Progression patterns of optic pathway tumours (OPT) need to be precisely defined for treatment planning. In patients with neurofibromatosis type 1 (NF1), this disease is usually indolent and the available literature rarely reports progression after the age of 6 years. In patients without NF1, the disease course seems to be less favourable. We reviewed the clinical and radiological files of 106 children referred to our institution for the treatment of a symptomatic OPT since 1980. NF1 was present in 51 of them. Progression patterns in children with NF1 differed markedly from those in the other patients. A total of 83 children had tumour extension beyond the chiasm (Dodge type III). Children with NF1 had progressive tumours later during follow-up (47% after the age of 6 years), had more often proptosis and infiltrating tumours but less frequently nystagmus or increased intracranial pressure. 32 children were not treated at diagnosis because they had only mild symptoms related to the OPT. In these patients, progression occurred more often in children without than with NF1 (12/12 versus 12/20 respectively, P = 0.04). A high number of patients needed treatment for progression or severe symptoms after 6 years of age. Of the patients, 33% needed treatment for progression or severe symptoms after 6 years of age.
Conclusion: Progression patterns of optic pathway tumours in children with neurofibromatosis type 1 differ markedly from those in other patients. This study emphasises the need for prolonged follow-up of children with optic pathway tumours, especially in neurofibromatosis type 1.