The Use of 5-Aminolevulinic Acid to Assist Gross Total Resection of Paediatric Posterior Fossa Tumours

5-aminolevulinic acid (5-ALA) use is well established in the resection of adult high-grade gliomas. There is growing interest in its usefulness in the paediatric population. The potential benefit of 5-ALA-guided resection motivated our unit to offer the established adult protocol as off-label use.

to determine if 5-ALA guided resection was routinely useful and offered increased gross total resection (GTR) results.

Nineteen patients harbouring a posterior fossa tumour suggestive of either an ependymoma or medulloblastoma (MB) underwent surgery between January 2018 and October 2019. The mean age was 5 years (range 2-12 years). A dose of 20 mg/kg of 5-ALA (Gliolan®) was given 4 h preoperatively. Intraoperatively, the tumours were viewed under violet-blue light and the presence of fluorescence was recorded. Fluorescence status was compared with histopathological classification and grade, Ki-67 index, GTR rate, and a subjective determination of "usefulness" was determined.

The case series included ependymoma grade II (n = 6), ependymoma grade III (n = 4), and MB grade IV (n = 9). For the combined cohort, the strong fluorescence rate was 68% (n = 13), the heterogenous fluorescence rate was 26% (n = 5), and the completely negative fluorescence rate was 5% (n = 1). The strong fluorescence rate of 90% found in the combined ependymoma group compared to the 45% strong fluorescence rate in the MB group was statistically significant (p = 0.05). Within the MB group the Ki-67 index was found to be significantly higher in the strongly fluorescent group as opposed to the patchy or non-fluorescent group (77.5 vs. 40%, p = 0.016). Fluorescence was determined to be useful in 63% of all cases. There was no significant relationship between fluorescence and GTR. The relationship between perceived usefulness and resection was not statistically significant. No adverse drug reactions were recorded.

This case series adds to the growing body of evidence demonstrating the safety of 5-ALA in the paediatric population. 5-ALA guided resection was found to be useful in the majority of cases but this did not correlate with GTR status. Ependymomas reliably fluoresce in 90% of cases, and 5-ALA-guided resection should be considered when a preoperative diagnosis of ependymoma is suspected.