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Flashcards in Traditional Brain Tumors Deck (32):
1

Epidemiology
Intracranial (Incidence)
Intraspinal (Incidence)
Children (Relative Prevalence)

Intracranial Brain Tumors: 10-17/100K  persons/year
Intraspinal Tumors: 1-2/100K persons/year
Children: 20% of all tumors are brain

2

Location

Childhood
Adult
Metastatic vs Primary

Childhood – CNS tumors most often (75%) in posterior fossa

Adult – CNS tumors are most often supratentorial
Localization of adult CNS tumors follows a mass distribution
Cerebral hemispheres, most frequently frontal lobes as they are biggest

Metastatic more common than primary brain tumors

3

Behavior of CNS tumors

Significant morbidity and mortality
Diffusely infiltrative
Involvement of critical anatomic areas
Inability to resect critical anatomic areas

Most of time do not spread outside of brain (extremely uncommon – metastases)
May spread through subarachnoid

4

Astrocytoma

Relative incidence
Behavior
Epidemiology
What percent are glioblastomas?

Most common glial tumor
Diffuse astrocytomas have an inherent tendency to progress to higher grades (anaplastic astrocytoma, glioblastoma) over time

Annual incidence ~3-4 per 100,000

At least 80% are glioblastomas

5

Astrocytoma

Clinical S/S

Seizures
Focal neurologic deficits – gradual (not abrupt) onset
Headaches

6

Astrocytoma

Prognosis

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7

Astrocytoma Grading

Grade 1 - Pilocytic astrocytoma
Grade 2 - Astrocytoma (diffuse)
Grade 3 - Anaplastic astrocytoma
Grade 4 - Glioblastoma multiforme

8

Pilocytic astrocytoma

Epidemiology
Clinical Features
Imaging
Prognosis

Most common glioma in children – 1st Two Decades

Clinical Features
Commonly occur in cerebellum
Optic nerve, 3rd ventricle, hypothalamus, brainstem, occasionally cerebral hemispheres
S/S: Presentation with focal neurologic deficit, seizures, increased ICP

Imaging
Well demarcated, often cystic contrast-enhancing tumor

Slow growing, overally excellent prognosis (surgery is often curative)
 

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9

What is this?
Describe the microscopic pathology.

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Pilocystic Astrocytoma

Biphasic pattern: Densely fibrillary (pilocytic) areas alternating with microcystic component

Rosenthal fibers (“hair-like”)

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10

What is the pathology of diffuse astrocytoma?

Cellularity is moderately increased and occasional nuclear atypia

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11

What is the pathology of anaplastic astrocytoma?

Increased cellularity, distinct nuclear atypia, marked mitotic activity

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12

What is this?

What is the microscopic pathology?

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Plemorphic astrocytic cells, brisk mitotic activity
Prominent microvascular proliferation and/or necrosis

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13

Oligodendroglioma

Epidemiology
Clinical S/S
Prognosis

Epidemiology
Adults in 5th to 6th decade

Clinical S/S
Long history of progressive neurological symptoms (seizures, headache focal signs)
Imaging – Well defined hypodense/hypointense mass, may see calcification

Px
Median survival: 5-10 years for grade II
Better survival than with astrocytomas

14

What is this?

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Grade II Oligodendroglioma

15

What is the pathologic characteristics of anaplastic oligodendroglioma (Grade III)?

Necrosis
Vascular Proliferation
Mitosis

16

Ependymoma

Epidemiology
Location
Clinical S/S
Imaging
Grading
Prognosis

Typically occurs in children and young adults

Occurs along ventricular system, usually posterior fossa (4th ventricles)

Clinical S/S – Hydrocephalus, occasionally seizures

Imaging – Well-circumscribed mass

Two WHO grades
Ependymoma (WHO II)
Anaplastic endymoma (WHO III)

Prognosis – Average survival for posterior fossa tumors is 4 years

17

What is this?

What is its behavior?
What is its microscopic pathology?

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Ependymoma

May invade brain parenchyma
Subarachnoid space dissemination

True rosettes (columnar, shown below), arranged around a central lumen
Aplastic - Highly cellular tumors with increased mitoses and proliferation

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18

Glioma

Etiology

No cause identified in most common types

Familial tumor syndromes (rare: <5%)
Li-Fraumeni syndrome: Predominantly astrocytomas
Turcot syndrome: Glioblastoma or medulloblastoma
Cowden syndrome: Cerebellar gangliocytoma
NF1, NF2, Tuberous sclerosis, von Hippel-Lindau

Only  environmental factor definitevely associated with increased risk of brain tumors is therapeutic radiation
Example: Children who underwent prophylactic CNS irradiation for acute lymphocytic leukemia have increased risk of development of brain tumors

19

Choroid plexus papilloma

Epidemiology
Location
Presentation
Carcinoma epidemiology
Prognosis
Pathology

Typically found in first two decades

Occurs in 4th ventricle, lateral ventricle, 3rd ventricle and cerebellopontine angle

Presentation with hydrocephalus
Overproduction of CSF
Obstruction of CSF flow

Carcinoma

Occurs in children < 10 years
Rare in adults

Prognosis
Choroid plexus papilloma – Very good with surgical resection
Chroid plexus carcinoma – Poor prognosis

Pathology
Well-demarcated, pedunculated, or cauliflower-like mass

20

Colloid Cyst

Location
Pathophysiology
Presetnation
Histology

Usually attached to roof of 3rd ventricle
Intermittent obstruction of foramen of Monro
Positional headache
Thin-walled cyst lined by cuboid/columnar epithelium

21

Ganglioglioma

Age
Location
Previous History
Treatment

Usually in the first three decades
Typically supratentorial and in temporal lobe
Long standing history of seizures is common
Surgical resection is usually curative - No radiation or chemotherapy needed

22

What is this?
What is the histology?

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Ganglioglioma
Atypical ganglion cells and neoplastic glial component

23

Medulloblastoma

Definition
Clinical Features
Characteristic Feature
Treatment

Embryonal neuroepithelial neoplasm of posterior fossa
Known as “primitive neuroectodermal neoplasm”
Represents approx. 33% of posterior fossa tumors in children

Clinical features: Cerebellar dysfunction (ataxia) and increased ICP

Characteristic feature: Tendency to spread through CSF pathways

Surgical resection followed by radiation (usually cranio-spinal)
Some subgroups: With total excision and radiation, 5-year survival may be 75%

24

What is this?

How does it appear on microscopy?

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Medulloblastoma

Microscopy
HIghly cellular and composed of undifferentiated cells
Homer Wright Rosettes
Synaptophysin immunoreactivity

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25

Primary CNS Lymphoma

Relative rate
Target population
Associated Risks
Majority are what kind of lymphoma?

5-10% of primary intracranial neoplasms

Typically occur b/w 40-60 years
Immunocompetent host – 55 years (rare, but increasing incidence)
Immunocompromised – 40 years

10% of AIDS patient, usually late-stage
20% of post-transplant patients
EBV association

98% B-cell; 2% T-cell

26

CNS Lymphoma

Signs/Symptoms
Location
Prognosis and Treatment

S/S: Non specific, referable to mass lesions

Location
2/3 are supratentorial
Meningeal spread in 25%

Poor px: Most die within one year
Rx: Chemotherapy & radiation
Spread of disease outside of CNS is rare

27

What is this?

What is the microscopy associated with it?

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Lymphoma

Perivascular arrangement of neoplastic cells

28

Meningioma

Relative frequency
Epidemiology
S/S
Imaging
 

Most common extra-parenchymal neoplasm of CNS

Epidemiology
Occurs in middle to late adult life
F > M

S/S
May lead to increased ICP, focal signs, seizures

Imaging
Dural based, usually well-defined, contrast-enhancing

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29

Meningioma

Pathogenesis

Unknown
Significance of presence of progeterone receptors on cells & increased occurrence in women is not understood
Associations: Previous radiotherapy and NF2
Genetic abnl: Monosomy of chromosomes 22 or muation of NF2 gene (chr. 22)

30

What is this?

What is the microscopy of it look like?

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Meningioma

Pathology: Whorls, psammomma, bone infiltration
Atypical/anapalastic features: Necrosis, mitoses, brain invasion

31

Meningioma

Grading and behavior

Typical (WHO Grade I): > 90%

Excellent 5-years survival
20% recur within 10 years (when all was grossly resected)

Atypical (WHO Grade II): 5%

More mitoses, less differentiation, and sometimes brain invasion
5-year recurrence – 40%, Mortality – 20%

Anaplastic (WHO Grade III): 2%

Even more mitoses and even less differentiation
Median surival – 1.5 years

32

Metastatic tumors of the CNS

What percent of the time is this the first presentation of the malignancy?
Common origins?
Clinical signs/symptoms
Radiology
Prognosis

First presentation of malignancy – 50%
Origin: Lung or breast carcinoma, kidney, GI tract, skin
Clinical S/S: Headaches, focal neurologic signs, altered mental status
Radiographically – Distinct contrast-enhancing mass with surrounding edema, usually multiple
Prognosis – Poor, most die within a few months