Long-term follow up of 69 patients treated for optic pathway tumours before the chemotherapy era

Arch Dis Child. 1998 Oct;79(4):334-8. doi: 10.1136/adc.79.4.334.


Aim: To analyse the long-term results of conservative management with radiotherapy in patients with optic pathway tumours.

Design: All 69 patients were symptomatic at diagnosis and most neoplasms involved the optic chiasm and hypothalamus.

Results: At 10 years, overall survival and progression free survival were 83% and 65.5%, respectively. After radiotherapy, vision improved in 18 patients and remained stable in 29 other patients. Cerebrovascular complications occurred in nine of 53 patients treated with radiotherapy after a median interval of two and a half years. These complications were five times more frequent in patients with neurofibromatosis type 1 (NF1). Severe intellectual disabilities were present in 18 children, most of whom underwent irradiation at a very young age (median age, 4 years).

Implications: Radiotherapy is a valuable treatment in terms of tumour response, visual outcome, and progression free survival. However, in young children and in patients with NF1, major sequelae are encountered and new treatment strategies should be proposed for these patients.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cranial Nerve Neoplasms / mortality
  • Cranial Nerve Neoplasms / radiotherapy*
  • Follow-Up Studies
  • Humans
  • Hypothalamic Neoplasms / mortality
  • Hypothalamic Neoplasms / radiotherapy*
  • Infant
  • Neurofibromatosis 1 / mortality
  • Neurofibromatosis 1 / radiotherapy*
  • Optic Chiasm*
  • Optic Nerve Neoplasms / mortality
  • Optic Nerve Neoplasms / radiotherapy
  • Survival Rate
  • Treatment Outcome