Background/Aim: This study aimed to examine surgical outcomes of spinal ependymomas in NF2-related schwannomatosis, focusing on tumor and cyst reduction, neurological function, and possible predictive factors. Patients and Methods: Fifteen patients with symptomatic, asymptomatic, and/or progressive spinal ependymomas and associated cysts were retrospectively evaluated, with tumor and cyst volumes and neurological function (Klekamp-Samii Score) assessed. Results: Over a mean follow-up of 20±20 months before and 57±43 months after surgery, 932 volumetric measurements were performed. Surgery significantly reduced tumor and cyst size (mean resection rates: tumors, 89%; cysts, 67%), with cyst volume continuing to decrease throughout the postoperative course and correlating with neurological status at 12 months (r=0.617, p=0.025). Patients with non-truncating NF2 mutations achieved higher mean resection rates for tumors (100%) and cysts (93%) than those with truncating mutations (84% and 70%, respectively). Neurological function showed variability: 60% of patients experienced immediate postoperative decline, but 80% improved or stabilized within 3-6 months. No other predictors for outcome were found. Conclusion: Given the complexity, surgery of spinal NF2-associated ependymomas with cysts should be performed in specialized centers, and careful consideration is needed in neurologically intact patients due to the lack of reliable outcome predictors.
Grimm et al. (Wed,) studied this question.