Oculomotor Schwannomas: A Systematic Review and Report of Two Pediatric Cases Treated with Fractionated Cyberknife Stereotactic Radiotherapy

Objective: Pediatric oculomotor nerve schwannomas are rare and challenging lesions due to the high morbidity associated with surgical intervention and their proximity to critical structures limiting the opportunity for stereotactic radiosurgery. We aim to report and review the novel use of fractionated Cyberknife (Accuray, Inc., Sunnyvale, California, USA) stereotactic radiotherapy in pediatric patients with oculomotor schwannomas. Methods: A systematic review of PubMed, Embase, and Cochrane was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Two patients, ages 8 and 10 years, with tumor volumes of 0.1 cm³ and 0.2 cm³, respectively, were treated with fractionated Cyberknife radiotherapy at our institution. A total dose of 45–50 Gy was administered over 25 fractions (1.8–2.0 Gy per fraction) to the 82%−84% isodose line. Serial magnetic resonance imaging was obtained for long-term follow-up (56–58 months). Results: We found 14 articles published between 1982 and 2018 that reported a total of 18 pediatric patients with intracranial oculomotor schwannomas. No previously described cases of pediatric intracranial oculomotor schwannomas were treated with radiation therapy. In both of our patients, radiographic tumor control was achieved at a mean follow-up of 57 months, with 1 patient displaying a decrease in tumor volume. Neither patient exhibited any worsening of their presenting symptoms, nor did either patient develop any new neurocognitive deficits following treatment. Conclusions: Fractionated Cyberknife radiotherapy is an effective and well-tolerated treatment option for intracranial oculomotor nerve schwannomas with excellent tumor control rates, similar to surgical and radiosurgical techniques, while sparing critical surrounding structures.