Epidemiology

  • Peak incidence: 1st decade
  • Most common malignant pediatric brain tumor
  • Associated conditions: Turcot syndrome

Etiology


Pathophysiology


Clinical features

  • most common location is the cerebellum → cerebellar defects (e.g., broad-based gait)
  • cerebellar vermis (midline) → truncal ataxia
  • Invasion or compression of the 4th ventricle → noncommunicating hydrocephalus → features of raised intracranial pressure (e.g., papilledema, vomiting, headache)
  • Drop metastases to the spinal cord are common → paraplegia

Diagnostics

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  • Synaptophysin ⊕
    • A neuron marker found in synaptic vesicles in the central nervous system and neuroendocrine cells. Used as a biomarker for tumors of neuroendocrine origin.

Treatment