Updated: 10/27/2019

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 
  • 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
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"><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><span style="font-size: medium;">Epidemiology </span>
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    <li><span style="font-size: medium;">Pathophysiology </span>
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    <li><span style="font-size: medium;">Associated conditions </span>
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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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    <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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    <td><strong><span style="font-size: medium;">Differential</span></strong></td>
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    <td><strong><span style="font-size: medium;">Treatment</span></strong></td>
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    <td><strong><span style="font-size: medium;">Complications</span></strong></td>
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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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