Abstract
OBJECTIVES: To investigate the clinical presentation, treatment breakdown, and overall survival (OS) outcomes of patients with neurofibromatosis type 2 (NF2)-associated bilateral vestibular schwannoma (NVS). METHODS: The 2004 to 2016 National Cancer Database was queried for patients with a diagnosis of VS. The "Laterality" code was used to stratify patients into sporadic unilateral vestibular schwannoma (UVS) and NVS. RESULTS: Of the 33,839 patients with VS, 155 (0.46%) were coded for NVS with an average age and tumor size of 37.4 ± 20.5 years and 23.5 ± 18.2 mm. Patients underwent observation (45.3%), surgery (29.3%), and radiotherapy (20.0%), and had a 5.8% 5-year mortality rate. Compared with UVS, NVS was negatively associated with receiving surgery (40.2% versus 29.3%, p = 0.02) while watchful observation was more prevalent (30.1% versus 45.3%, p = 0.001). In NVS, undergoing surgery was associated with larger tumor size (34.5 ± 21.2 versus 17.8 ± 13.3 mm, p = 0.001) and shorter diagnosis-to-treatment time (49.1 ± 60.6 versus 87.0 ± 78.5 d, p = 0.02), radiotherapy was associated with older age (44.4 ± 18.9 versus 35.2 ± 20.6 yr, p = 0.02) and longer diagnosis-to-treatment time (85.9 ± 77.9 versus 53.9 ± 65.5 d, p = 0.04), and observation was associated with smaller tumor size (17.8 ± 15.9 versus 28.0 ± 19.2 mm, p = 0.01). Kaplan-Meier log-rank analysis demonstrated similar 10-year OS between NVS and UVS patients (p = 0.58) without factoring the earlier age of presentation. Furthermore, there were no temporal changes in presentation/management of NVS, and OS was not dependent on the received treatment (p = 0.30). CONCLUSIONS: With younger age, larger tumors, and more conservative management, NVS's OS was not treatment-dependent and was similar to sporadic UVS, though the latter should not be interpreted as similar life expectancies due to the much earlier presentation.