Brain Tumors Flashcards
What are the three subtypes of gliomas?
The three types of gliomas are astrocytomas, oligodendroglioma, and ependymomas they are the most common group of primary brain tumor
Astrocytomas: Low-grade astrocytomas are usually associated with mutations in _______ and an over expression of ______. High grade astrocytomas- are a disruption of two _____ ________. what are they?
Astrocytomas: Low-grade astrocytomas are usually associated with mutations in p53 and an over expression of PDGF-A and its receptor. High grade astrocytomas- are a disruption of two Tumor Supressor genes RB and p16/CDKN2A.
Astrocytomas: Secondary astrocytomas are associated with a mutation in ______. Primary Astrocytomas are associated with an ________. Primary glioblastomas are associated with activation of ____ and ____ and inactivation of ______.
Astrocytomas: Secondary astrocytomas are associated with a mutation in p53. (because they usually progress from a low grade tumor which is associated with the mutation in p53) Primary Astrocytomas are associated with an amplification of MDM2. Primary glioblastomas are associated with activation of RAS and PIP-3 kinase and inactivation of p53 and RB.
Glioblastoma Grade: what are the two pathways?
Glioblastoma: Grade IV (infiltrating astrocytoma) primary- usually in older patients Secondary- younger patients with a progression from a a lower grade tumor
Diffuse astrocytoma grade: morphology: microscopically:
Diffuse astrocytoma grade: Grde II infiltrating astrocytoma morphology: poorly defined, gray infiltrative tumor microscopically: increased glial cells, nuclear pleomorphism, GFAP + astrocytic processes, indistinct border between normal cells and tumor cells
What type of tumor is this?
What are the defining fetures?
Grade?
where do they usually occur?
what age group?
What are typical clinical presentations?
Diffuse Astrocytomoa
Grade II infiltrating astrocytoma
poorly defined, grey, infiltrative tumor
usually located in hte cerebral hemisphere
Usually occurs in adults
infiltrating astrocytomas usually present as headache and seizures with focal neurologic deficits
What type of tumor is this?
What are the defining fetures?
Grade?
where do they usually occur?
what age group?
What are typical clinical presentations?
Diffuse Astrocytomoa
Grade II infiltrating astrocytoma
poorly defined, grey, infiltrative tumor
usually located in hte cerebral hemisphere
Usually occurs in adults
infiltrating astrocytomas usually present as headache and seizures with focal neurologic deficits
Gemistiocytic Astrocytoma
Grade:
Histology:
Gemistiocytic Astrocytoma
Grade: WHO grade II
Histology: predominant neoplastic astrocyte shows bright eosinophilic asteroid cell bodies
Anaplastic astrocytoma
Grade:
Anaplastic astrocytoma
Grade: III
Greater cellularity and more mitotic figures than diffuse astrocytoma (one step before glioblastoma)
What type of tumor is this?
Grade:
Morphology:
Histology
Characteristics of this type of tumor:
Glioblastoma (multiforme, GBM)
Grade: IV
Morphology: variable gross appearance
Histology: Similar to anaplastic astrocytoma with necrosis and vascular/endothelial cell proliferation.
*Tumor cells collect along areas of necrosis, AKA pseudopalisading
Characteristics of this type of tumor: AMEN
nuclear Atypia, Mitosis, Endothelial cell proliferation, Necrosis
Gliomatosis cerebri
Grade:
Location:
Gliomatosis cerebri
Grade: III/IV because of its aggressive potential
Location: diffuse glioma with infiltration within multiple areas of the brain, sometimes spreading to the entire brain
Astrocytoma Presentation:
Factors affecting presentation:
What is the prognosis, mean survival rate, growth rate, and mutation (if applicable) for:
Low-grade:
High-Grade:
Astrocytoma Presentation:
Factors affecting presentation: Location and Tumor Growth Rate
Low-grade: the tumor remains somewhat stable or slowly progresses, pt. have a mean survial >5 years, they typically evolove to a higher grade tumor.
High-Grade: typically enhance on imagining studies, poor prognosis (especially glioblastomas) mean survival is 15 months, IDH1 mutation in glioblastoma is associated with a better outcome
What type of tumor is this?
Grade:
Age:
location:
mutation:
gross appearance:
Histology:
Treatment:
Pilocytic astrocytoma
Grade: I
Age: children and young adults
location: cerebellum
mutation: BRAF is common
gross appearance: cystic, solid and well-circumscribed tumor. limited infiltration in surrounding brain
Histology: bipolar cells with long, thin, “hairlike” processes. The processes are GFAP positive. Will have Rosenthal fibers andd eosinophilic granular bodies
Treatment: resection alone
Pleomorphic Xanthoastrocytoma
Grade:
location:
age:
associated symptoms:
histology:
prognosis and survival rate:
Pleomorphic Xanthoastrocytoma
Grade: II
location: temporal lobe
age: children and young adults
associated symptoms: history of seizures
histology: neoplastic astrocytes which are filled with lipid
prognosis and survival rate: 80% 5-year survival rate because there is NO necrosis or mitotic activity
What type of tumor is this?
grade:
age:
location:
mutations:
morphology:
histology:
prognosis/survival:
Treatment:
Oligodendroglioma
grade: II
age: 4th and 5th decades
location: cerebral hemisphere
mutations: IDH1 and IDH2 are common mutations and portend a faborable diagnosis
morphology: well-circumscribed gelatinous gray masses often with cysts, focal hemorrhage and calcification
histology: sheets of regular cells with round nuclei, finely granular chromatin with surrounding clear halo
prognosis/survival: more favorable than astrocytomas 5-10 years but progression to higher stages (anaplastic oligodendroglioma) usually occurs
Treatment: chemo and radiation
Three other types of tumors that may be included in your differential diagnosis for ependyomomas:
Supependyomomas
Choroid plexux papilloma
Colloid cyst of 3rd ventricle
What type of tumor is this?
Grade:
Location:
Mutation:
Morphology:
Histology
Clinical presentation:
Factors affecting prognosis:
Ependymoma
Grade: II
Location: tumors arise next to the ependymal-lined ventricular system including the spinal cord. Well demarcated but difficult to remove
- in younger patients, occurrence is typically the 4th ventricle
- in older patients its usually the spinal cord
Mutation: NF2 patients
Morphology: well demarcatd from adjacent brain
Histology: Perivascular pseudo rosettes (tumor cells around vessels). regular nuclei and abundant granular chromatin
Clinical presentation: hydrocephalous secondary to obstruction of 4th ventricle.
Clinical presentation: CSF dissemination= poor prognosis (not uncommon)
Neuronal Tumors:
definition
age:
typical presentation:
types:
Neuronal Tumors:
definition: exhibit neuronal differentiation (far less common than glial tumors)
age: younger adults
typical presentation: seizures
Types: gangliomas, dysembryonic neuroepithelial tumoros
Central Neurocytoma
What is the most common neuronal tumor?
location:
growth rate:
treatment:
The most common neuronal tumor is a Gangliolioma
location: temporal lobe
growth rate: slow
treatment: surgical resection is often curative
What type of tumor is this?
Grade:
Age:
Location:
Growth rate:
Histology:
Presentation:
Treatment:
Medulloblastoma
Grade: IV
Age: Children
Location: cerebellum
Growth rate: rapid growth may occur blocking CSF flow
Histology: densely cellular with sheets of anaplastic cells, numerous mitoses
Presentation: hydrocephalus
Treatment: radiation and surgical resection. 5 year survival is 75%. Exquisitely radiosensitive
Atypical Teratoid/Rhabdoid Tumor
Grade:
Age:
Location:
Histology:
Mutation:
Prognosis:
Atypical Teratoid/Rhabdoid Tumor
Grade: IV
Age: young children <5
Location: surface of the brain
Histology: cells stain for muscle markers.
Mutation: chromosome 22 (hSNF5/INI1)
Prognosis: patients usually live less than 1 year
Primary CNS lymphoma
Common in ______ patients. When occurring in these types of patients they are almost always infected with____?
What type of lymphoma is it?
what do we stain for on an IHS?
Primary CNS lymphoma
Common in immunocompromised patients. When occurring in these types of patients they are almost always infected with EBV.
the vast majority are B-cell lymphomas, typically DLBCL.
Stain for B-cell markers, CD19, CD20
Germ Cell Tumors
age:
location:
Germ cell tumors are ________ tumors.
Germ Cell Tumors
age: children, very rare
location: midline (pineal and suprasellar regions)
Germ cell tumors are congenital tumors.
What type of tumor is this?
Grade:
Age:
Location:
Risk Factors:
Mutation:
Morphology:
Histology:
This type of tumor may show increased growth during
Meningioma
Grade: I
Age: benign tumor of adults
Location: attached to the dura
Risk Factors: prior radiation
Mutation: chromosome 22. Also associated with NF2 patients
Morphology: Well rounded masses with dural base that may compress underlying brain
Histology: psammoma bodies. Typically reactive to epithelial membrane antigen
This type of tumor may have immunoreactivity to progesterone receptor and may show increased growth during pregnancy. Get an HCG if woman has a tumor and is in childbearing years.
Paraneoplastic Syndromes
definition:
treatment:
Types:
- 3 CNS
- 2 PNS
Paraneoplastic Syndromes
definition: development of an immune response against tumor antigens that cross-react with antigens in the central or peripheral nervous systems
treatment: removal of the tumor
Types:
- 3 CNS- Subacute cerebellar degeneration, Limbic Encephalitis, Eye movement disorders
- 2 PNS: Subacute sensory neuropathy, Lambert-Eaton myasthenic syndrome
CNS Paraneoplastic syndromes:
Subacute cerebellar degeneration:
Limbic Encephalitis:
Eye Movement disorders
CNS Paraneoplastic syndromes:
Subacute cerebellar degeneration:
- loss of coordination and balance, purkinge cell loss, women with ovarian, uterine or breast carcinoma
Limbic Encephalitis:
- sub acute dementia
- syndrome may occur before malignancy is detected
Eye Movement disorders
- most commonly opsoclonus (dancing eyes)
- associated with neuroblastoma
PNS Paraneoplastic Syndromes
Subacute sensory neuropathy
Lambert-Eaton myasthenic syndrome
PNS Paraneoplastic Syndromes
Subacute sensory neuropathy
- loss of sensation upper>lower
- lymphocytic inflammation
- loss of neurons from the dorsal root ganglia
Lambert-Eaton myasthenic syndrome
- weakness, visual loss, difficulty swallowing
- antibodies against the voltage-gated Ca++ channel at NMJ
- can also occur in the absence of malignancy
