LINC-27. PAEDIATRIC SUPRASELLAR TUMOURS: CLINICAL EXPERIENCE FROM A SINGLE TERTIARY CENTRE IN KUALA LUMPUR, MALAYSIA

INTRODUCTION: The outcome of suprasellar tumours in children varies with the diagnosis and morbidity is significant. We herein report the clinical features of children with suprasellar tumours treated at our centre. METHODS: Clinical data were collected by retrospective review from January 2000 to December 2019. The patients were identified from the paediatric haematology and oncology unit registry. RESULTS: There was a total of 103 children with brain tumour and suprasellar tumours comprise of 14.6% (n=15). Median age at presentation was 7 years old. Male to female ratio was 3:2. Majority of cases was low grade glioma, 40% (n=6) and germ cell tumour(GCT) 33.3% (n=5) followed by craniopharyngioma, 13.3% (n=2) and Rathke cleft cyst, 6.7% (n=1). All patients had tissue diagnosis except one with secreting GCT and one with unsatisfactory tissue sample. Mean duration of follow up was 7.4 years. One patient with germinoma was lost to follow-up after radiotherapy. Three out of 13 (23%) patients died; 2 with GCT from disease progression; 1 craniopharyngioma after 11 years of unknown cause. All survivors have significant morbidity; 70% have moderate to severe visual impairment, 90% have at least two pituitary hormones deficiency, 20% have neurological deficit and 1 was surgically related. Two boys have precocious puberty not related to disease progression. Two with GCT with diabetes insipidus had history of thromboembolism (stroke and pulmonary embolism). CONCLUSIONS: Suprasellar tumours in children at our centre pose a significant long-term complications and multidisciplinary team management and follow up is required to improve the morbidity.