ICL 10.13: CNS Tumors Flashcards

Question

what are the microscopic changes in the brain with a low grade astrocytoma?

Answer 1

too many astrocytes and too much pleomorphism (lots of variation in how the cells appear)

Answer 2

WHO grade III malignant astrocytoma it's intermediate grade with intermediate prognosis Anaplastic astrocytomas generally receive post-operative therapy, unlike low grade astrocytoma pathology shows greater cellularity and nuclear pleomorphism than a low grade astrocytoma plus mitotic activity

Answer 3

WHO grade IV malignant astrocytoma = highest grade key features: 1. tumor necrosis due to the tumor outgrowing its blood supply 2. proliferating balls of small blood vessels responding to VEGF secreted by ischemic tumor cells

Answer 4

1. dedifferentiation of lower grade astrocytoma into a glioblastoma (more common in younger patients) 2. de novo (more common in older patients) these two tumors are different molecularly

Answer 5

1. greater cellularity of astrocytes 2. tons of nuclear pleomorphism 3. mitotic activity (cells look like they're trying to divide; reflects growth of tumor) 4. tumor necrosis = no blue staining nuclei; the tumor looks white because the tumor cells are dying due to tumor outgrowing its blood supply -- you see a ton of cells around this ischemic area and it's cells trying to run away from the ischemia = pseudopalisading 5. microvascular proliferation = proliferating balls of small blood vessels from VEGF secreted by ischemic tumor cells glioblastoma is a bunch of different colors and it doesn't all look like a uniform tumor, it looks horrible slide 19

Answer 6

malignant brain tumors vs. metastatic brain tumors love to infiltrate in the brain, particularly white matter tracts the corpus collosum is the major white matter tract in the brain and what a glioblastoma does is crawl across to the other hemisphere = "butterfly tumor"(basically diagnostic of a glioblastoma)

Answer 7

1. pilocytic astrocytoma 2. pleomorphic xanthoastrocytoma 3. subependymal giant cell astrocytoma of tuberous sclerosis

Answer 8

WHO grade I astrocytomas that is benign usually seen in children and usually in the cerebellum in the midline -- can also occur in the hypothalamus, optic nerve and brainstem also usually has a cyst! the cyst is what usually causes the problems too because it outgrows the tumor so a midline, cerebellar mass with a cyst in a kid, it's basically 100% a pilocytic astrocytoma

Answer 9

1. alternating biphasic growth pattern of fibrillar and loose areas 2. Rosenthal fibers

Answer 10

WHO grade I astrocytomas that is benign superficial, circumscribed, cerebral tumor of children and young adults *striking tumor cell pleomorphism with giant cells may falsely suggest a high grade tumor (slide 27) usually also has a cyst that is the cause of the problems because it gets so big

Answer 11

WHO grade I astrocytomas that is benign it's only seen in patients with tuberous sclerosis

Answer 12

tumor involving ependymal cells which is seen in both children and adults slow growing, malignant and clinical features depend on the location they tend to grow in the ventricles (duh, the ependymal cells line ventricles) and a consequence is that pieces of the tumor can break off and float through the CSF = seeding of the subarachnoid space in aggressive tumors poor prognosis; average survival is 4 years

Answer 13

1. kids and young adults: primarily in the 4th ventricle --> close proximity to the pons and medulla makes a complete resection really difficult 2. adults: primarily an intraspinal glioma --> easier to ressect

Answer 14

cells are arranged around vessels with thin ependymal processes directed towards vessels --> they create a ribbon appearance the tumor cells have long thin tails which go down and attach to the blood vessels so that's why you get this clear pink halo of tails surrounding the blood vessels with no blue nuclei slide 31

Answer 15

1. tiny lumens filled with microvilli 2. long serpentine cell junctions now it looks like an adenocarcinoma as expected! slide 32

Answer 16

slow growing tumors of oligodendrocytes that comprise 5-15% of gliomas they have a predilection for white matter (duh that's where all the oligodendrocytes are) tumors occur in the 4th-5th decade of life and the prognosis is relatively good (:

Answer 17

1. fried egg appearance = blue tumor cell surrounded by a halo of white 2. delicate branching vessels = "chicken wire" 3. microcalcifications

Answer 18

neurons are mature post-mitotic cells and after embryogenesis they usually don't divide so you wouldn't expect them to make tumors so usually if you have a tumor made of neurons it's probably something that's been sitting there since embryogenesis so it's more of a malformation than a tumor usually benign

Answer 19

neuronal tumors: 1. ganglion cell tumor 2. central neurocytoma 3. dysembryoplastic neuroepithelial tumor

Answer 20

a type of neuronal tumor that is general benign -- usually only found when someone has a seizure large, mature neurons may be the only feature = gangliocytoma or may occur with a glial component = ganglioglioma slide 39

Answer 21

a type of neuronal tumor that is general benign it can be a calcified intra or periventricular mass it's composed of uniform small neurons that aren't growing this mass is usually in the middle of the brain around the area of the germinal matrix so they're basically left over cells in the germinal matrix that never made it to the cortex slide 40

Answer 22

a type of neuronal tumor that is general benign and seen in kids patients will present with seizures

Answer 23

multiple cortical nodules show diverse histological patterns a key pattern is normal neurons that “float” in a mucinous matrix between vessels lined by small round cells (“specific glioneuronal component”) when you see a floating neuron, you hit de net, get it? DNET? slide 42

Answer 24

1. choroid plexus papilloma | 2. colloid cyst of the 3rd ventricle

Answer 25

a ventricular tumor they're usually just masses that block CSF and then the surgeon will go in and take it out can sometimes rarely cause hydrocephalus in children due to a choiroid plexus papilloma that is overproducing CSF

Answer 26

a ventricular tumor that has a "ball valve" effect with intermittent hydrocephalus patients will have intermittent headache, loss of consciousness, etc. --> can cause sudden death slide 46

Answer 27

all of these are tumors of childhood!! they are all known as primitive neuroectodermal tumors = PNETs 1. medulloblastoma (in the cerebellum) 2. neuroblastoma (in the cerebral hemispheres) 3. pineoblastoma 4. retinoblastoma

Answer 28

a type of poorly differentiated tumor that is the most common malignant brain tumor of children (pilocytic tumor is benign) it's a solid tumor arising from the cerebellar vermis -- primitive cells with *high nuclear to cytoplasmic ratios highly malignant, but sensitive to radiation and chemotherapy: 5-year survival is ~75%

Answer 29

it disseminates through the CSF pathways and metastases to other parts of the CNS (never outside the CNS) they often go to the bottom of the spinal cord to the cauda equina due to gravity = "drop metastases"

Answer 30

a disease of the elderly and immunocompromised --> it's the most common CNS neoplasm in immunosuppressed individuals including AIDS tumors are mostly made of B-cell, large cells lymphomas have a preference for periventricular areas --> tumors collect in perivascular spaces --> damage to vessel walls leads to "hooping" seen in reticulin stain slide 51

Answer 31

benign tumors derived from arachnoid cells common locations = convexity, skull base, spinal cord nerve roots

Answer 32

meningiomas do not invade the brain!!! it's outside the brain because it's made from the meninges!! it just looks like a ball inserted into the middle of the brain (slide 53) however, they may invade the overlying skull --> but invasion of bone does not make it malignant!!! it's only malignant if it invades the brain which isn't common

Answer 33

Most meningiomas are “transitional” types with whorls of spindled cells wrapped around more polygonal cells whorls are characteristic! slide 55

Answer 34

a benign, highly vascular tumor of adults -- most common in the cerebellum often cystic but cell of origin is unknown (tumors that make cysts are usually benign) may produce erythropoietin, causing erythrocytosis = increased Hct levels multiple hemangioblastomas are seen in von Hippel-Lindau syndrome

Answer 35

gross anatomy shows a red tumor in the cerebellum from all the blood vessels microscopically, the tumor cells have foamy cytoplasm, and fill the spaces between small blood vessels slide 57

Answer 36

1. lung 2. breast 3. skin 4. kidney 5. GI these are also the most common tumors overall

Answer 37

the gray-white junction in territory of MCA it's because these things arise as tumor emboli --> anything arriving from the blood comes through the cortex to the white matter; the white matter needs less oxygen so the blood vessels there get smaller so anything coming to the brain will get stuck at this grey-white matter junction where the blood vessels get smaller

Answer 38

mets are generally sharply demarcated (except melanoma because they come from the neural crest) and multiple, with adjacent edema even on histology, you can see a straight line between the met and the brain tissue! they don't invade because they aren't in an environment that they like (except melanoma, that invades)

Answer 39

if you have a tumor in the body that has some antigens that resemble antigens of the brain then when the body makes antibodies against them, your own antibodies will attack your brain too signs and symptoms of paraneoplastic syndromes of the nervous system often begin even before a cancer is diagnosed! they can involve the central or peripheral nervous system

Answer 40

small cell carcinoma of the lung

Answer 41

1. Lambert-Eaton myasthenic syndrome 2. Stiff-man syndrome 3. Retinal degeneration 4. Limbic and brain stem encephalitis 5. Subacute cerebellar degeneration 6. Subacute sensory neuropathy

Answer 42

1. meningioma (because meninges go all the way down the spinal cord) 2. schwannoma CN 8, spinal nerve roots, peripheral nerves 3. neurofibroma spinal roots (rare), peripheral nerves

Answer 43

CN 8 this is an acoustic neuroma!!! it's Schwann cells growing on the auditory nerve!!

Answer 44

these are both benign tumors of peripheral nerves SCHWANNOMA 1. made of schwann cells 2. adjacent to nerve 3. encapsulated 4. easily resettable without nerve damage 5. anybody can get a schwannoma NEUROFIBROMA 1. made of intramural fibroblasts 2. infiltrates nerve 3. not encapsulated 4. not resectable without sacrificing nerve 5. almost nobody gets neurofibromas unless they have neurofibromatosis

Answer 45

palisading of tumor cells in the Antoni A areas can be striking -- looks like jelly fish tentacles there are dense, spindly Antoni type A areas and looser Antoni type B areas; there are no nerve fibers within the tumor slide 66

Answer 46

The involved nerve shows normal nerve fibers (dense, pink) separated by infiltrating tumor (lighter areas) looks like streaks of pink in-between lighter pink slide 67

Answer 47

meningioma think meningiomas wrap around the brain

Answer 48

oligodendroglioma

Answer 49

glioblastoma

Answer 50

schwannoma

Answer 51

ependymoma

Answer 52

1. neurofibromatosis = von Recklinghausen disease 2. tuberous sclerosis 3. spurge-weber disease 4. von hippel-lindae disease these people have mutations that causes them to have multiple brain or peripheral tumors

Answer 53

aka von Recklinghausen disease it's a familial tumor syndrome: a genetic disorder characterized by the development of multiple (benign) tumors of nerves and skin (neurofibromas) and areas of abnormal skin color it's dominant inheritance there is neurofibromatosis type 1 and 2 --> type 1 has lesions all over the body on peripheral nerves while type 2 has tumors in the head

Answer 54

NF1: 1. multiple peripheral nerve neurofibromas like nodules literally all over the body; very disfiguring and hopeless to try and remove them unless one of them is growing because then you worry it's malignant 2. pigmented nodules (Lisch) in iris 3. cafe-au-lait spots (melanosis) in skin 4. lephantiasis: increased connective tissue 5. increased incidence of malignant tumors

Answer 55

NF2: *bilateral* schwannomas of 8th cranial nerve increased incidence of meningiomas and gliomas

Answer 56

it's a familial tumor syndrome with dominant inheritance that causes congenital cognitive disability there are benign neural-glial hamartomas in the brain and eye --> they can be in the gyri or down in the subependymal cells of the ventricles or they can be retinal it kind of appears like acne on the face, near the nails, etc. = facial angiofibromas

Answer 57

1. giant, dimpled gyrus that is hardened | 2. cortex is white white the grey matter is white; it's reverse!

Answer 58

it's a familial tumor syndrome with dominant inheritance it causes multiple hemangioblastomas in the cerebellum, retina, and spinal cord there's also erythrocytosis due to erythropoietin production so it's a hereditary condition associated with tumors arising in multiple organs --include hemangioblastomas, which are blood vessel tumors of the brain, spinal cord, and eye. The eye tumors are also called retinal angiomas

Answer 59

1. cysts in pancreas and kidneys 2. renal cell carcinoma --> this is why you get increased EPO production! 3. pheochromocytoma of adrenal