Central Nervous System Neoplasia Flashcards
What are the common childhood CNS neoplasms?
Medullablastoma, Pilocytic Astrocytoma, Diffuse Pontine Astrocytoma
What is the most common cns tumor? Three methods by which these tumors arise? What genetic abnormality most often contributes to this tumor?
Meningioma; Sporadic, Iatrogenic (Typically Post-Irradiation), Tumor Predisposition Syndrome (Neurofibromatosis Type 2)
A patient with frequent seizures over many years expires. The histological examination of her brain is shown below. What is your Dx? What are the typical histologic findings observed?
Meningioma; Whorled pattern, psammoma bodies, epithelial membrane antigen (EMA)
A histologic section of a brain is shown below. What is your Dx? What are these morphological findings? To what degree does this tumore infiltrate the brain parenchyma?
Meningioma; These are Psamomma bodies! It generally doesn’t!
What is the most common primary brain tumor? Which subtype infiltrates the brain?
Astrocytoma; Diffuse astrocytoma
What makes treatment of astrocytomas difficult?
The tumor has the ability to infiltrate the brain and spinal cord
A patient presents and the MRI shows the following lesion. What is this lesion called and what is it indicative of? What is the likely pathophys?
Butterfly lesion; Astrocytoma; Astrocytoma spread across the corpus callosum
A brain biopsy shows a very cellular, pleomorphic and highly proliferating astrocytoma. What subtype of astrocytoma is this? What type of tumor does this tumor typically progress to?
Anaplastic astrocytoma (WHO grade III); Glioblastoma
A brain biopsy shows a tumor with vascular proliferation, foci of tumor necrosis, and a densely cellular cuff of tumor cells. What type of tumor is this? What is the term used for the tumor cells?
Glioblastoma multiforme (WHO grade IV); Pseudopalisading necrosis)
A brain at biopsy is shown. What type of astrocytoma is this? What is the range of grades of this tumor?
Diffuse pontine astrocytoma; Grade II-IV
T/F
The vast majority of GBM are due to a genetic predisposition
False, GBMs are primarily sporadic
What are the genes associated with primary (de novo) astrocytomas? Secondary (progression from low grade tumors)?
EGFR, PTEN; p53
A brain biopsy shows neoplastic pilocytes (hair cells) and elongated bipolar cytoplasmic processs (dark red). What type of tumor is this? What are the dark red processes called? What is the general prognosis? What variant of this has a worse prognosis?
Pilocytic astrocytoma (WHO grade I); Rosenthal fibers (HALLMARK); Surgery can be curative if in an accessible place; Pilomyxoid astrocytoma (WHO grade II)
A brain biopsy is shown below showing pleomorphic tumor cells, low mitotic activity. Characteristic eosinophilic granular bodies are observed. What tumor is this? In what patient population is it usually seen? Syx? Location in brain?
Pleomorphic Xanthoastrocytoma (WHO Grade II); Children and young adults; Seizures; Temporal Lobe
A young child experiences signs of obstructive hydrocelphalus and is admitted to the ER. The MRI of the patients head is shown. What is the likely tumor? Describe the histology of the tumor. What condition is associated with this type of tumor?
Subependymal Giant Cell Astrocytoma (SEGA); Globular eosinophilic cytoplasm with single prominent nucleoli; Tuberous Sclerosis;
A brain biopsy from a 45 yo patient is shown below. Describe the histological findings. What type oof tumor is this?
Perinuclear halo/Fried egg histology (DIAGNOSTIC); Oligodendroglioma (WHO Grade II);
This tumor reveals that of anoligodendroglioma but has increased mitotic activity and presence of microvascular proliferation. What type of tumor is it?
Anaplastic Oligodendroglioma (WHO Grade III)
A young patient has a tumor growing in the posterior fossa fourth ventricle. It is excised with relative ease and shown is the histological analysis. What is the characteristic histological hallmark shown? What type of markers would this section stain for? What type of tumor is this and what can it advance to?
Perivascular pseudorosette; Epithelial membrane antigen (EMA), Glial fibrillary acidic protein (GFAP); Ependymoma (WHO Grade II); Anaplastic ependymoma (WHO Grade III)
An adult patient was found to have a tumor in his lumbar cistern. The tumor was easily removed without complications. The histological analysis is shown below. Myxoid microcysts and perivascular myxoid cuffs separate nests and cords of ependymal cells. What is this tumor? What does it stain for?
Myxopapillary Ependymoma (WHO Grade I); S-100 protein, GFAP, EMA
An adult patient has a brain scan and the following MRI is shown. An asymptomatic mass is shown. What is the mass? Describe its histology
Subependymoma (WHO Grade I); Cluster of small, bland glial nuclei embedded within an abundant finely fibrillar matrix of tumor cell processes
A tumor is found in the ventricle of a child. The histology of the tumor is shown. The tumor was found to originate from the choroid plexus. What is the tumor? Why? What does it stain for? What are other subtypes of this tumor?
Choroid Plexus Papilloma (WHO Grade I); The cytology shows crowded columnar and organized cells. Not disorganized compared to Grade II and III; S-100, GFAP, Transthretin; Atypical CPP (II), Choroid Plexus Carcinoma (III)
In what population do Medulloblastomas primarily occur?
Children
Where do medulloblastomas most commonly occur?
Cerebellum
Amplification of what gene is associated with a poor outcome in medullablastoma?
MYCN
Which two subtypes of medulloblastoma have the best prognosis? Which two subtypes have the worst prognosis?
Desmoplastic/nodular and extensive nodularity; Anaplastic and large cell
What population are desmoplastic/nodular MBs generally associated with? MBs with extensive nodularity?
Adults; Infants
What markers do Anaplastic and Large Cell MB show?
Synaptophysin and some have GFAP
What are the two genes mutations associated with MBs and their respective subtypes?
- Wnt - classic anaplastic/large cell MBs
- SHH - Desmoplastic/nodular MB and MB with extensive nodularity
What is the hallmark for Atypical Teratoid/Rhabdoid Tumor (WHO grade IV)? What are hallmark findings on immunostaining?
Invactivation of INI-1 tumor suppressor gene through mutation or deletion; Loss of INI-1 protein on immunostaining
From where do craniopharyngiomas arise? What are the two subtypes? Relative patient population?
Sella turcica and suprasellar region; Adamantinomatous (children and adults), papillary (adults)
A brain biopsy is shown below. The tumor arose in the midline from the pineal gland. The tumor has a biphasic cell population: large tumor cells and small reactive lymphocytes. What type of tumor is this? What does this type of tumor stain for?
Germinoma; OCT3/4 and cytoplasmic positivity for placental alkaline phosphatase (PLAP)
A 30 yo patient has a tumor in the cerebellum. The patient also has polycythemia. What type of tumor is it? What can it secrete? What disease is it associated with? Describe its histology. What does it stain for on IHC?
Hemangioblastoma; Erythropoietin; von Hippel-Lindau disease; Vacuolated stromal cells amid a dense capillary vasculature; Inhibin-Alpha
What syndrome is associated with Gangliocytomas?
Cowden syndrome
What marker is associated with Central neurocytoma and extraventricular neurocytoma?
Synaptophysin is strongly expressed
What is a ganglioglioma? Where is it generally located? What condition is it associated with? What can it progress to?
Neoplastic ganglion cells with glioma component; Temporal lobe; Chronic temporal lobe epilepsy; Anaplastic Ganglioglioma (WHO Grade III)
An autopsy of a child’s brain is shown below. The child had history of recurrent seizures. The tumor was found in the cerebral cortex. Oligogliodenral cells that “float” in cystic spaces in cortial parenchyma. What is the tumor?
Dysembryoplastic Neuroepithelial Tumor
A brain tumor expressing CD20 antigen is treated with corticosteroids. Treatment is initially effective but then fails and the patient expires. What is the likely Dx?
Primary Central Nervous Lymphoma
Where are Rathke Cysts located?
Sellar/Suprasellar Regions
Where are colloid cysts located?
Ventricle near the foramen of Monro
Where are neurenteric cysts located?
Subarachnoid space anterior to the brainstem medulla or cervical spinal cord
What are the most common central nervous system tumors?
Metastases
What is Tuberous Sclerosis?
Autosomal Dominant disease characterized by hamartomas of the brain, retina, and viscera. Extracranial lesions include facial angiofibromas, cardiac rhabdomyomas, mesenchymal tumor of the kidney
What is Sturge-Weber Syndrome?
Non-familial congenital autosomal dominant disorder characterized by angiomas of brain and face (unilateral face lesion - port wine lesion)
