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70. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
Joint Meeting mit der Skandinavischen Gesellschaft für Neurochirurgie

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

12.05. - 15.05.2019, Würzburg

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Management of NF2-associated vestibular schwannomas in children and young adults – influence of surgery and clinical factors on tumour volume and growth rate

Management von NF2-assoziierten Vestibularisschwannomen bei Kindern, Jugendlichen und jungen Erwachsenen – Einfluss der Chirurgie auf das Tumorvolumen und die Wachstumsrate

Meeting Abstract

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  • presenting/speaker Isabel Gugel - Universität Tübingen, Neurochirurgie, Tübingen, Deutschland
  • Florian Grimm - Universität Tübingen, Neurochirurgie, Tübingen, Deutschland
  • Christian Teuber - Universität Tübingen, Neurochirurgie, Tübingen, Deutschland
  • Victor-Felix Mautner - Universitätsklinikum Hamburg-Eppendorf, Neurologie, Hamburg, Deutschland
  • Lan Kluwe - Universitätsklinikum Hamburg-Eppendorf, Neurologie, Hamburg, Deutschland
  • Marcos Tatagiba - Universität Tübingen, Neurochirurgie, Tübingen, Deutschland
  • Martin Schuhmann - Universität Tübingen, Neurochirurgie, Tübingen, Deutschland

Deutsche Gesellschaft für Neurochirurgie. 70. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit der Skandinavischen Gesellschaft für Neurochirurgie. Würzburg, 12.-15.05.2019. Düsseldorf: German Medical Science GMS Publishing House; 2019. DocV313

doi: 10.3205/19dgnc331, urn:nbn:de:0183-19dgnc3311

Veröffentlicht: 8. Mai 2019

© 2019 Gugel et al.
Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). Lizenz-Angaben siehe http://creativecommons.org/licenses/by/4.0/.

Gliederung

Text

Objective: The present study evaluates tumor volume and growth rate of neurofibromatosis type 2 (NF2)-associated vestibular schwannomas (VSs) as well as clinical factors or type of mutations before and after surgery in children and young adults under the age of 25 at time of diagnosis.

Methods: For a total of 46 operated tumors (28 NF2 patients), 579 volumetrical measurements were carried out using thin slices (<3mm) of T1-weighted contrast-enhanced magnetic resonance imaging. The follow-up period was 21 to 167 months (mean 75 months). Growth rate was calculated using a multilinear regression model. Mutation analysis of the NF2 gene was performed in 25 patients.

Results: Surgery significantly (p=0.013) slowed down VS growth rate from 0.69±1.30 cm3/year to 0.23±0.42 cm3/year. Factors significantly associated with higher growth rate of VSs were: patients’ age (p<0.0005), tumor volume (p<0.05) and size (p=0.007), presence of spinal ependymomas (p=0.037), right-sided location (p=0.025) and constitutional truncating mutations in the NF2 gene (p=0.019). By contrast, the presence of peripheral schwannomas was significantly associated with lower growth rate of VSs (p=0.022).

Conclusion: Decompression of the internal auditory canal with various degrees of tumor resection decreases postoperative tumor growth rate in children and young adults with NF2-associated VS. Patients with potential risk factors for accelerated growth (e. g. spinal ependymomas, truncating mutations) and with increasing age should be monitored more closely, also before and after surgery.