gms | German Medical Science

Objective: Vestibular schwannomas (VS) usually manifest between the 5th and 8th life decade and are rare tumor in children. Most pediatric cases are associated with Neurofibromatosis type 2, sporadic VS are even more rare in this age group. Few case series have been published with small patient numbers. Age at diagnosis is around 14-16 years and tumors tend to be larger than in adults. Some authors report on very large and highly vascularized tumors but due to small numbers, generalization of observations is difficult. We report on the largest case series of sporadic VS in children and discuss our observation with recent literature.

Methods: We included all patients between 2003 and 2021 who underwent surgery in our institution for a VS. 1635 patients were identified and of these, 28 patients who were younger than 21 years of age at the time of diagnosis with a sporadic tumor were included for further analysis. A retrospective review of all clinical parameters and surgical data as well as outcomes was performed. All procedures were performed via retrosigmoid approach. Preoperative MRIs and CT scans were assessed and tumor volumetry was performed. Institutional board approval was granted.

Results: Mean follow-up was 28 months, symptomatology was diverse. Most children presented with hearing loss and tinnitus. All cases with preoperatively performed repeated MRI scans showed volumetric growth between 1 and 18%/month (0.02cm3/month). Cystic tumor morphology and bone arosion is mostly seen with larger tumors. Gross total resection was possible in 78% and no recurrence was observed. All patients with subtotal resection showed tumor regrowth.

Conclusion: In conclusion, sporadic VS in children are very rare and present with a high clinical variability. Surgical resection is the primary therapy and is feasible with comparable results as in the adult age group Complication rate was low. Retrosigmoid craniotomy for VS in children is safe and feasible. GTR should be the goal of surgical intervention if feasible as STR does not lead to stable disease.

Figure 1 [Fig. 1]