Abstract
Gangliogliomas have traditionally been considered benign tumors, particularly in children. Following resection, the prognosis is thought to be excellent. We reviewed all cases of ganglioglioma at our institution over the preceding 10 years. Cases were identified by a review of the Neuro-Pathology records. Clinical and radiographic information was generated by a retrospective review of charts by the Dept. of Neuro-Oncology. 40 cases occurred in those age <18. Of these 48% were male. Age of onset peaked age 10-15. Similarly to adults, the majority of these were in the temporal lobe (or another cortical lobe). Pre-operative imaging showed the following characteristics: enhancing (70%), cystic (48%), calcification (30%). Gross total resection was achieved in 44%. Histology showed co-occurrence of cortical dysplasia in 7.5%. Survival information was available in 36 cases, out of which there were 3 deaths. These occurred at 1.3, 2.6 and 44.7 months following initial surgery. The only two cases where biopsy alone was performed died. In one, this appears to be due to location in the sella turcica; in the other due to the young age (age <1 year at diagnosis). No patients with gross total resection died. Two of the three also had features of juvenile pilocytic astrocytoma (JPA); only one other in the group had these features (chi-squared test p < 0.001). The one which was did not have features of JPA had features of anaplasia; only one other such case was present in the group (p = 0.03). All had a high p53 labeling index of >25% by immunohistochemistry (IHC); this was present in 3/9 cases checked in those who did not die (p < 0.05). Conclusion: While generally benign tumors, we suggest close surveillance in children with the following characteristics: biopsy alone undertaken, histological features of JPA or anaplasia and a high p53 by IHC.