Tumors of the Nervous System Flashcards
Tumor that presents microscopically with necrosis in a serpentine pattern; necrosis bordered by Pseudopalisading pattern; extensive vascular/endothelial cell proliferation; Glomeruloid bodies present
Glioblastoma
Double layer of endothelail cells that tufts to form a ball-like structure.
Glomeruloid body
What is the condition called when multiple regions of the brain are infiltrated by neoplstic astrocytes?
Gliomatosis cerebri
What mutations result in a primary glioblastoma? Secondary glioblastoma?
1) EGFR
2) PDGF
Cystic glioma that is benign; typically occurs in children; commonly found in the cerebellum; composed of bipolar cells with long “hair like” processess; GFAP positive; necrosis and mitoses are uncommon; Rosenthal fibers
Pilocytic Astrocytoma
Where are pilocytic astrocytomas most commonly found?
Cerebellum, third ventricle, or the optic nerve
Where are pleomorphic Xanthoastrocytomas located? What do they appear microscopically?
1) Temporal lobe
2) Appears microscopically as a high grade tumor due to atypia
Glioma that presents usually in the 4th to 5th decades of life; often found in the cerebrum, appears well circumscribed often with cysts, focal hemorrhage, and calcifications
Oligodendroglioma
What are the mutations found in oligodendrogliomas?
THINK: O119odendroglioma
1) Loss of heterozygosity for chromosomes 1 and 19
Ependymoma like tumor that appears in the filum terminale of the spinal cord; contains papillary elements in a myxoid background along with ependymoma like cells
Myxopapillary ependymoma
Where are Myxopapillary ependymoma most commonly found?
1) Filum terminale of the spinal cord
Neuronal tumor that at first progresses slowly and then rapidly becomes active
1) Ganglioma
Poorly differentiated neoplasm exclusively found int he cerebellum; commonly found in children; Composed of cells with small scant cytoplasm and hyperchromatic nuclei
Medulloblastoma
What is an important property that influences the treatment of Medulloblastoma?
1) It is exquisitely radiosensitive
Cell with eosiniophilic cytoplasm, sharp cell borders, and eccentrically located nuclei
Rhabdoid cell
What chromosome mutation are commonly associated with meduloblastoma?
1) chromosome 17
Highly aggressive tumor of the very young
Atypical teratoid/rhabdoid tumor
What tumor is commonly associated with calcifications?
Oligodendrogliomas
Tumor consisting of cells that express Bcl-6 and show “Hooping” on silver stain; multifocal masses
Primary CNS lymphoma
Where do germ cell tumors in the CNS usually arise?
Occur along the midline, commonly in the pineal and suprasellar regions
What causes an increased risk for meningiomas?
1) Radiation therapy
2) Mutation in NF2
Benign tumor consisting of round masses with dural bases that may compress underlying brain
Meningioma
What is the common genetic cause of meningiomas?
1) Loss of chromosome 22
What does a meningioma arise from?
1) Arise from the meningothelial cell of the arachnoid
What is the difference between a schwannoma and a plexiform neurofibroma?
1) Schwannomas can be separated from the nerve; plexiform neurofibroma cannot
S-100 positive indicates?
Schwannoma
Where are Schwannomas most commonly found?
1) At the cerebellopontine angle; also known as acoustic neuroma
Tumor composed of regular cells and abundant granular chromatin; cells form gland-like round elonggated structures and perivascular pseudorosettes
Ependymoma
Glomeruloid body and pseudopalisading indicates?
Glioblatoma
What is the mutation observed in Neurofibromatosis type 1?
1) Autosomal dominant mutation of the NF1 gene located on chromosome 17
2) Results in the absence of neurofibromin and uninhibited RAS
Neurofibromas, gliomas of the optic nerve, pigmented nodules of the iris, and cutaneous hyper pigmented macules (Cafe Au Lait Spots)
Neurofibromatosis Type 1
Disease associated with bilateral acoustic neuromas and meningiomas, as well as ependyoma of the spinal cord
Neurofibromatosis Type 2
What is the cause of Neurofibromatosis type 2?
1) Autosomal dominant mutation of the NF2 gene on chromosome 22
2) Results in a mutated merlin protein
Disease associated with the development of hamartomas and benign neoplasms of the brain; Ash-Leaf patches, angiofibromas
Tuberous Sclerosis complex
What is the cause of Tuberous Sclerosis complex?
1) Autosomal dominant mutation of the of TSC1 (chromosome 9) or TSC2 (chromosome 16)
Disease characterized by hemangioblastomas and cysts involving the pancreas, liver, and kidneys
Von Hippel Lindau disease
What is increased in a primary glioblastoma?
Increased EGFR
What is increased in secondary glioblastoma?
Increase PDGF
Tumor found in the cerebellum of a child composed of bipolar cells with hair like processes. What should you suspect?
1) Pilocytic Astrocytoma
Well circumscribed mass with cysts, focal hemorrhage, and calcifications; consists of small uniform round cells with perinuclear halos
Oligodendroglioma
Single ring enhancing mass; may cross the corpus callosum (butterfly lesion); onset is rapid and survival is low
Glioblastoma
What mutations are commonly found in Ependymomas?
1) NF2 mutations - encodes Merlin protein (chromosome 22)
What is a cause of a Schwannoma?
1) Mutation in NF2
What mutation is commonly associated with neurofibromas?
1) NF1
Tumor present in the dermis and subcutaneous fat that is composed of spindle cells and highly collagenized stroma
Cutaneous Neurofibroma
Poorly defined tumor that frequently infiltrates along the axis of a parent nerve and invades the tissue
Malignant Peripheral Nerve Sheath Tumor
Tumor with an irregular ropey tangled mass of musculature
Malignant Peripheral Nerve Sheath tumor
Characterized by neurofibromas, gliomas of the optic nerve, pigmented nodules of the iris, and cafe au lait spots? What is the syndrome? What is the gene mutated? What is the protein it encodes?
1) Neurofibromatosis type 1
2) NF1 gene (chromosome 17)
3) Neurofibromin
What is the result of a mutation in NF1?
1) Neurofibromin becomes absent and results in the uninhibited RAS pathway
Syndrome characterized by bilateral acoustic neuromas and multiple meningiomas, may also have ependymoma. What is the syndrome? What is the gene mutated? What is the protein it encodes?
1) Neurofibromatosis Type 2
2) NF2 (chromosome 22)
3) Merlin protein
Syndrome characterized by the development of hamartomas and benign neoplasms of the brain; Ash-Leaf patches and angiofibromas are also present. What is the syndrome? What is the gene lost? What does the gene encode?
1) Tuberous Sclerosis Complex
2) Mutation in TSC-1 & 2
3) TSC-1 = hamartin; TSC-2 = tuberin
Syndrome characterized by hemangioblastomas and cysts involving the pancreas, liver, and kidneys; may also develop renal cell carcinoma or a pheochromocytoma. What is the syndrome? What gene is mutated?
1) Von Hippel Lindaue Disease
2) Mutation of VHL gene (chromosome 3)
Presence of round, eosinophilic laminar structures composed of dense calcifications with surrounding collagen
Meningioma
Congenital disorder with port-wine stains, typically in V1 opthalamic distribution; ipsilateral leptomeningeal angioma; pheochromocytomas; causes glaucoma, mental retardation, seizures
Sturge- Weber syndrome
Lisch nodule
Neurofibromatosis type I
Butterfly glioma
Glioblastoma
Infiltrating tumor with perinuclear halos and prominent delicate vasculature; “fried egg appearance”
Oligodendroglioma
Perivascular Pseudorossetes
Ependyoma
How do you differentiate between a medulloblastoma and a pilocytic astrocytoma?
1) Pilocytic astrocytoma has cystic spaces on CT; medulloblastoma is soley solid tumor
Von Recklinghausen’s disease
Neurofibromatosis type 1
What type of tumors are GFAP positive? Synaptophysin?
1) Astrocytoma, Oligodendroglioma, and ependymal
2) Neuroma
What causes opsoclonus- myoclonus syndrome (non-rhythmic eye movements with myoclonus)?
Neuroblastoma
Suprasellar mass consisting of solid tumor, cystic component, and a calcified component
Craniopharyngioma
Syndrome that predisoposes pt. to the early development of cancer? What mutation is present?
1) Li-Fraumeni syndrome
2) p53
