CNS Tumors I Flashcards

1
Q

Grade II brain tumor is…

A

Benign

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2
Q

Grade III brain tumor is…

A

Anaplastic

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3
Q

Grade IV brain tumor is…

A

Very aggressive

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4
Q

Give the epidemiology of Pilocytic Astrocytoma

A

Most common CNS tumor in children (usually between the ages of 8-13) Grade I 15% of patients with Neurofibromatosis have this 33% of patients have Trisomy 7 and 8

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5
Q

Give the morphology of Pilocytic Astrocytoma

A

Well-circumscribed, homogenous, contrast enhancing, and cyst formation tends to occur Location is infratentorial, around the ventricles (mainly 3rd and 4th) and midline structures. Pathoma says it usually arises in the cerebellum. These cells are GFAP positive.

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6
Q

Histology of Pilocytic Astrocytoma

A

These cells look spindly and hair-like despite being astrocytic in nature Rosenthal fibers (google an image of this!)

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7
Q

Diffuse Astrocytoma Epidemiology

A

Peak age of incidence is 34 yrs old (30’s for astrocytomas, 40’s for glioma’s) Grade II Male: Female ratio: 1.18:1 60% have a p53 mutation ***Old test question p16/p15 mutations also seen

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8
Q

Diffuse Astrocytoma Morphology

A

On neuroimaging: ill-defined and diffuse, homogenous masses, cystic changes and calcifications Location: Cerebral hemispheres (front-temporal lobe), but they can occur anywhere there are astrocytes Gross: ill-defined, no necrosis or hemorrhage, frequently see cystic degenerations

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9
Q

Diffuse Astrocytoma Histology

A

Fibrillary backround (looks like a spider’s web!). Anytime you see this think Astrocytoma GFAP +

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10
Q

Gemistocytic Astrocytoma

A

Gemisto- “full or fat” One variety of the diffuse astrocytoma that is more aggressive Still has fibrillary backround and GFAP+

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11
Q

Anaplastic Glioma Epidemiology

A

Peak incidence: 40s Grade III This is the result of astrocytoma that recurs. Glioma is more aggressive than astrocytoma > 90%: p53 mutation 30% : p16 deletion 25%: Rb alterations 40%- loss of heterozygotic 19q

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12
Q

Anaplastic Glioma Morphology

A

Neuroimaging: Ill-defined mass that is not homogenous like Astros, RING OF ENHANCEMENT. Location: Cerebral hemispheres

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13
Q

Anaplastic Glioma Histology

A

Irregular cells but fibrillary backround is still there so they still mark positive with GFAP. Will see nuclear atypia, increased cellularity, and mitotic activity.

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14
Q

Differential Diagnosis for Ring Enhancing Lesions

A

1) Toxoplasmosis 2) Lymphomas 3) Glioblastomas/ anaplastic gliomas

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15
Q

What is the difference between primary and secondary Glioblastoma Multiforme (GBM)?

A

Primary: No specific p53 mutations. EGFR amplification and overexpression. “When it was born, it was aggressive” Secondary: Begins with p53 mutation. Progresses from low grade astrocytoma to anaplastic astrocytoma to glioblastoma.

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16
Q

Epidemiology of Glioblastoma Multiforme

A

Most aggressive glial brain tumor (1 year survival) Grade IV Peak age: 50s >90% p53 mutation

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17
Q

Glioblastoma Multiforme Morphology

A

Neuroimaging: Irregularly shaped lesions, ring-like enhancement, ill-defined, heterogenous Gross: Extensive necrosis and hemorrhages Location: cerebral hemispheres, contralateral invasion through Corpus Callosum (sometimes called the “Butterfly Glioma”)

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18
Q

Glioblastoma Multiforme Histology

A

Pseudo-palisading, multinucleated cells, mitotic activity, endothelial cell proliferation Still see fibrillary backround, so it will be GFAP +

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19
Q

3 Things Used to Diagnose Glioblastoma Multiforme

A

1) Pleomorphism 2) Necrosis…pseudo-palisading 3) Endothelial cell proliferation…endothelial cells become thick, glomerular-like structures

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20
Q

Pontine Glioma

A

Diffuse expansion of the pons, usually high grade astrocytoma or GBM Grade IV Tumors in the pons tend to be very aggressive Cells are multinucleated due to overzealous mitosis

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21
Q

Giant Cell Glioblastoma Epidemiology

A

Age: 40s Grade IV Location: Cerebral hemispheres 75-90%- p53 mutations 33%- PTEN mutations

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22
Q

Giant Cell Glioblastoma Histology

A

Giant multinucleated cells Pseudo-palisading necrosis Perivascular accumulation of tumor cells (“rosette-like pattern)

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23
Q

Gliosarcoma Epidemiology

A

Mixture of glioblastoma from brain parenchyma and a sarcoma stemming from the meninges Age of onset: 50s Grade IV p53 mutation, loss of chromosomes 10 and 17, Rb alterations

24
Q

Gliosarcoma Morphology

A

Location: Exclusively in the cerebral hemispheres Gross: Ring-enhancement

25
Q

Gliosarcoma Histology

A

Areas of glial differentiation Mesenchymal portion: vascular, cartilage, bone, smooth and striate muscle Contains two distinct areas: one part is mainly collagen while the other part is the glioblastoma part

26
Q

Gliomatosis Cerebri Epidemiology

A

Age: 40-50 yrs old Grade II Karyotype 44, XY

27
Q

Gliomatosis Cerebri Morphology

A

Eventually substitute the entire brain. This tumor does not form a mass. Neuroimaging: Type1…extensive infiltration of white and grey matter. Type 2: Like type I plus obvious neoplastic mass

28
Q

Gliomatosis Cerebri Histology

A

Mild astrocytic tumor with elongated glial cells, fibrillary backround with homogenous cells

29
Q

Oligodendroglioma Epidemiology

A

Age: 40 yrs Grade II 80%- deletions in 19q 67%- loss of heterozygosity in chromosome 1p

30
Q

Oligodendroglioma Morphology

A

Well-defined soft masses that are usually superficial located in the cortex Gross: reddish areas of hemorrhage

31
Q

Oligodendroglioma Histology

A

“Fried eggs” with clear halo Delicate vascular pattern…“chicken wire” Calcifications GalC marker positive

32
Q

Central Neurocytoma Epidemiology

A

Age: 20s Grade II Location: Arise from the septum Histology: Looks identical to oligodendrogliomas with fried egg appearance, but this is a tumor of neurons

33
Q

Medulloblastoma Epidemiology

A

Very common pediatric CNS tumor Peak incidence: 7-12 yrs old Grade IV 65% of pts are male Location: Infratentorial 50% have 17q mutations

34
Q

Medulloblastoma Morphology

A

3 Types: 1) Classic: Midline cerebellum, usually the vermis 2) Neuroblastic: Tumor of the vermis and of young children 3) Desmoplastic: Well circumscribed, solid masses. More often on the hemispheres of the cerebellum and a tumor of adolescence (age 12-20)

35
Q

Medulloblastoma Histology

A

Classic: Densely packed sheets of blue cells, undifferentiated. Markers: Synaptophysin and class 3 beta tubulin Neuroblastic: Rosettes…look like piles of coins Desmoplastic: Most differentiated. Pale islands

36
Q

Ependymoma Epidemiology

A

Age 5-10 Most common glioma in the spinal cord when found in adults Grade II Location: Most commonly in the 4th ventricle Alterations in 22q

37
Q

Ependymoma Histology

A

Pseudorosettes and Rosettes

38
Q

Choroid Plexus Tumors Epidemiology

A

Age: Younger than 20 Choroid Plexus Papilloma: Grade I Choroid Plexus Carcinoma: Grade III Associated with Li-Fraumeni Syndrome- mutation in TP53 codon 248

39
Q

Choroid Plexus Tumor Morphology

A

Neuroimaging: hyperdense tumors w/in the lateral ventricle, but can also occur in the 4th ventricle Hydrocephalus is the most common abnormality

40
Q

Choroid Plexus Tumor Histology

A

Papillary tumor: Positive for cytokeratin

41
Q

Craniopharyngioma Epidemiology

A

Most common non-neuroepithelial intracerebral tumor Derived from Rathke’s pouch epithelial cells Age: 5-14 yrs. Common in younger children. Grade I

42
Q

Craniopharyngioma Morphology

A

Solid masses w/ predominant cyst component

43
Q

Craniopharyngioma Histology

A

Nodules of compact keratin and calcifications

44
Q

Hemangioblastoma Epidemiology

A

Age: 20s Associated with von Hippel-Lindau (VHL) Exclusively in lateral cerebellar hemisphere Grade I

45
Q

Hemangioblastoma Morphology

A

Well-circumscribed nodules within the wall of larger cysts usually seen in the lateral cerebellar hemisphere Yellow (lipid rich), highly vascularized mass

46
Q

Hemangioblastoma Histology

A

Clear cells with a prominent vascular network Markers: NSE (neuronal specific enolase) and CD31

47
Q

CNS Lymphoma Epidemiology

A

Peak incidence: 60-70 yrs old. If you have AIDS, 39 years old.

48
Q

CNS Lymphoma Morphology

A

Single or multiple masses Ring of enhancement Location: Most common location is the basal ganglia Abnormalities in chromosomes 1, 6, 7, and 14

49
Q

CNS Lymphoma Histology

A

Angiocentric pattern “perivascular cuffing” of lymphocytes B-cell lymphomas (CD 20, CD 79a)

50
Q

Meningioma Epidemiology

A

Originate in the meninges Age: 60-70 yrs Grade I: most common 22q12 and NF2 gene mutations

51
Q

Meningioma Morphology

A

Well demarcated masses that originate in the dura. Has a broad dural attachment. Cerebral convexity associated with the falx cerebri

52
Q

Meningioma Histology

A

Psammoma bodies…pink, round concentric calcifications

53
Q

Schwannoma Epidemiology

A

Age: 40-60 yrs Involves CN VIII at the Pontocerebellar Angle If a patient has bilateral Schwannomas, they have Neurofibromatosis Grade I Tumor markers: S-100

54
Q

Schwannoma Histology

A

Spindle-shaped interdigitating cells, Nuclear palisading 2 types of areas: Antoni A…densely packed cells with Verocay bodies Antoni B…loosely packed cells, more myxoid

55
Q
A