Traditional Brain Tumors Flashcards
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
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
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
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
Astrocytoma
Clinical S/S
Seizures
Focal neurologic deficits – gradual (not abrupt) onset
Headaches
Astrocytoma
Prognosis
Astrocytoma Grading
Grade 1 - Pilocytic astrocytoma
Grade 2 - Astrocytoma (diffuse)
Grade 3 - Anaplastic astrocytoma
Grade 4 - Glioblastoma multiforme
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)
What is this?
Describe the microscopic pathology.
Pilocystic Astrocytoma
Biphasic pattern: Densely fibrillary (pilocytic) areas alternating with microcystic component
Rosenthal fibers (“hair-like”)
What is the pathology of diffuse astrocytoma?
Cellularity is moderately increased and occasional nuclear atypia
What is the pathology of anaplastic astrocytoma?
Increased cellularity, distinct nuclear atypia, marked mitotic activity
What is this?
What is the microscopic pathology?
Plemorphic astrocytic cells, brisk mitotic activity
Prominent microvascular proliferation and/or necrosis
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
What is this?
Grade II Oligodendroglioma
What is the pathologic characteristics of anaplastic oligodendroglioma (Grade III)?
Necrosis
Vascular Proliferation
Mitosis
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
What is this?
What is its behavior?
What is its microscopic pathology?
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
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
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
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
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
What is this?
What is the histology?
Ganglioglioma
Atypical ganglion cells and neoplastic glial component
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%
What is this?
How does it appear on microscopy?
Medulloblastoma
Microscopy
HIghly cellular and composed of undifferentiated cells
Homer Wright Rosettes
Synaptophysin immunoreactivity
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
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
What is this?
What is the microscopy associated with it?
Lymphoma
Perivascular arrangement of neoplastic cells
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
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)
What is this?
What is the microscopy of it look like?
Meningioma
Pathology: Whorls, psammomma, bone infiltration
Atypical/anapalastic features: Necrosis, mitoses, brain invasion
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
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
