The H3 K27-altered, Extent of Resection and Survival in Pediatric High-grade Spinal Cord Gliomas

Purpose: Pediatric high-grade spinal cord gliomas (pHGSG) are classified into two prognostically meaningful subtypes. The purpose of our study is to describe clinical characteristics and management of two pHGSG subtypes, especially focus on the effect of surgical resection in these two subtypes. Methods We described the clinical characteristics and management and analyzed the prognostic factors of 19 pHGSGs. Based on H3 K27-altered and isocitrate dehydrogenase (IDH) mutation status, pHGSGs were classified into H3 K27-altered and H3/IDH-wildtype pHGSG for assessing effect of surgical resection. Results Nineteen patients (11 males and 8 females) with a mean age of 12.7 ± 4.7 years were included in the analysis. The median survival time of all patients was 14 months. Thirteen pHGSG patients had H3 K27-altered tumors, and 6 had H3/IDH-wildtype tumors. All patients received therapeutic surgical resection, 10 (55.6%) patients underwent gross total resection (GTR) and 8 (33.3%) patients underwent subtotal resection (STR). pHGSG patients with H3 K27-altered underwent GTR (HR 0.311, 95% CI 0.075–1.289, p = 0.091) had tended to increase overall survival. For patients of H3/IDH-wildtype pHGSG, there was no survival advantage associated with GTR (p = 0.157). Conclusions The extent of surgical resection has the different effects on survival in H3 K27-altered and H3/IDH-wildtype pHGSGs. The patients with H3 K27-altered pHGSG have tended to benefit from a greater extent of surgical resection. Nevertheless the benefit was not evident in the patients with H3/IDH-wildtype pHGSG.