Prognostic and Neurological Outcomes Following Surgery for Spinal NF2-Associated Ependymomas With Cysts

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.

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.

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.

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.