Tumors/cysts Flashcards
Astrocytoma
Pilocytic astrocytoma
SEGA
Pleomorphic xanthoastrocytoma
Diffuse astrocytoma
Anaplastic astrocytoma
Glioblastoma multiforme
Oligodendroma
Neuronal and mixed neuronal glial tumors
Ganglioglioma
Desmoplastic infantile astrocytoma/ganglioglioma
Dysembryonic neuroeoithelial tumor (DNET)
Central neurocytoma
Pineal region tumors
Pineoblastoma
Pineocytoma
Embryonal tumors
Medulloblastoma
Meningeal tumors
Meningioma
Cranial nerve tumors
Schwannoma
Neurofibroma
Optic nerve glioma
Carcinomatosis meningitis
Lymphomatous meningitis
Lymphomas and hematopoietic tumors
Hemangioblastoma
CNS lymphoma
Intravascular lymphoma
Hemangiomas
Multiple myeloma
Langerhans cell histocytosis
Germ cell tumors
Germinoma
Sella region tumors
Craniopharyngioma
Pituitary macroadenoma
Pituitary microadenoma
Hypothalamic hamartoma
Familial tumor syndromes of CNS
NF-1
NF-2
TS
Sturge Weber
Von Hippel Lindau
Li-Fraumeni
Codes syndrome
Turcot
Paraneoplastic disorders
Limbus encephalitis
Paraneoplastic cerebellar degeneration
Lambert Eaton
A pediatric, slow growing tumor composed of astrocytes.
Pilocytic astrocytoma
Syndromes can develop Pilocytic astrocytomas?
TS
NF1
Li-Fraumeni
Symptoms of Pilocytic astrocytoma:
Increased ICP - HA, N/V, vision loss, ataxia, CN nerve deficits
Location based
What tumors are associated with NF1?
Optic nerve and chiasm glioma
Imaging findings for Pilocytic astrocytoma
Cystic mass w/ contrast rim enhancing mural nodule
Minimal vasogenic edema
Where are Pilocytic astrocytomas most commonly found?
Cerebellum
Supratentorial hemispheres > optic pathway/hypothal > spinal cord
Characteristic gene fusion for pilocytic astrocytoma
KIAA1549-BRAF
Pathology characteristics of pilocytic astrocytoma
Rosenthal fibers
Eosinophilia granular bodies in stacked bipolar cells
Describe the classic presentation of a pilocytic astrocytoma in children
D w/ increased ICP/ataxia found to have a cystic cerebellar mass
Which patients are subependymal giant cell astrocytoma almost exclusively seen in?
Tuberous sclerosis
When do SEGAs become symptomatic?
If they become large enough to cause obstructive hydrocephalus if blocking the foramen of Monro
What is seen on pathology of SEGAs/
Large polygonal cells with eosinophilic cytoplasm and a smaller number of giant pyramidal ganglioid astrocytes
What is the treatment for SEGAs?
Resection
What tumor is commonly found in younger patients presenting with temporal lobe epilepsy?
Pleomorphic xanthoastrocytoma (PXA)
What is seen in imaging of a Pleomorphic xanthoastrocytoma (PXA)?
Supratentorial peripheral cystic and contrast-enhancing mass abutting the leptomeninges with enhancing dural tail sign and scalloping of overlying bone
What mutation are PXA associated with?
BRAF
What age group is most common for diffuse astrocytoma?
Young adults 20-45
Common presentation for diffuse astrocytoma:
Seizure and headaches
What genes are diffuse astrocytoma associated with?
Isocitrate dehydrogenase (IDH)
TP53
ATRX
What is a gemistocytic astrocytoma?
Subtype of IDH mutant diffuse astrocytoma
More aggressive behavior
What are the MRS findings for diffuse astrocytoma?
Elevated choline peak, low NAA peak, elevated choline: creatine ratio
Treatment for diffuse astrocytoma:
Maximal safe resection
Field radiation
Chemo
What indicated a better prognosis in anaplastic astrocytoma?
IDH1/2 mutation and MGMT methylation
What does imaging appear as for anaplastic astrocytoma?
An infiltrating mass that predominantly involves hemispheric white matter with variable enhancement
What pathologic findings are seen in anaplastic astrocytoma?
Increased cellularity
Marked mitotic activity
Distinct nuclear atypia
No necrosis or endothelial proliferation
What is the characteristic pattern seen in imaging for a glioblastoma (GBM)?
Butterfly lesion
Contrast-enhancing multicystic lesions with significant vasogenic edema
Internal necrosis and can extend through the corpus callosum
What can indicate a better response to alkylating chemotherapy for GBM?
MGMT promoter methylation
If a GBM has a IDH-1/2 mutation what could this mean?
Secondary GBM arising from a lower-grade glioma
What is the pathology associated with GBM?
Increased mitotic activity with pseudopalisading necrosis
Microvascular endothelial proliferation
Treatment for GBM
Maximal safe resection
Intensity-modulated radiation therapy (IMRT) and temozolomide
In GBM what indicated a better treatment response to temozolomide?
Methylation of the MGMT gene
What chemotherapy can be used in recurrent or progressive GBM?
Bevacizumab
Inhibits vascular endothelial growth factor (VEGF)
What grade are oligodendromas?
WHO grade II or WHO grade III
What genetic findings are associated with oligodendromas?
1p/19q co-deletion and IDH mutations
What mutation has a better prognosis in oligodendromas?
1p/19q co-deletion
Where are oligodendromas generally found in the brain?
Frontal or temporal lobes
Pathologic findings in oligodendroma:
Monotonous round nuclei, surrounded by prominent perinuclear halos
“Fried egg” appearance
Pathologic findings in ependymal tumors:
Monomorphic cells with round/oval nuclei
Salt and pepper chromatin
Perivascular pseudorosettes
What age group of choroid plexus papilloma or carcinoma occur in?
Usually infants, before age one
What are the symptoms of choroid plexus papilloma?
Symptoms of increased ICP due to overproduction of CSF
Imaging findings for ganglioma:
A cystic mass with a mural nodule
Usually temporal lobe
What is a symptoms based on location of a ganglioma?
Refractory epilepsy
Mutations associated with gangliomas:
BRAF
IDH negative
Pathology of ganglioma:
Dysmorphic ganglion cells with binucleated forms
What are the characteristics of a Desmoplastic infantile astrocytoma and ganglioglioma?
WHO grade 1
Present before age 2
M>F
Rapid increase in head size
Imaging findings for Desmoplastic infantile astrocytoma and ganglioglioma:
Large cystic tumor with a peripheral solid component attached to the meninges of frontal or parietal lobes
What are the characteristics of a Dysembryonic neuroepithelial tumor (DNET)?
WHO grade 1
Arises from cortical gray matter
Associated with cortical dysplasia
Often temporal lobes
What do DNETs tend to cause?
Temporal lobe epilepsy
Pathology associated with DNET:
Myxoid clear areas with floating neurons
Imaging associated with DNET:
A wedge-shaped cystic cortical mass lesion “soap-bubble” appearance
Typically non-enhancing
What are the characteristics of a central neurocytoma?
WHO grade II
Signs/symptoms of increased ICP
Imaging findings for a central neurocytoma:
Cystic/bubbly lesion within the lateral ventricle near the foramen of Monro
Often attached to septum pellucidum
Pathologic findings in central neurocytoma:
Neurocytic rosettes and uniform round blue cells
What are the characteristics of a pineoblastoma?
Seen in children
Can lead to hydrocephalous
Imaging findings in pineoblastoma?
A calcified heterogeneous soft tissue mass in the pineal region with enhancement
Pathologic findings in pineoblastoma:
Densely cellular primitive neuroectodermal tumors that often form Homer Wright (medulloblastoma-type) rosettes
What symptoms will pineocytoma present with?
Hydrocephalus or mass effect
What is the most common malignant brain tumor in childhood?
Medulloblastoma
