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 (A)</p> Signup and view all the answers

What contributes to the immortalization of tumor cells in glioblastomas?

<p>Mutations in the promoter for telomerase (A)</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 (C)</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 (D)</p> Signup and view all the answers

Which system do ependymomas most often arise next to?

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

Where are medulloblastomas located in children?

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

What is the most common CNS neoplasm in immunosuppressed individuals?

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

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

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

What virus are primary brain lymphomas nearly always positive for?

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

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

<p>Poorer response (D)</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; (D)</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 (B)</p> Signup and view all the answers

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

<p>Astrocytomas (D)</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 (B)</p> Signup and view all the answers

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

<p>Headaches (B)</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 (C)</p> Signup and view all the answers

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

<p>Anatomic site (C)</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 (A)</p> Signup and view all the answers

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

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

What is the principal neurologic manifestation of hemangioblastoma?

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

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

<p>Cerebellum (B)</p> Signup and view all the answers

What is the main association of hemangioblastoma in the brain?

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

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

<p>Tauopathy (B)</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 (A)</p> Signup and view all the answers

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

<p>Hypothalamus (B)</p> Signup and view all the answers

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

<p>Neuron (B)</p> Signup and view all the answers

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

<p>Genetic disorder (D)</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 (B)</p> Signup and view all the answers

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

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

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

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

Which lesions are extracerebral in tuberous sclerosis?

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

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

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

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

<p>Large vesicular nuclei with nucleoli (C)</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 (D)</p> Signup and view all the answers

What is a characteristic cutaneous lesion associated with tuberous sclerosis?

<p>Angiofibromas (A)</p> Signup and view all the answers

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