Introduction: Central nervous system tumours account for 15- 25% of all childhood tumours and are the commonest solid tumour in childhood. About 30% of these are pilocytic astrocytomas (PA), commonly located in the posterior fossa. In children, suprasellar gliomas (involving optic pathway, hypothalamus or both) consist of 5% of CNS tumours. Tumours of the suprasellar region pose a challenge to surgical management and their behavior can be unpredictable although most literature indicates a slow course with very rare malignant transformation
Methods: We conducted a retrospective review of children diagnosed with suprasellar lesions from 1999- October 2012.
Patients were selected from our departmental database. We included those who had radiological diagnosis of suprasellar glioma or optic pathway lesion. We excluded those with a diagnosis of craniopharyngioma and those patients who had a histological diagnosis other than glioma. Clinical information was collected including neurofibromatosis 1 (NF-1) status, initial surgical management, initial treatment, progression and time to progression.
Results: 53 patients, with a median follow-up of 62 months were included. 54.7% fulfilled criteria for NF-1 and 45.3% did not. In the non NF-1 group, 15/24 had biopsy with histology of pilocytic astrocytoma in all but 1 who had Grade 2 astrocytoma.
In total, 15 were treated with chemotherapy or radiotherapy and 9 were observed. Only 1 patient had partial surgical resection. Progression occurred in 4/9 patients who were under observation with no biopsy and in 10/15 that had been biopsied. In the NF-1 group, 2 patients were biopsied, 2 had surgical resection in other institutions. 13 were treated with chemotherapy and 15 were observed with 1 having radiotherapy. Progression occurred in 7/29 with a further progression in 2 patients (1 chemotherapy, 1 observation).
Conclusions: Biopsy has a role to play in patients with sporadic tumours. Observation with treatment guided by radiological or ophthalmological progression remains the mainstay in NF-1 patients.
Patient Care: Comparing our practice with international practice in the management of these patients will help to refine our protocols to provide the best and current management for our patients
Learning Objectives: Assessing the trend in management of these lesion