History of Management of Supratentorial Pleomorphic Xanthoastrocytomas in Children

Understanding of Disease

  • 1979–first described: PXA was first described in 1979 as an astrocytic tumor, distinct in behavior, as compared to diffuse astrocytomas (1). A relatively favorable prognosis was shown in patients having PXA, with long-term survival, despite the tumor exhibiting significant histological pleomorphism and atypia.
  • 1993–term accepted by W.H.O.: The term PXA was added to the W.H.O. classification of tumors in 1993.

Technological Development

  • Imaging: The development of imaging and microsurgical techniques for all cerebral tumors has also improved the management of PXAs.
  • Immunological markers: The use of GFAP (glial fibrillary acidic protein) enabled the distinction of PXAs from other astrocytic tumors.

Surgical Technique

  • Appreciation of importance of gross total resection: In the past these tumors were managed as anaplastic astrocytic tumors (giant cell glioblastomas) or sarcomas. Appreciation of the diagnosis of PXA has changed the management to surgery as the treatment of choice, with primary gross total resection having an important role in outcome.