Orphanet: Subependymal giant cell astrocytoma
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Subependymal giant cell astrocytoma

Disease definition

A rare low-grade astrocytoma characterized by a benign, slowly growing lesion typically arising in the wall of the lateral ventricles, composed of large ganglioid astrocytes. The tumor corresponds to WHO grade I and typically occurs during the first two decades of life in patients with tuberous sclerosis complex. Most patients present with worsening of epilepsy or symptoms of increased intracranial pressure.

ORPHA:251618

Classification level: Disorder
  • Synonym(s):
    • SEGA
  • Prevalence: -
  • Inheritance: -
  • Age of onset: -
  • ICD-10: D43.2
  • ICD-11: 2A00.0Y  XH1L48
  • OMIM: -
  • UMLS: C0205768
  • MeSH: -
  • GARD: 10632
  • MedDRA: -

Detailed information

Guidelines

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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