Purpose: Low-grade gliomas are the most common paediatric brain tumours affecting 15 to 20% of the subjects with Neurofibromatosis type 1 (NF1). The NF1-related gliomas appear to have a different clinical behaviour compared to the sporadic cases.
Patients and methods: 60 patients (34 boys and 26 girls) with the median age of 4 years and low-grade glioma (42 sporadic cases and 18 cases with NF1) were treated. Thirty-nine patients (28 sporadic cases and 11 cases with NF1) underwent exclusive or post-surgical chemotherapy (with Vincristine and Carboplatin). The median follow-up was 5 years and 5 months.
Results: On brain MRI, tumour reduction was achieved in 12 of 28 patients (42.8%) among sporadic cases and in 9 of 11 patients (81.8%) among those with NF1, with a statistically significant difference between the two groups (p < 0.05). The response to therapy in both patient groups was not significantly influenced by gender, age, tumour site and histopathology, although the disease reduction occurred more frequently in children under 3 years of age.
Conclusions: Our study shows that paediatric patients with low-grade glioma and NF1 are more likely to respond to chemotherapy compared to non-NF1 patients.
Key words: children, Neurofibromatosis type 1, glioma