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68th Annual Meeting of the German Society of Neurosurgery (DGNC)
7th Joint Meeting with the British Neurosurgical Society (SBNS)

German Society of Neurosurgery (DGNC)

14 - 17 May 2017, Magdeburg

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Delayed malignant transformation of vestibular swannoma after stereotactic radiation

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  • Sebastian Simmermacher - Universitätsklinikum Halle-Wittenberg, Neurochirurgische Klinik, Halle (Saale), Deutschland
  • Dirk Vordermark - Universitätsklinikum Halle, Klinik und Poliklinik für Strahlentherapie, Halle/S., Deutschland
  • Thomas Kegel - Universitätsklinikum Halle-Wittenberg, Universitätsklinik und Poliklinik für Innere Medizin IV, Halle (Saale), Deutschland
  • Christian Strauss - Universitätsklinikum Halle/Saale, Klinik und Poliklinik für Neurochirurgie, Halle/Saale, Deutschland

Deutsche Gesellschaft für Neurochirurgie. Society of British Neurological Surgeons. 68. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), 7. Joint Meeting mit der Society of British Neurological Surgeons (SBNS). Magdeburg, 14.-17.05.2017. Düsseldorf: German Medical Science GMS Publishing House; 2017. DocP 054

doi: 10.3205/17dgnc617, urn:nbn:de:0183-17dgnc6170

Published: June 9, 2017

© 2017 Simmermacher et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.

Outline

Text

Objective: Casereport of delayed malignant transformation of a vestibular schwannoma 13 years after stereotactic radiation.

Methods: 13 years after stereotactic radiation of a left sided vestibular schwannoma (VS) (Koos II) a female patient showed acute clinical signs of increased intracranial pressure. Recurrence of VS was initially suspected as the cause of occlusive hydrocephalus.

Results: After stereotactic radiation in 2002 a stable disease was documented on regular MRI controls up to 2013. Since march 2015, the patient developed a left sided facial nerve paresis (HB°V) and vestibular dysfunction. MRI (04/2015) showed an increase in tumor size. A tumor resection was scheduled. The patient then rapidly developed signs of elevated intracranial pressure and brainstem herniation. cCT (06/2015) showed a dramatic increase in tumor size as compared to april 2015. Emergency tumor resection was necessary. Histopathology revealed a malignant peripheral nerve sheath tumor. The patient was scheduled for adjuvant therapy after 10 weeks of rehabilitation. MRI on readmission showed progressive residual tumor, necessitating a second surgery. Systemic chemotherapy (EVAIA protocol) in combination with radiation followed. The latest MRI follow up (08/2016) showed only residual contrast enhancement within the CPA and the petrosal bone, indicating stable disease.

Conclusion: Malignant transformation is an uncommon, but critical problem after radiation therapy. Several cases of malignant transformation of VS after radiotherapy have been reported and Seferis et al. (2014) calculated a 10 times elevated risk of malignant transformation of VS after radiation over a 20 year course. Although typical is a late incidence of the malignant transformation. Therefore and especially in younger patients who received stereotactic radiation of VS lifelong MRI follow up is necessary.