Stereotactic Radiosurgery for Vestibular Schwannoma in Neurofibromatosis Type 2: An International Multicenter Case Series of Response and Malignant Transformation Risk

Neurosurgery. 2023 May 1;92(5):934-944. doi: 10.1227/neu.0000000000002436. Epub 2023 Mar 2.

Abstract

Background: Vestibular schwannomas (VSs) related to neurofibromatosis type 2 (NF2) are challenging tumors. The increasing use of stereotactic radiosurgery (SRS) necessitates further investigations of its role and safety.

Objective: To evaluate tumor control, freedom from additional treatment (FFAT), serviceable hearing preservation, and radiation-related risks of patients with NF2 after SRS for VS.

Methods: We performed a retrospective study of 267 patients with NF2 (328 VSs) who underwent single-session SRS at 12 centers participating in the International Radiosurgery Research Foundation. The median patient age was 31 years (IQR, 21-45 years), and 52% were male.

Results: A total of 328 tumors underwent SRS during a median follow-up time of 59 months (IQR, 23-112 months). At 10 and 15 years, the tumor control rates were 77% (95% CI: 69%-84%) and 52% (95% CI: 40%-64%), respectively, and the FFAT rate were 85% (95% CI: 79%-90%) and 75% (95% CI: 65%-86%), respectively. At 5 and 10 years, the serviceable hearing preservation rates were 64% (95% CI: 55%-75%) and 35% (95% CI: 25%-54%), respectively. In the multivariate analysis, age (hazards ratio: 1.03 [95% CI: 1.01-1.05]; P = .02) and bilateral VSs (hazards ratio: 4.56 [95% CI: 1.05-19.78]; P = .04) were predictors for serviceable hearing loss. Neither radiation-induced tumors nor malignant transformation were encountered in this cohort.

Conclusion: Although the absolute volumetric tumor progression rate was 48% at 15 years, the rate of FFAT related to VS was 75% at 15 years after SRS. None of the patients with NF2-related VS developed a new radiation-related neoplasm or malignant transformation after SRS.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Cell Transformation, Neoplastic
  • Female
  • Follow-Up Studies
  • Hearing Loss* / surgery
  • Humans
  • Male
  • Middle Aged
  • Neurofibromatosis 2* / complications
  • Neurofibromatosis 2* / surgery
  • Neuroma, Acoustic* / complications
  • Neuroma, Acoustic* / radiotherapy
  • Neuroma, Acoustic* / surgery
  • Radiosurgery* / adverse effects
  • Retrospective Studies
  • Treatment Outcome
  • Young Adult