Clinical features, management and outcomes of paediatric central nervous system tumours disseminated and nondisseminated at presentation: a fourteen-year experience

BACKGROUND: It is unusual for paediatric central nervous system (CNS) tumours to be disseminated at presentation, and literature on the clinical features, management and outcomes of this specific group is scarce. Surgical... [ view full abstract ]

BACKGROUND: It is unusual for paediatric central nervous system (CNS) tumours to be disseminated at presentation, and literature on the clinical features, management and outcomes of this specific group is scarce. Surgical management decisions can be challenging, particularly in the presence of hydrocephalus. We present our experience in managing paediatric CNS tumours disseminated and nondisseminated at presentation over fourteen years. METHODS: Retrospective review of prospectively-collected data on children presenting to our tertiary centre between 2003 and 2016 inclusive. RESULTS: We identified 53 patients with CNS tumour dissemination at presentation (M:F=34:19, median age=3.8 years, range=7 days-15.6 years) and 308 without dissemination (M:F=161:147, median age=5.8 years, range=1 day-16.9 years). Five tumour groups were studied: medulloblastoma (disseminated n=29/nondisseminated n=74), other primitive neuroectodermal tumour (n=8/n=17), atypical teratoid rhabdoid tumour (n=8/n=22), pilocytic astrocytoma (n=6/n=138), and ependymoma (n=2/n=57). Median follow-up in survivors was not significantly different between those with (64.0 months, range=5.2-152.0 months) and those without disease dissemination at presentation (74.5 months, range=4.7-170.1 months; P>0.05). Dissemination status at presentation significantly impacted survival, risk of recurrence, rates of complete resection of the operated lesion, chance of proceeding to palliative care as an early management strategy, surgical complication rate, and risk of requiring CSF diversion (all Ps<0.05). Differences between the five tumour groups were evident. No factors to predict the need for permanent CSF diversion following temporary external ventricular drainage were identified on multivariate analysis, and there was no clear superiority of either ventriculoperitoneal shunt or endoscopic third ventriculostomy as a permanent CSF diversion procedure. CONCLUSIONS: Tumour type and dissemination status at initial presentation significantly affect outcomes across a range of measures. The management of hydrocephalus in patients with CNS tumours is challenging, and further prospective studies are required to identify the optimal CSF diversion strategy or strategies in this population.