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22 Cards in this Set

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What kind of tumor is pictured? What are these types of cells called?
Retinoblastoma
Flexner-Wintersteiner Rosettes
What kind of tumor is shown? What is depicted?
• Retinoblastoma
• Ki67-immunostain showing a high proliferative index
What type of tumor is pictured? What characteristic is depicted?
Astrocytoma
• with Intermediate Filaments that contain glial fibrillary acid protein.
• AD inheritance
• Mutations on 17q
• What does 17q encode?
• Von Recklinghousen Neurofibramatosis (VNRF) or Neurofibramatosis Type I (NF-1)
• Chromosome 17q encodes neurofibromin, a tumor suppressor
• AD inheritance
• Results in bilateral acoustic & spinal schwannomas
• Bilateral Acoustic Neurofibramatosis (BANF) or Neurofibramatosis Type II (NF-2)
• AD inheritance
• Characteristics: mental retardation, seizures, subependymal giant cell astrocytomas, cortical hamartomas (tubers) of the CNS, tumors & hamartomas of other organs
• Mutations on 9q & 16p (which encode?)
• Tuberous Sclerosis
• TS1 on 9q encodes: hamartin
• TS2 on 16p encodes Tuberin
• Tuberin is a tumor suppressor
• AD inheritance
• Associated with: hemangioblastomas of the retina & cerebellum, cysts of the liver & pancreas, pheochromocytomas, kidney tumors, pancreatic tumors
• Associated w/ what mutation/gene product?
• What does this gene product do?
• Von Hipple Lindau disease
• Linked to VHL a tumor suppressor gene on 3p
• pVHL blocks elongation during mRNA transcription
• pVHL inhibits vascular endothelial growth factor (VEGF)
• Genetic tumor
• TP53 mutation
Low grade & anaplastic astrocytoma
• Genetic tumor
• Loss of heterozygosity (LOH) 10q
• EGFR amplification
• P16INK4a mutation
• TP53 mutation
• PTEN mutation
Glioblastoma
• Genetic tumor
• Loss of heterozygosity (LOH) 17p
Medulloblastoma
• Genetic tumor
• Loss of 22q
Atypical Teratoid-rhabdoid tumor OR Meningioma
• Genetic tumor
• Loss of 22q and mutations of the NF2 gene
Schwannoma
What are the best known tumor suppressor genes? What do they do?
• RB (retinoblastoma gene) on 13q14 (regulates G1/S phase in the cell cycle)
• p53 gene on 17p (induces apoptosis through regulating bcl2 and bax)
What gene regulates the G1/S phase in the cell cycle? What chromosome is it on?
RB (retinoblastoma gene) on 13q14.
What gene regulates apoptosis through regulating bcl1 and bax? What chromosome is it on?
p53 on 17p
What two types of tumors occur predominantly in children?
Cerebellar medullablastoma & adrenal neuroblastoma
A pilocytic astrocytoma w/ benign cytological features is a grade ___ astrocytoma?
Grade I astrocytoma
An astrocytoma with moderate cellularity, no anaplasia or mitotic activity is a grade ___ ?
Grade 2 astrocytoma
An astrocytoma with increased cellularity, nuclear atypia, and mitoses is grade ___?
Grade 3 astrocytoma
An astrocytoma with increased cellularity, nuclear atypia, mitoses, vascular endothelial proliferation and necrosis is grade ___?
Grade 4 astrocytoma
What astrocytomas most commonly affect children? What part of the brain do they tend to affect? What is a unique characteristic?
• Pilocytic astrocytoma
• Tend to appear in the cerebellum
• Have cystic mural enhancement
What type of tumors account for 80% of adult 1º brain tumors? Usually occur in the 4th-6th decades and occur in the cerebral hemispheres?
Fibrillary astrocytomas
Symptoms depend on location, survival depends on differentiation.