Artikel
Effects of integrated operative and medical management of vestibular schwannomas in adolescents and young adults with NF2 on tumour growth rates and long-term preservation of hearing quality
Ein integriertes Behandlungskonzept mit limitierter Chirurgie und medikamentöser Wachstumshemmung bei NF2-assoziierten Vestibularisschwannomen bei Jugendlichen und jungen Erwachsenen verringert die Tumorwachstumsraten und ermöglicht einen längerfristigen funktionellen Hörerhalt
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Autoren
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Martin Ulrich Schuhmann
- Universitätsklinikum Tübingen, Zentrum für Neurofibromatosen des Zentrums für Seltene Erkrankungen, Tübingen, Deutschland; Universitätsklinikum Tübingen, Klinik für Neurochirurgie, Bereich Pädiatrische Neurochirurgie, Tübingen, Deutschland
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Julian Zipfel
- Universitätsklinikum Tübingen, Zentrum für Neurofibromatosen des Zentrums für Seltene Erkrankungen, Tübingen, Deutschland; Universitätsklinikum Tübingen, Klinik für Neurochirurgie, Bereich Pädiatrische Neurochirurgie, Tübingen, Deutschland
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Lan Kluwe
- Universitätsklinikum Hamburg-Eppendorf, Internationales Neurofibromatose Zentrum, Hamburg, Deutschland; Universitätsklinikum Hamburg-Eppendorf, Klinik für Mund-Kiefer-Gesichtschirurgie, Hamburg, Deutschland
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Victor-Felix Mautner
- Universitätsklinikum Hamburg-Eppendorf, Internationales Neurofibromatose Zentrum, Hamburg, Deutschland; Universitätsklinikum Hamburg-Eppendorf, Klinik für Neurologie, Hamburg, Deutschland
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Marcos Tatagiba
- Universitätsklinikum Tübingen, Klinik für Neurochirurgie, Tübingen, Deutschland; Universitätsklinikum Tübingen, Zentrum für Neurofibromatosen des Zentrums für Seltene Erkrankungen, Tübingen, Deutschland
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Isabel Gugel
- Universitätsklinikum Tübingen, Klinik für Neurochirurgie, Tübingen, Deutschland; Universitätsklinikum Tübingen, Zentrum für Neurofibromatosen des Zentrums für Seltene Erkrankungen, Tübingen, Deutschland
| Veröffentlicht: | 4. Juni 2021 |
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Gliederung
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Objective: We reviewed our institutional experience in managing of NF2-associated vestibular schwannoma (NF2-VS) aiming at long-term hearing preservation in adolescents and young adults. Our focus was on effects of limited surgery and - if indicated - bevacizumab treatment in the further course of disease .
Methods: A total of 579 MRI based tumor volumetric measurements and hearing data sets were analyzed over time including all clinical follow-up data. The effect of surgery on tumor volume and growth rate was investigated in 46 tumors and on hearing function in 39 tumors. Long-term hearing follow-up behavior was compared with 20 non-operated ears in additional 15 patients. 16 operated NF2-VS were treated with bevacizumab in due course. Mutation analysis of the NF2 gene was performed in 25 patients.
Results: Limited surgery aiming at hearing preservation significantly slowed down NF2-VS growth rate. Factors associated with a higher growth rate were increasing patient age, tumor volume, and constitutional truncating mutations. Immediately after surgery, functional hearing was maintained in 82% of ears. Peri-operative deterioration of hearing was associated with initial hearing quality, larger tumor volumes, and larger resection amounts. Average hearing scores were initially better in the group of non-operated NF2-VS. Over time, hearing scores in both groups worsened with a similar dynamic. During bevacizumab treatment of residual tumors, four different patterns of growth were observed.
Conclusion: Decompression of the internal auditory canal with various degrees of tumor resection decreases postoperative tumor growth rates. Carefully tailored BAEP-guided surgery does not cause additional hearing deterioration. Secondary bevacizumab treatment showed heterogenous effects both regarding tumor size and hearing preservation. Overall, the integrated surgical and medical management of NF2-VS enables hearing preservation for years to enable these young patients in a vulnerable period of life to finish school and professional education with sufficient functional hearing in the majority of cases. In NF2-VS there is no place for initial surgery aiming at tumor removal.
