromas, cafe au lait spots, Lisch nodules, optic glioma, oseeous lesions, axillary freckling, family hx associated tumors: MOST IMPORTANT-NEUROFIBROMAS THAT UNDERGO TRANSFORMATION TO MALIGNANT PERIPHERAL NERVE SHEATH TUMORS

Question 41

NF2

Answer 41

criteria include: BILATERAL VESTIBULAR SCHWANNOMAS (most common manifestation), first degree relative w NF2, lens opacity, cerebral calcifications other associations: meningiomas, schwannomas, gliomas, neurofibromas

Question 42

Von Hippel Lindau dis

Answer 42

hemangioblastomas of CNS and retina (CEREBELLAR HEMANGIOBLASTOMAS) renal cell carcinoma, pheo, visceral cysts, retinal angiomas

Question 43

tuberous sclerosis

Answer 43

clinical: SEIZURES, autism, cognitive dysfxn CORTICAL HAMARTOMAS (TUBERS-neuronal and glial properties), subependymal giant cell astrocytomas, etc

Question 44

paraneoplastic syndrome

Answer 44

clinical syndrome produced by remote effect of systemic malignancy hypothesized that some visceral cancers express certain neural antigens. immune system recognizes these antigens as foreign and attacks own CNS/PNS

Question 45

Lambert Eaton myasthenic syndrome

Answer 45

paraneoplastic neurologic syndrome muscle weakness esp in legs that improves w testing on exam

Question 46

metastatic tumor in brain--what to tx w?

Answer 46

radiation therapy