Management and outcome of pediatric brainstem and cerebellar peduncular low-grade gliomas: a retrospective analysis of 62 cases.

Abstract

This study was designed to present our experience and recommendations regarding the management of pediatric brainstem and peduncular low-grade gliomas (LGGs). Retrospective analysis was performed for pathologically proven brainstem or cerebellar peduncular LGGs in patients admitted between 2014 and 2019. These lesions were classified into the dorsal exophytic, focal brainstem, cervicomedullary, lower peduncular, and upper peduncular groups, and this classification was the basis for the surgical approach for the lesions. Sixty-two pediatric patients were included, and their distribution among the aforementioned groups were as follows: 12, 12, 3, 16, and 19 cases in the dorsal exophytic, focal brainstem, cervicomedullary, upper peduncular, and lower peduncular groups, respectively. Stereotactic biopsy was performed for all cases in the focal brainstem group, whereas other groups underwent open excision. Gross total resection (GTR) was achieved in 20 cases (40%), near-total resection (NTR) was achieved in 17 cases (34%), and subtotal resection (STR) was achieved in 13 cases (26%). The extent of GTR and NTR for the upper peduncular, lower peduncular, dorsal exophytic, and cervicomedullary groups were 81.2%, 68.4%,75%, and 66.6%, respectively. Then, 32 cases received chemotherapy. The 3- and 5-year progression-free survival rates were 95% (95% confidence interval (CI) 89.5-100%) and 90.3% (95% CI 79.9-100%), respectively. A significant difference in the 3-year progression-free survival rate was observed between the GTR and NTR groups (p = 0.06; 100% vs. 88.2% (95% CI 72.9-100%)). Surgery plays a definitive curative role in grossly resected cases. Additionally, the role of surgical debulking should be considered, even if GTR is impossible. Meanwhile, chemotherapy showed a beneficial role in patients with focal brainstem lesions and progressive lesions, those with STR, and some patients with NTR.