Neuro-oncology Flashcards
Patient presents with opsoclonus myoclonus syndrome (spontaneous rapid, irregular and high-amplitude conjugate eye movements that occur in all direction). What are the possible diagnosis?
Neuroblastoma
Paraneoplastic Breast Cancer (Anti-Ri/ANNA-2), Ovarian and Small Cell Lung Cancer
What is the management for neuroblastoma?
ACTH
What are examples of diffuse astrocytoma?
fibrillary gemistocytic protoplasmic small cell giant cell epithelioid granular cell glioblastoma with oligodendroglioma component
What type of diffuse astrocytoma presents with elongated hyperchromatic nuclei, scant cytoplasm, and the presence of a fibrillary background?
A. Gemistocytic Astrocytoma
B. Fibrillary Astrocytoma
C. Proteoplasmic Astrocytoma
D. Pleomorphic Xanthoastrocytoma
B. Fibrillary Astrocytoma
What type of diffuse astrocytoma presents with rounded cells with prominent eosinophilic cytoplasm?
A. Gemistocytic Astrocytoma
B. Fibrillary Astrocytoma
C. Proteoplasmic Astrocytoma
D. Pleomorphic Xanthoastrocytoma
A. Gemistocytic Astrocytoma
What type of diffuse astrocytoma presents with cells with oval-shaped nuclei and wispy cobweb-like processes?
A. Gemistocytic Astrocytoma
B. Fibrillary Astrocytoma
C. Proteoplasmic Astrocytoma
D. Pleomorphic Xanthoastrocytoma
C. Proteoplasmic Astrocytoma
What is the most common intracranial brain tumor?
Meningioma
Buzzwords
Perinecrotic Pseudopalisading
Glioblastoma
What are the histopathologic findings in Glioblastoma?
nuclear atypia
mitoses
endothelial hyperplasia and necrosis
Perinecrotic Pseudopalisading
Buzzwords
Cells with “fried egg” appearance
oligodendrogliomas
Buzzwords
Perivascular pseudorosettes
Ependymoma
Buzzwords
Homer-Wright rosettes
Medulloblastoma
Buzzwords
Rosenthal fibers
- Alexander’s disease
- Pilocytic astrocytoma
- Pleomorphic xanthoastrocytoma
- Chronic reactive gliosis.
This is the most common glioma in children. Most commonly seen in the cerebellum. Imaging findings would show cystic lesions with gadolinium enhancing mural nodule. However, in the hypothalamus and optic nerve they are solid looking
Pilocytic Astrocytoma WHO Grade I
Which type of Pilocytic Astrocytoma most commonly associated with Neurofibromatosis Type I?
Optic Nerve Glioma
What is the most common location of pleomorhic xanthoastrocytoma (PXA)? A. Frontal Lobes B. Temporal Lobes C. Brainstem (pons) D. Cerebellum
B. Temporal Lobes
What are the histopathologic findings of pleomorphic xanthoastrocytoma?
• pleomorphic astrocytes arranged in fascicles, with intercellular reticulin deposition, mesenchymal-like cells, and multinucleated giant cells.
- There are lipidized astrocytes
- Eosinophilic granular bodies are typically seen, and Rosenthal fibers may be seen in the periphery of the lesion.
What are the histopathologic findings of subependymal giant cell astrocytoma?
- glioneuronal appearance, and the cells are packed in fascicles and around blood vessels, giving the appearance of perivascular pseudorosettes
- Immunoreactivity for both GFAP and neuronal markers
In oligodendroglioma, there is the deletion of 1p/19q. What does this imply?
Deletion of 1p/19q is associated with prolonged survival and better response to treatment.
Chromosome 1p
• predictive of high degree of responsiveness to PCV chemotherapy regimen
Chromosome 19q (+) longer survival
What is the most common location of oligodendroglioma? A. Frontal Lobes B. Temporal Lobes C. Brainstem (pons) D. Cerebellum
A. Frontal Lobes
Is oligodendroglioma, GFAP positive or negative?
GFAP negative except menigemistocystic or gliofibrillary oligodendrocytes are present (GFAP Positive)
Oligodendroglioma’s borders are well-defined and non-infiltrating. True or False?
False. Oligodendrogliomas are diffuse glioma which are infiltrating with ill-defined borders.
What are the histopathologic findings in pilocytic astrocytoma?
- Presence of biphasic pattern with compact fibrillar and loose microcytic areas
- Presence of Rosenthal fibers located in the compact region. There is also eosinophilic granular bodies seen.
Buzzwords
Eosinophilic Granular Bodies
- Pleomorphic xanthoastrocytoma
- Pilocytic Astrocytoma
- Gangliogliomas
What are the histopathologic findings in oligodendroglioma?
hypercellular tumor with uniformly rounded nuclei and clear pericellular haloes, giving it a “fried egg” appearance. There are also capillaries with a “chicken wire” appearance
Clear halos is caused by a delayed fixation artifact
What are the histopathologic findings in ependymoma?
- arranged in sheets of spindled cells with round nuclei and small nucleoli
- perivascular pseudorosettes, in which the cells surround blood vessels
- True ependymal Rosettes (Flexner Wintersteiner) - cells surround clear space
Tumor that is reactive with synaptophysin, a marker of neuronal differentiation and which detects neuronal vesicle proteins.
A. Oligodendroglioma
B. Neurocytoma
C. Ependymoma
D. Meningioma
B. Neurocytoma
Where is the most common location of colloid cyst?
Anterior 3rd Ventricle
What is the histopathologic finding of colloid cyst?
hin-walled lining and contain thick and cloudy gelatinous fluid. Microscopically, there is a single layer of columnar ciliated or goblet cells.
Where is the most common location of dermoid cyst?
cerebellar vermis
In HIV and immunocompromised patients, primary CNS Lymphoma is frequently associated with Epstein–Barr virus (EBV). True or False?
True
PCNSL can also occur in immunocompetent hosts; however, this is rare, and there is a lower association with EBV.
Buzzwords
Verocay Bodies
Vestibular Schwannoma
What are the common locations of these cysts?
Epidermoid
Epidermoid- CP-Angle
Among intracranial tumors, which 2 have female predominance?
Meningioma
Match the disease with associated CNS tumor.
Neurofibromatosis 2
A- Meningioma B- Lhermitte Duclos C- Medulloblastoma D- SEGA E- Hemangioblastoma
A- Meningioma
Match the disease with associated CNS tumor.
Tuberous Sclerosis
A- Meningioma B- Lhermitte Duclos C- Medulloblastoma D- SEGA E- Hemangioblastoma
D- SEGA
Match the disease with associated CNS tumor.
Von Hippel Lindau
A- Meningioma B- Lhermitte Duclos C- Medulloblastoma D- SEGA E- Hemangioblastoma
E- Hemangioblastoma
Most serious and most common primary malignant tumor
Glial Cell Origin
Meningioma is a disease of the dura and arachnoid. Which is a pial disease?
A. CNS Lymphoma
B. CNS Leukemia
C. Sarcoidosis
B. CNS Leukemia
What are the 4 malignancies with tendency to metastasize to CNS?
Lung
Breast
Melanoma
Renal Cell CA
A patient presents with solitary metastatic brain lesion. You decide to screen for the following primary malignancy EXCEPT: A. Breast ca B. Melanoma C. Thyroid ca D. Renal cell ca
B. Melanoma
*Remember paired organs present as solitary tumors
Identify 5 malignancies that can present as intracranial hemorrhage:
Lung Melanoma Thyroid Renal Cell CA Chorioepithelioma
What are the common locations of these cysts?
Arachnoid
Arachnoid- Sylvian Fissure
Among intracranial tumors, which 2 have female predominance?
Meningioma
Glioma of Optic Chiasm
- Everthing else, male predominance
- Schwannoma male = female
When do the MOST SERIOUS RT-related injury occur?
A. ACUTE - when there are signs of increased ICP
B. EARLY DELAYED- when there is increased tumor mass on MRI
C. LATE DELAYED- when there is necrosis of white matter
C. LATE DELAYED- when there is necrosis of white matter
*In Pedia, LAte Delayed effects include PANHYPOPITUITARISM which causes growth retardation
Your patient who underwent RT developed severe headache and aphasia. Repeat MRI showed gyriform enhancement and focal narrowing of blood vessels. What is your Diagnosis? Treatment?
SMART
Steroids
Which autoantibody is not associated with Lung ca? A. Anti-Ri B. Anti-NMDA C. Anti-GAD D. Anti-CRMP5
B. Anti-NMDA
Anti-NMDA (ovarian CA)
Your patient underwent transphenoidal excision of pituitary tumor. Unfortunately, like 15% of GH secreting tumors and prolactinomas, the tumor recurred after 1 year. You offered RT. An alternative primary treatment is a stereotactic radiosurgery. Give 2 conditions where this may be applicable.
Vision not threateneed
No urgent need for surgery
Advantage: Tumor recurrence is rare
Disadvantage: Obtained Several Months
If you’re only allowed to do 3 hormone tests for a pituitary adenoma, which hormones will most likely yield an abnormal result?
Prolactin, GH, TSH
*chromophobe or acidophils, most common tumor of pituitary adenoma
vs Basophilic: ACTH, B, lipotopin, LH, FSH
*Frequency: Chromo > acido >basophil (5:4:1)
What is the most sensitive test to check for presence of Acoustic Schwannoma?
BAER
According to Adams, ependymomas occur twice as frequently as oligodendrogliomas. True or false?
False
GBM 80%
Low grade glioma 25-30%
Oligodendroglioma 5-7%
Ependymoma 6%
Match the disease with associated CNS tumor.
Cowden Syndrome Neurofibromatosis 2 Tuberous Sclerosis Von Hippel Lindau Gorlin and Turcot syndrome
A- Meningioma B- Lhermitte Duclos C- Medulloblastoma D- SEGA E- Hemangioblastoma
Cowden Syndrome = B Neurofibromatosis 2 = A Tuberous Sclerosis = D Von Hippel Lindau = E Gorlin and Turcot syndrome = C
If you were a choroid plexus papilloma, where would you most likely be found?
A. Lateral vent
B. 3rd vent
C. 4th vent
A. Lateral vent
Ependymoma 4th vent is most common cerebral site
Cranial nerve palsy suggestive of lymphoma? A. CN 5 B. CN 6 C. CN 7 D. CN 8
D. CN 8
What is the gene defect associated with Meningioma?
Merlin Gene (Neurofibromatosis 2) chromosome 22q
What is the most common initial manifestation of primary and metastatic neoplasm? A. Nocturnal/ first awakening headache B. First seizure C. Vomiting and Dizziness D. Mental Asthenia
B. First seizure
A cancer patient will want which type of mutation for better prognosis? A. Deletion on chromosome 17p B. MYC N oncogene amplification C. IDH 1 and 2 mutation D. Chrom 1p,19q duplication E. C&D
C. IDH 1 and 2 mutation
A- Deletion on Chromosome 17p will inactivate Tumor Suppressor Gene
B-MYC N oncogene amplification will result in aggressive clinical course
C. IDH 1 and 2 mutation will cause less tumor progression
D. Chromosome 1p, 19q DELETION (not duplication) will offer good response to chemotherapy
- True/False regarding causes of edema:
VASOGENIC: tumor growth
INTERSTITIAL: Hypoxic-ischemic
CYTOTOXIC: obstructive hydrocephalus
VASOGENIC: tumor growth =TRUE
INTERSTITIAL: Hypoxic-ischemic =FALSE
CYTOTOXIC: obstructive hydrocephalus =FALSE
When you see this finding on one eye (picture of papilledema) plus optic atrophy on the other eye, what is your diagnosis?
Foster-Kennedy Syndrome
Where is the most common location of ependymoma?
A. Cerebellar Vermis
B. Frontal Lobe
C. Parietal Lobe
D. 4th Ventricle
D. 4th Ventricle
Where is the most common location of oligodendroglioma?
A. Cerebellar Vermis
B. Frontal Lobe
C. Parietal Lobe
D. 4th Ventricle
B. Frontal Lobe
Where is the most common location of medulloblastoma?
A. Cerebellar Vermis
B. Frontal Lobe
C. Parietal Lobe
D. 4th Ventricle
A. Cerebellar Vermis
What other tumors may show Homer-Wright Rosettes?
Medulloblatoma
Pineoblastoma
PNET
Olfactory Neuroblastoma
What is the pathophysiology of papilledema in brain tumors?
A. Due to Elevation of ICP and perioptic pressure
B. Impairs axonal transport in the optic nerve
C. Impairs venous drainage from optic nerve head and retina
D. All of the above
D. All of the above
Why is white matter more involved than grey matter in during edema formation?
White matter is affected due to its loose structural organization –> less resistance to fluid under pressure
Tumor growth
What kind of edema?
A. Vasogenic
B. Cytotoxic
C. Interstitial
A. Vasogenic
Lead encephalopathy
What kind of edema?
A. Vasogenic
B. Cytotoxic
C. Interstitial
A. Vasogenic
Malignant hypertension
What kind of edema?
A. Vasogenic
B. Cytotoxic
C. Interstitial
A. Vasogenic
Osmotic dysequilibrium syndrome of hemodialysis
What kind of edema?
A. Vasogenic
B. Cytotoxic
C. Interstitial
B. Cytotoxic
Inappropriate secretion of antidiuretic hormone
What kind of edema?
A. Vasogenic
B. Cytotoxic
C. Interstitial
B. Cytotoxic
Hypoxic-ischemic injury
What kind of edema?
A. Vasogenic
B. Cytotoxic
C. Interstitial
B. Cytotoxic
Acute hepatic encephalopathy
What kind of edema?
A. Vasogenic
B. Cytotoxic
C. Interstitial
B. Cytotoxic
Acute hypoosmolality of plasma
What kind of edema?
A. Vasogenic
B. Cytotoxic
C. Interstitial
B. Cytotoxic
What is the pathophysiology of vasogenic edema?
A. Defect in the gap endothelial cell junctions
B. Defect in the tight endothelial cell junctions
C. Shift of water from extracellular to intracellular compartment
D. Failure of ATP dependent sodium pump within cells (Na-K-ATPase Pump)
E. Ependymal lining is disrupted
B. Defect in the tight endothelial cell junctions
What is the pathophysiology of interstital edema?
A. Defect in the gap endothelial cell junctions
B. Defect in the tight endothelial cell junctions
C. Shift of water from extracellular to intracellular compartment
D. Failure of ATP dependent sodium pump within cells (Na-K-ATPase Pump)
E. Ependymal lining is disrupted
E. Ependymal lining is disrupted
What is the pathophysiology of cytotoxic edema?
A. Defect in the gap endothelial cell junctions
B. Defect in the tight endothelial cell junctions
C. Shift of water from intracellular to extracellular compartment
D. Failure of ATP dependent sodium pump within cells (Na-K-ATPase Pump)
E. Ependymal lining is disrupted
D. Failure of ATP dependent sodium pump within cells (Na-K-ATPase Pump)
How to differentiate cyctotoxic from vasogenic edema based on imaging?
DWI Vasogenic Edema: Increased diffusivity Cytotoxic Edema Decreased diffusivity
What is the effect of CO2 in the brain?
A. Vasodilation
B. Vasoconstriction
A. Vasodilation
Most common solid tumor of childhood.
A. Neuroblastoma
B. Ependymoma
C. Medulloblastoma
D. Metastatic Carcinoma
A. Neuroblastoma
N-MYCN amplification
good or poor prognosis?
Poor prognosis
WNT mutation
good or poor prognosis?
Good prognosis