Background: Isolated trigeminal schwannomas occur in 0.07% to 0.3% of intracranial tumors and account for 0.8% to 8% of intracranial schwannomas and 1/3 of Meckel cave tumors. The presence of multisegmental schwannoma is rare, resulting in a limited understanding of its optimal management.
Objective: To describe potential surgical options to manage this rare entity.
Methods: A 2-institution retrospective review of all patients with pathologically confirmed trigeminal schwannoma managed with resection from January 2009 through January 2019 was conducted. A manual chart review was performed to verify patients' inclusion and collect data about age, sex, tumor size, tumor site, treatment modality, surgical approach, complications, and follow-up duration and status.
Results: A total of 4 patients (age range 12-50 yr) who underwent a variety of cranial and orbitocranial approaches for tumor resection were identified. Patients achieved good outcomes with improvement of visual outcomes. One case of infection and 1 case of partial tumor recurrence requiring reresection were identified.
Conclusion: Multisegmental trigeminal schwannoma is a rare and unique entity, often associated with trigeminal schwannomatosis. Interdisciplinary management has been shown to be the most effective method for improving patient outcomes with these complex and poorly understood diseases.
Keywords: NF2; SMARCB1; Schwannoma; Schwannomatosis; Segmental; Trigeminal nerve.
Copyright © 2020 by the Congress of Neurological Surgeons.