Gliomas and Brain Tumors
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Questions and Answers

What alteration is commonly observed in IDH-mutated tumors?

  • Co-deletion of 1p and 19q chromosomal segments
  • Overexpression of the EGF receptor
  • Loss of function mutations in ATRX (correct)
  • Promoter mutations for telomerase
  • Which enzyme activity is interfered with by the increased production of 2-hydroxyglutarate in IDH-mutated tumors?

  • Enzymes that regulate p53
  • Enzymes that regulate gene expression (correct)
  • Enzymes that suppress recombination events
  • Enzymes that regulate telomerase
  • What is a common genetic alteration found in oligodendrogliomas?

  • Mutations in RB
  • Promoter mutations for telomerase
  • Overexpression of the EGF receptor
  • Co-deletion of 1p and 19q chromosomal segments (correct)
  • Which brain region is most commonly affected by midline gliomas?

    <p>Pons</p> Signup and view all the answers

    What contributes to the immortalization of tumor cells in glioblastomas?

    <p>Mutations in the promoter for telomerase</p> Signup and view all the answers

    In IDH-mutated tumors, what is uncommonly observed compared to tumors with telomerase mutations?

    <p>Promoter mutations for telomerase</p> Signup and view all the answers

    What mechanism is involved in preserving telomere length in tumors with loss of function mutations in ATRX?

    <p>Alternative lengthening of telomeres</p> Signup and view all the answers

    Which system do ependymomas most often arise next to?

    <p>Nervous system</p> Signup and view all the answers

    Where are medulloblastomas located in children?

    <p>In the midline of the cerebellum</p> Signup and view all the answers

    What is the most common CNS neoplasm in immunosuppressed individuals?

    <p>Primary CNS lymphoma</p> Signup and view all the answers

    What percentage of intracranial tumors does Primary CNS lymphoma account for?

    <p>1%</p> Signup and view all the answers

    What virus are primary brain lymphomas nearly always positive for?

    <p>EpsteinBarr virus (EBV)</p> Signup and view all the answers

    At what age does the incidence of primary brain lymphoma increase in nonimmunosuppressed populations?

    <p>After 60 years of age</p> Signup and view all the answers

    What is the color and texture of a medulloblastoma as described in the text?

    <p>Gray and friable</p> Signup and view all the answers

    How does primary brain lymphoma respond to chemotherapy compared to peripheral lymphomas?

    <p>Poorer response</p> Signup and view all the answers

    What is a characteristic feature of patients with primary brain lymphoma according to the text?

    <p>&quot;Multiple tumor nodules within the brain parenchyma&quot;</p> Signup and view all the answers

    What is the main reason that even low-grade lesions in the brain can lead to serious deficits and poor prognosis?

    <p>Infiltration of large brain regions</p> Signup and view all the answers

    Which type of glioma accounts for about 80% of adult gliomas?

    <p>Astrocytomas</p> Signup and view all the answers

    What is the most common age group for astrocytomas to occur in adults?

    <p>Fourth to sixth decades</p> Signup and view all the answers

    Which of the following is a common symptom associated with diffuse astrocytomas?

    <p>Headaches</p> Signup and view all the answers

    Why do histologic patterns continue to guide treatment of gliomas despite molecular characterization?

    <p>Accuracy in predicting prognosis</p> Signup and view all the answers

    What can influence the outcome of a neoplasm apart from histologic classification?

    <p>Anatomic site</p> Signup and view all the answers

    Which type of glioma is classified based on morphologic resemblance to different types of glial cells?

    <p>Gliomas</p> Signup and view all the answers

    Why do even highly malignant gliomas rarely spread outside of the CNS?

    <p>'Seed and soil' hypothesis</p> Signup and view all the answers

    What is the principal neurologic manifestation of hemangioblastoma?

    <p>Highly vascular neoplasm</p> Signup and view all the answers

    Which part of the brain is hemangioblastoma most commonly found in?

    <p>Cerebellum</p> Signup and view all the answers

    What is the main association of hemangioblastoma in the brain?

    <p>Mural nodule</p> Signup and view all the answers

    Which feature is characteristic of the neuropathology of chronic traumatic encephalopathy?

    <p>Tauopathy</p> Signup and view all the answers

    What is the role of primary cilia in brain development according to the text?

    <p>Related to posterior fossa development</p> Signup and view all the answers

    Which condition is NOT mentioned as typically being affected by hemangioblastomas?

    <p>Hypothalamus</p> Signup and view all the answers

    In which publication was the role of primary cilia in brain development explored?

    <p>Neuron</p> Signup and view all the answers

    What type of disorder was mentioned in relation to disruptions in cortical development?

    <p>Genetic disorder</p> Signup and view all the answers

    What is the characteristic appearance of cortical hamartomas in tuberous sclerosis?

    <p>Firmer than normal cortex and potato-like</p> Signup and view all the answers

    Which proteins are disrupted in tuberous sclerosis resulting in increased mTOR activity?

    <p>TSC1 and TSC2</p> Signup and view all the answers

    What is the role of the TSC protein complex in cellular metabolism?

    <p>Negatively regulates mTOR activity</p> Signup and view all the answers

    Which lesions are extracerebral in tuberous sclerosis?

    <p>Renal angiomyolipomas and retinal glial hamartomas</p> Signup and view all the answers

    What is the dark color of tumor nodules in metastatic melanoma attributed to?

    <p>Presence of melanin</p> Signup and view all the answers

    Which cellular features are characteristic of cortical hamartomas in tuberous sclerosis?

    <p>Large vesicular nuclei with nucleoli</p> Signup and view all the answers

    What is the main disruption caused by loss of TSC1 or TSC2 proteins in tuberous sclerosis?

    <p>Upregulation of mTOR activity</p> Signup and view all the answers

    What is a characteristic cutaneous lesion associated with tuberous sclerosis?

    <p>Angiofibromas</p> Signup and view all the answers

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