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Ependymal cyst in cerebello-pontine angle of fourth ventricle

Ependymal cyst in cerebello-pontine angle of fourth ventricle of a 3.5-year-old girl with several months of progressive apneic spells and vomiting. Excision was successful and she was asymptomatic after surgery. Immunocytochemical reactivities of (A) S-100β protein, X200 (B) vimentin, X250, and (C) glial fibrillary acidic protein (GFAP), X200, were all strongly positive in the simple cuboidal epithelial cells, typical of ependymal cysts. GFAP distinguishes ependymal cysts because it is not expressed in choroid plexus cyst epithelium. In addition, chronic parenchymal gliosis is seen at the base of the cyst. The convoluted appearance of the epithelium is artifactual due to intraoperative collapse of the cyst. (Contributed by Dr. Harvey Sarnat.)

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Associated Disorders

  • Aicardi syndrome
  • Callosal dysgenesis
  • Neuronal heterotopia
  • Orofaciodigital syndromes, especially types I and II
  • Polymicrogyria
  • Trisomy-18