Updated: 3/6/2022

Pilocytic Astrocytoma

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  • Snapshot
    • A 7-year-old boy presents to the pediatric emergency department for lethargy, nausea, and vomiting. Medical history is unremarkable. Physical examination is notable for papilledema and right-sided dysmetria. An MRI brain with and without contrast demonstrates a cerebellar cystic mass.
  • Introduction
    • Overview
      • a slow growing central nervous system (CNS) tumor that arise from astrocytes
        • most frequently arises in the cerebellum but can appear anywhere else in the CNS
  • Epidemiology
    • Incidence
      • most common glial tumor in children
  • ETIOLOGY
    • Pathophysiology
      • exact mechanism unknown but may be due to disrupted cell cycle regulation, leading to abnormal cellular proliferation
    • Associated conditions
      • neurofibromatosis type 1
  • Presentation
    • Symptoms
      • symptoms of increased intracranial pressure
        • headache
        • nausea
        • vomiting
        • lethargy
    • Physical exam
      • papilledema
      • ataxia (in cerebellar tumors)
      • visual deficits (in optic pathway tumors)
  • Imaging
    • MRI brain with and without contrast
      • indication
        • imaging study of choice
      • findings
        • cystic mass
          • cyst wall enhances in ~50% of cases
          • often has a brightly enhancing mural nodule
  • Studies
    • Histopathology
      • indication
        • confirms the diagnosis
          • obtained via tumor resection or biopsy
        • prognosis
          • degree of atypia (tumor grade) is the best prognostic indicator
      • findings
        • GFAP+
        • Rosenthal fibers
          • eosinophilic corkscrew fibers
  • Differential
    • Other malignant brain neoplasm
      • differentiating factors
        • based on histological findings
          • e.g., glioblastomas will have pleomorphic tumor cells surrounding a central area of necrosis
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      <td height="12" bgcolor="#F4F4F4"><strong>Snapshot</strong></td>
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      <li><span style="color: #0000cc;"><span style="font-size: medium;">A</span></span></li>
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      <td height="12" bgcolor="#F4F4F4"><span style="color: #ffffff;"><span style="font-family: Arial, Helvetica, sans-serif; color: #000000; font-size: xx-small;"><span style="font-size: medium;"><strong><span style="font-size: medium;">Introduction</span></strong></span></span></span></td>
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      <li><span style="font-size: medium;">Definition </span>
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      <li><span style="font-size: medium;">a </span>
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      </li>
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      <li><span style="font-size: medium;">Epidemiology </span>
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      <li><span style="font-size: medium;">i</span></li>
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      <li><span style="font-size: medium;">Pathophysiology </span>
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      <li><span style="font-size: medium;">t</span></li>
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      <li><span style="font-size: medium;">Associated conditions </span>
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      <li><span style="font-size: medium;">a</span></li>
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      <td height="12" bgcolor="#F4F4F4"><span style="font-family: Arial, Helvetica, sans-serif; color: #ffffff; font-size: xx-small;"><span style="color: #000000;"><span style="font-size: medium;"><strong><span style="font-size: medium;">Presentation</span></strong></span></span></span></td>
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      <ul>
      <li><span style="font-size: medium;">Symptoms</span></li>
      <li><span style="font-size: medium;">Physical exam</span></li>
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      <td><strong><span style="font-size: medium;">Imaging</span></strong></td>
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      <li><span style="font-size: medium;"><span style="color: #000000;"><span style="font-size: medium;">M</span></span></span></li>
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      <td><strong><span style="font-size: medium;">Studies</span></strong></td>
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      <li><span style="font-size: medium;">A</span></li>
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      <td><strong><span style="font-size: medium;">Differential</span></strong></td>
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      <li><span style="font-size: medium;">N</span></li>
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      <td><strong><span style="font-size: medium;">Treatment</span></strong></td>
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      <li><span style="font-size: medium;">M</span></li>
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      <td><strong><span style="font-size: medium;">Complications</span></strong></td>
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      <li><span style="font-size: medium;">D<br /></span></li>
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      • differentiating factors
        • based on histological findings
          • e.g., glioblastomas will have pleomorphic tumor cells surrounding a central area of necrosis
    • differentiating factors
      • based on histological findings
        • e.g., glioblastomas will have pleomorphic tumor cells surrounding a central area of necrosis
  • Treatment
    • Surgical
      • resection
        • indication
          • first line in accessible tumors
            • chemotherapy and/or radiation therapy is considered when the tumor is inoperable
  • Complications
    • Leptomeningeal disease
      • spread of the CNS tumor to the leptomeninges (pia and arachnoid mater)

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