Arch Dis Child 1998;79:334-338 ( October )

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

C Cappelli,^a J Grill,^a M Raquin,^a A Pierre-Kahn,^e A Lellouch-Tubiana,^f M-J Terrier-Lacombe,^b J-L Habrand,^c D Couanet,^d R Brauner,^g D Rodriguez,^h O Hartmann,^a C Kalifa^a

^a Department of Pediatrics, Gustave Roussy Institute, 39 rue Camille Desmoulins, 94805 Villejuif, Cedex, France, ^b Department of Pathology, Gustave Roussy Institute, ^c Department of Radiation Therapy, Gustave Roussy Institute, ^d Department of Radiology, Gustave Roussy Institute, ^e Department of Neurosurgery, Hôpital Necker-Enfants Malades, 75015 Paris, France, ^f Department of Pathology, Hôpital Necker-Enfants Malades, ^g Department of Endocrinology, Hôpital Necker-Enfants Malades, ^h Department of Neuropediatrics, Hôpital Saint-Vincent de Paul, 75674 Paris Cedex 14, France

Correspondence to: Dr Grill. e-mail: jgrill{at}igr.fr

Accepted 8 May 1998

AIMTo analyse the long term results of conservative management with radiotherapy in patients with optic pathway tumours.
DESIGNAll 69 patients were symptomatic at diagnosis and most neoplasms involved the optic chiasm and hypothalamus.
RESULTSAt 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).
IMPLICATIONSRadiotherapy 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.

Keywords: optic pathway tumours, neurofibromatosis type 1; radiotherapy; long term follow up

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© 1998 by Archives of Disease in Childhood
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