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Original article
Long term follow up of 69 patients treated for optic pathway tumours before the chemotherapy era
  1. C Cappellia,
  2. J Grilla,
  3. M Raquina,
  4. A Pierre-Kahne,
  5. A Lellouch-Tubianaf,
  6. M-J Terrier-Lacombeb,
  7. J-L Habrandc,
  8. D Couanetd,
  9. R Braunerg,
  10. D Rodriguezh,
  11. O Hartmanna,
  12. C Kalifaa
  1. aDepartment of Pediatrics, Gustave Roussy Institute, 39 rue Camille Desmoulins, 94805 Villejuif, Cedex, France, bDepartment of Pathology, Gustave Roussy Institute, cDepartment of Radiation Therapy, Gustave Roussy Institute, dDepartment of Radiology, Gustave Roussy Institute, eDepartment of Neurosurgery, Hôpital Necker-Enfants Malades, 75015 Paris, France, fDepartment of Pathology, Hôpital Necker-Enfants Malades, gDepartment of Endocrinology, Hôpital Necker-Enfants Malades, hDepartment of Neuropediatrics, Hôpital Saint-Vincent de Paul, 75674 Paris Cedex 14, France
  1. Dr Grill. e-mail: jgrill{at}igr.fr

Abstract

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.

  • optic pathway tumours, neurofibromatosis type 1
  • radiotherapy
  • long term follow up

https://doi.org/10.1136/adc.79.4.334

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