CORE Brain Tumors Flashcards
Approach to the evaluation of focal brain lesion
- Are there any tumor-related complications?
- Is the mass intra- or extra-axial?
- Where specifically is the lesion located?
- Are there multiple lesions?
- Distinct MR signal characteristics?
- Does the lesion enhance?
“Complications, location, location, location(s), MR signals and enhancement”
What are the 3 emergent complications of a brain tumor?
The three emergent complications of a brain tumor are the three H’s:
- Hemorrhage: Primary or metastatic brain tumors are often associated with neovascularity and tumoral vessels are more prone to hemorrhage than normal vasculature.
- Hydrocephalus: A tumor can cause hydrocephalus by blocking the flow of CSF. Posterior fossa tumors have increased risk of causing hydrocephalus by effacing the fourth ventricle.
- Herniation: The overall mass effect from the tumor is a combination of the tumoral mass and associated vasogenic edema, which may contribute to brain herniation.
Most common primary brain tumor to hemorrhage?
Glioblastoma
Hemorrhagic metastases to the brain include what primary neoplasms?
- Hemorrhagic metastases include melanoma, renal cell carcinoma, thyroid carcinoma (follicular), and choriocarcinoma.
- Note that this includes 3 / 4 of the neoplasms that metastasize hematogenously.
- Although breast and lung cancer metastases are less frequently hemorrhagic on a case-by-case basis, these two malignancies are so common that they should also be considered in the differential of a hemorrhagic metastasis.
What are the findings of an extra-axial mass?
- Findings of an extra-axial mass include a CSF cleft between the mass and the brain, buckling of gray matter, and gray matter interposed between the mass and white matter.
- Dural tails! (thank you helmer :)
- The presence of white matter edema is not specific to intra-axial masses. In particular, meningioma (an extra-axial dural neoplasm) is known to cause white matter edema of the underlying brain.
- Meningeal enhancement is seen more commonly in extra-axial masses (most commonly meningioma), but can also be seen in intra-axial masses.
What are the findings of intra-axial CNS masses?
- Findings of an intra-axial mass include the absence of intervening gray matter between the mass and the white matter.
- The presence of white matter edema is not specific to intra-axial masses. In particular, meningioma (an extra-axial dural neoplasm) is known to cause white matter edema of the underlying brain.
- Meningeal enhancement is seen more commonly in extra-axial masses (most commonly meningioma), but can also be seen in intra-axial masses.
Do mets enhance? Why?
- Metastases always enhance due to tumoral neo-vessels, which lack a blood-brain barrier.
Tumors hypointense on T2 include:
- Metastases containing desiccated mucin, such as some gastrointestinal adenocarcinomas. Note that mucinous metastases to the brain can have variable signal intensities on T2-weighted images, depending on the water content of the mucin. Hydrated mucin is hyperintense on T2-weighted images.
- Hypercellular tumors, including lymphoma, medulloblastoma, germinoma, and some glioblastomas.
Tumors that are hyperintense on T1 include:
- Metastatic melanoma (melanin is hyperintense on T1-weighted images).
- Fat-containing tumors, such as dermoid or teratoma.
- Hemorrhagic metastasis (including renal cell, thyroid, choriocarcinoma, and melanoma).
Overview the glial cells
- Astrocyte
- Oligodendrocyte
- Ependymal Cells
- Choroid Plexus Cells
Juvenile Pilocytic Astrocytoma (JPA)
What is it?
Imaging?
Specific circumstance with its association?
- Juvenile pilocytic (hair-like) astrocytoma (JPA) is a benign World Health organization WHO grade I tumor seen typically in the posterior fossa in children.
- Imaging shows a well-circumscribed cystic mass with an enhancing nodule and relatively little edema. When in the posterior fossa, JPA may compress the fourth ventricle.
- JPA can also occur along the optic pathway, with up to 1/3 of optic pathway JPA associated with neurofibromatosis type 1. Posterior fossa JPA is not associated with NF1.
What are fibrillary astrocytomas and what do they include?
- Fibrillary astrocytomas are infiltrative tumors that include low-grade astrocytoma, anaplastic astrocytoma, and glioblastoma multiforme (GBM).
- Astrocytomas can occur in the brain or the spinal cord.
What is a low-grade astrocytoma?
Imaging appearance?
- It is one of three fibrillary astrocytomas (low-grade, anaplastic, glioblastoma)
- Low-grade astrocytoma is a WHO grade II tumor that typically presents as a hyperintense mass on T2-weighted images, without enhancement. Imaging findings may be subtle.
Anaplastic astrocytoma
What is it?
Imaging appearance?
- It is one of three fibrillary astrocytomas (low-grade, anaplastic, glioblastoma)
- Anaplastic astrocytoma is a WHO grade III tumor. It features a range of appearances from thickened cortex (similar to low-grade astrocytoma) to an irregularly enhancing mass that may appear identical to glioblastoma. The natural history of the disease is an eventual progression to glioblastoma.
What is Glioblastoma Multiforme?
- It is one of three fibrillary astrocytomas (low-grade, anaplastic, glioblastoma)
- Glioblastoma multiforme (GBM) is an aggressive WHO grade IV tumor of older adults. It is the most common primary CNS malignancy. GBM has a highly variable appearance “multiforme” but typically presents as a white matter mass with heterogeneous enhancement and surrounding non-enhancing T2 prolongation.
- Most of the surrounding T2 prolongation is thought to represent infiltrative tumor.
- GBM is an infiltrative disease that spreads through white matter tracts, through the CSF, and subependymal.
- A GBM that crosses the midline via the corpus callosum is called a butterfly glioma. The differential diagnosis of a transcallosal mass includes glioblastoma, lymphoma, and demyelinating disease.
What is the DDx of a transcallosal mass?
The differential diagnosis of a transcallosal mass includes glioblastoma, lymphoma, and demyelinating disease.
Gliomatosis Cerebri
What is it?
Diagnostic Criteria?
Prognosis?
Typical imaging appearance?
Enhancement pattern?
- Gliomatosis cerebri is a diffuse infiltrative mid-grade (WHO II or III) astrocytoma that affects multiple lobes.
- Diagnostic criteria include involvement of at least two lobes plus extra-cortical involvement of structures such as the basal ganglia, corpus callosum, brainstem, or cerebellum.
- Gliomatosis has a poor prognosis and may degenerate into GBM.
- The typical imaging appearance is diffuse T2 prolongation throughout the involved brain.
- Gliomatosis exerts mass effect but typically does not enhance.
Diffuse T2 CNS prolongation can be seen in what entities?
- Diffuse T2 prolongation can be seen in several entities, typically in immunocompromised patients, including, gliomatosis, lymphoma, progressive multifocal leukoencephalopathy (demyelination caused by JC virus), and AIDS encephalopathy.
Oligodendroglioma
What is it?
The typical presenting patient?
Characteristic feature?
Variants?
- Oligodendroglioma is a WHO grade II tumor that usually presents as a slow-growing cortical-based mass in a young to middle-aged patient presenting with seizures.
- Oligodendrogliomas have a propensity to calcify (approximately 75% calcify).
- Variants such as oligoastrocytoma and anaplastic oligodendroglioma are much more aggressive.
- oligoastrocytoma is a mixed tumor with an astrocytic component. Although oligoastrocytoma can degenerate into GBM, typically prognosis is better than a pure GBM.
- Anaplastic oligodendroglioma is indistinguishable from GBM on imaging and has a poor prognosis.
Ependymoma
What is it?
In who and where does it occur?
Nick name of the tumor?
- An ependymoma is a tumor of ependymal cells that tend to occur in the posterior fossa in children and in the spinal cord in older adults.
- The pediatric posterior fossa ependymoma has been called the toothpaste tumor for its propensity to fill the fourth ventricle and squeeze through the foramina of Magendie or Luschka into the adjacent basal cisterns. Medulloblastoma, the most common pediatric brain tumor, also usually arises in the posterior fossa but does not typically squeeze through the foramina.
- The adult spinal ependymoma can occur anywhere in the intramedullary spinal cord. The main differential diagnosis of an intramedullary spinal cord mass is an astrocytoma, which tends to occur in younger patients. It is not possible to reliably differentiate spinal cord ependymoma from astrocytoma on imaging.
What is Lhermitte-Duclos?
Association?
Classical imaging finding?
Enhancement pattern?
- Lhermitte-Duclos, also called dysplastic cerebellar gangliocytoma, is a WHO grade I cerebellar lesion that is part hamartoma and part neoplasm.
- It is almost always seen in associated with Cowden syndrome (multiple hamartomas and increased risk of several cancers).
- The classical imaging finding is a corduroy or tiger-striped striated lesion in the cerebellar hemisphere. Enhancement is rare.
lhermitte-duClos associated with Cowden syndrome with a Corduroy striated lesion in the cerebellar hemisphere
What are embryonal tumors?
- Embryonal tumors represent a spectrum of WHO grade IV, aggressive childhood malignancies that are known as primitive neuroectodermal tumors (PNET).
- Intracranial PNET tumors are more commonly located in the posterior fossa but may occur supratentorially.
Atypical teratoid/rhabdoid tumor
What is it?
In who and what location does it occur?
Association?
- Atypical teratoid/rhabdoid tumor (ATRT) is a WHO IV, aggressive tumor that may appear similar to medulloblastoma, but occurs in slightly younger patients. The majority occur in the posterior fossa. ATRT is associated with malignant rhabdoid tumor of the kidney.
Medulloblastoma
What is it?
Where does it occur and what is its imaging appearance on CT and MR?
How do differentiate between other other most common entities?
What is Zuckerguss?
Where does it occur in young adults?
- Medulloblastoma is a WHO grade IV tumor of small-blue-cell origin. It is one of the most common pediatric brain tumors.
- Medulloblastoma most commonly occurs in the midline in the cerebellar vermis. It is slightly hyperattenuating on CT due to its densely packed cells and is accordingly hypointense on T2-weighted images and has low ADC values. The tumor is avidly enhancing and may appear heterogeneous due to internal hemorrhage and calcification.
- The low ADC values can be a useful finding to differentiate medulloblastoma from ependymoma and pilocytic astrocytoma, the two other most common childhood posterior fossa tumors.
- Leptomeningeal metastatic disease is present in up to 33% of patients. Sugar-coating (Zuckerguss) is the icing-like enhancement over the brain surface. Imaging of the entire brain and spine should be performed prior to surgery.
- When medulloblastoma occurs in a young adult (as opposed to a child), the tumor tends to arise eccentrically in the posterior fossa, from the cerebellar hemisphere.
Hemangioblastoma
What is it? Location?
Association?
Classic Appearance?
When in the spinal cord, it is associated with what?
- Hemangioblastoma is a highly vascular WHo grade I tumor associated with von Hippel-Lindau (VHL) syndrome that occurs most commonly in the cerebellum, medulla, or spinal cord. It only rarely occurs supratentorially.
- Although associated with VHL, only 30% of patients with hemangioblastoma have VHL. Hemangioblastoma in a patient with VHL has a worse prognosis.
- The classic appearance of hemangioblastoma is a cystic mass with an enhancing mural nodule. Prominent vessels are often seen as tubular areas of flow void. Less commonly, a hemangioblastoma may be solid or hemorrhagic.
- When in the spinal cord, hemangioblastoma is often associated with a syrinx.
Pleomorphic Xanthoastrocytoma
What is it?
Presentation?
Location? Appearance?
Main differential consideration and how to differentiate?
- Pleomorphic xanthoastrocytoma (PXA) is a low-grade WHo grade II astrocytoma variant.
- PXA is a rare tumor of childhood and adolescents, typically with a history of chronic epilepsy.
- The most common location of PXA is the temporal lobe, where it typically presents as a supratentorial cortical cystic mass with an enhancing mural nodule. The overlying dura may be thickened and enhancing.
- The main differential consideration, both by imaging and clinical presentation, is ganglioglioma; however, ganglioglioma does not usually cause dural thickening.
Ganglioglioma
What is it?
Common presentation?
Characteristic imaging appearance?
- Ganglioglioma is a rare, slow-growing neuroglial tumor that typically presents in an adolescent or young adult with medically refractory temporal lobe epilepsy.
- Ganglioglioma characteristically appears as a temporal lobe cyst and enhancing mural nodule, often with calcification. ganglioglioma may cause calvarial remodeling and scalloping.
What are the brain tumors with a cyst and an enhancing nodule?
- Juvenile Pilocytic Astrocytoma
- Pleomorphic Xanthoastrocytoma
- Gangliogloma
- Hemangioblastoma
(remember JPA and remember PXA has “astrocyoma” in it and then remember ganglioglioma is similar to PXA. . . then you just gotta remember hemangioblastoma)
What are the intraventricular tumors?
- Subependymoma
- Intraventricular Meningioma
- Choroid plexus papilloma/carcinoma
- Central Neurocytoma
- Subependymal Giant Cell Astrocytoma
MNEMONIC: “SMCkNS (smackens!) in the ventricles”
Central neurocytoma
What is it?
Imaging appearance?
- Central neurocytoma is a low-grade tumor likely of neuronal origin that occurs in young adults, from teenagers to young middle aged-patients. Prognosis is excellent.
- Typical imaging appearance is a lobulated mass attached to the septum pellucidum, with numerous intratumoral cystic areas. Calcification is common.
Choroid Plexus Papilloma/Carcinoma
What is it?
Image appearance?
Location in children and in adults?
How to differentiate between papilloma and CA?
- Choroid plexus papilloma is a rare intraventricular tumor. Choroid plexus papilloma is a low-grade (WHO I) neoplasm arising from choroid plexus epithelial cells. It is the most common brain tumor in babies <1 year old, but it may also occur in adults.
- T2-weighted images show a lobulated, heterogeneous or hyperintense mass that avidly enhances on T1-weighted MRI.
- In children, the atrium of the lateral ventricle is the most common location.
- In adults, the fourth ventricle is the most common location.
- Less commonly, choroid plexus papilloma may arise from the third ventricle or cerebellomedullary angle (shown).
- Choroid plexus papilloma and carcinoma (WHO III) are not reliably distinguishable.
Intraventricular Meningioma
Common location? In what population?
How to differentiate this from other intraventricular neoplasms?
- Intraventricular meningioma appears as a solid mass, typically in the trigone of the lateral ventricle. It tends to occur in older patients, similar to other meningiomas.
- Intraventricular meningiomas are typically hypercellular and homogeneously enhance, distinguishing them from other intraventricular neoplasms.