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Journal of Neuro-Oncology

, Volume 124, Issue 2, pp 229–236 | Cite as

Bevacizumab decreases vestibular schwannomas growth rate in children and teenagers with neurofibromatosis type 2

  • Audrey Hochart
  • Vianney Gaillard
  • Marc Baroncini
  • Nicolas André
  • Jean-Pierre Vannier
  • Matthieu Vinchon
  • Frederique Dubrulle
  • Jean-Paul Lejeune
  • Christophe Vincent
  • Véronique Nève
  • Héléne Sudour Bonnange
  • Nicolas Xavier Bonne
  • Pierre Leblond
Clinical Study

Abstract

Vestibular schwannoma (VS) growth in neurofibromatosis type 2 (NF2) can be responsible for brainstem compression and hearing loss. Surgical removal remains the standard therapy despite potential morbidity. Previous studies suggested that the inhibition of the VEGF-pathway with bevacizumab could result in hearing improvement, reduction of the tumor volume or both in adults. We retrospectively describe the French experience of bevacizumab treatment delivered for progressive VS in pediatric NF2 patients. Patients received Bevacizumab 5 or 10 mg/kg every 2 weeks according to the physician’s choice. Follow-up included clinical assessment, audiometry and volumetric MRI every 3–6 months. Seven patients harboring 11 VS were included. The median age at inclusion was 15 years (11.4–18.8), and the median treatment duration was 11.3 months (3.2–55.6). At baseline, the median tumor volume was 1.2 cm3 (0.52–13.5) and the median word recognition score was 90 % (0–100). We observed one major response, two minor responses and a decrease in the rate of tumor growth for the 4 other patients. The median annual growth rate before treatment was significantly higher than after 1 year of treatment (138 vs. 36 %, n = 5, p = 0.043). We noted one hearing improvement over the course of 1 year under treatment (hearing response rate was 14 %). Overall, the treatment was well tolerated. Our study supports that bevacizumab is an attractive therapeutic option for pediatric NF2 patients with growing VS. Thorough multidisciplinary evaluation is necessary to identify the best candidates prior to treatment. It is likely that a better functional outcome would be expected if targeted therapies were discussed early in the management of the disease.

Keywords

Neurofibromatosis type 2 Vestibular schwannoma Pediatric Bevacizumab 

Notes

Acknowledgments

This study was presented in part at the 16th International Symposium on Pediatric Neuro-Oncology in Singapore, June 2014. We thank Isabelle Villers, Cecile Girod and Sylvie Abed for their work with data collection.

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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  • Audrey Hochart
    • 1
  • Vianney Gaillard
    • 2
  • Marc Baroncini
    • 3
  • Nicolas André
    • 4
    • 5
  • Jean-Pierre Vannier
    • 6
  • Matthieu Vinchon
    • 3
  • Frederique Dubrulle
    • 2
  • Jean-Paul Lejeune
    • 3
  • Christophe Vincent
    • 7
  • Véronique Nève
    • 8
  • Héléne Sudour Bonnange
    • 1
  • Nicolas Xavier Bonne
    • 7
  • Pierre Leblond
    • 1
  1. 1.Pediatric Oncology UnitOscar Lambret CenterLilleFrance
  2. 2.Department of Radiology, C. Huriez HospitalLille University HospitalLilleFrance
  3. 3.Department of Pediatric NeurosurgeryLille University HospitalLilleFrance
  4. 4.Department of Pediatric Hematology and OncologyLa Timone hospitalMarseilleFrance
  5. 5.INSERM UMR 911, Centre de Recherche en Oncologie biologique et en OncopharmacologieAix-Marseille UnivMarseilleFrance
  6. 6.Department of Pediatric Hematology and OncologyCharles-Nicolle HospitalRouenFrance
  7. 7.Department of Otology and Neuro-otology, R. Salengro HospitalLille University HospitalLilleFrance
  8. 8.Pediatric Pulmonary Function Testing UnitLille University HospitalLilleFrance

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