Proton Radiotherapy for Vestibular Schwannomas in Patients with NF2-Related Schwannomatosis: A Case Series

Jules P.J. Douwes*, Kimberley S. Koetsier, Victor S. van Dam, Scott R. Plotkin, Frederick G. Barker, D. Bradley Welling, Jeroen C. Jansen, Erik F. Hensen, Helen A. Shih

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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(1) Background: This study aimed to evaluate the efficacy and treatment-related toxicity of proton radiotherapy (PRT) for vestibular schwannoma (VS) in patients with neurofibromatosis type 2-related schwannomatosis (NF2). (2) Methods: Consecutive NF2 patients treated with PRT for VS between 2004 and 2016 were retrospectively evaluated, focusing on tumor volume, facial and trigeminal nerve function, hearing, tinnitus, vestibular symptoms, and the need for salvage therapy after PRT. (3) Results: Eight patients were included (median age 36 years, 50% female). Median follow-up was 71 months. Five (63%) patients received fractionated PRT and three (38%) received PRT radiosurgery for VS. Six patients (75%) received prior VS surgery; three also received bevacizumab. Six patients (75%) did not require salvage therapy after PRT. Two patients (25%) with residual hearing lost it after PRT, and six had already lost ipsilateral hearing prior to PRT. Tumor and treatment-related morbidity could be evaluated in six patients. Following PRT, conditions that occurred or worsened were: facial paresis in five (83%), trigeminal hypoesthesia in two (33%), tinnitus in two (33%), and vestibular symptoms in four patients (67%). (4) Conclusion: After PRT for VS, the majority of the NF2 patients in the cohort did not require additional therapy. Tumor and/or treatment-related cranial nerve deficits were common. This is at least partly explained by the use of PRT as a salvage treatment after surgery or bevacizumab, in the majority of cases. There remains the further opportunity to elucidate the efficacy and toxicity of proton radiotherapy as a primary treatment.

Original languageEnglish
Pages (from-to)3473-3483
Number of pages11
JournalCurrent Oncology
Issue number3
Publication statusPublished - 20 Mar 2023

Bibliographical note

Funding Information:
K.S.K. receives salary partly funded by (1) the Surcharge for Top Consortia for Knowledge and Innovation (TKIs) from the Ministry of Economic Affairs and Climate and the HollandPTC consortium—Erasmus MC, Rotterdam; Holland Proton Therapy Center, Delft; Leiden University Medical Center, Leiden; Delft University of Technology, Delft, the Netherlands, and (2) Varian Medical System (the principal investigator of this research grant is E.F.H.).

Publisher Copyright: © 2023 by the authors.


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