Epidemiology of Supratentorial Pleomorphic Xanthoastrocytomas in Children

Incidence and Prevalence

  • Rare tumor: More than 200 cases have been described in the literature (2). PXAs comprise less than 1% of astrocytic tumors (3).

Age Distribution

  • Second and third decades: PXAs mainly occur in children and young adults, most commonly in the second and third decades of life. The median age at time of presentation is approximately 15 years (4, 5).

Sex Predilection

  • None (2)

Geographic Distribution

  • None

Risk Factors

  • None known

Relationships to Other Disease States and Syndromes

  • Neurofibromatosis: PXA has been observed in association with NF1 (6).
  • Cortical dysplasias: Associations with cortical dysplasia have been described (7, 8).