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J Neurosurg Spine. 2017 Apr;26(4):474-482. doi: 10.3171/2016.8.SPINE16589. Epub 2016 Dec 16.

The response of spinal cord ependymomas to bevacizumab in patients with neurofibromatosis Type 2.

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Nuffield Department of Neurosciences and NF2 Unit.
University of New South Wales, Sydney, Australia.
Department of Neurology, Guy's & St Thomas' Hospital, London.
Genomic Medicine, Institute of Human Development, MAHSC, University of Manchester, St Mary's Hospital, Manchester.
Department of Clinical Neurosciences, Addenbrooke's Hospital, Cambridge.
Centre for Paediatric, Teenage, and Young Adult Cancer, Institute of Cancer Sciences, University of Manchester, United Kingdom; and.
Department of Genetics and NF2 Unit.
Department of Neuroradiology, The West Wing, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford.


OBJECTIVE People with neurofibromatosis Type 2 (NF2) have a genetic predisposition to nervous system tumors. NF2-associated schwannomas stabilize or decrease in size in over half of the patients while they are receiving bevacizumab. NF2 patients treated with bevacizumab for rapidly growing schwannoma were retrospectively reviewed with regard to ependymoma prevalence and response to treatment. METHODS The records of 95 NF2 patients receiving bevacizumab were retrospectively reviewed with regard to spinal ependymoma prevalence and behavior. The maximum longitudinal extent (MLE) of the ependymoma and associated intratumoral or juxtatumoral cysts were measured on serial images. Neurological changes and patient function were reviewed and correlated with radiological changes. RESULTS Forty-one of 95 patients were found to have ependymomas (median age 26 years; range 11-53 years). Thirty-two patients with a total of 71 ependymomas had scans appropriate for serial assessment with a mean follow-up of 24 months (range 3-57 months). Ependymomas without cystic components showed minimal change in MLE. Twelve patients had ependymomas with cystic components or syringes. In these patients, reductions in MLE were observed, particularly due to decreases in the cystic components of the ependymoma. Clinical improvement was seen in 7 patients, who all had cystic ependymomas. CONCLUSIONS Bevacizumab treatment in NF2 patients with spinal cord ependymomas results in a decrease in the size of intratumoral and juxtatumoral cysts as well as adjacent-cord syringes and a decrease in cord edema. This may provide clinical benefit in some patients, although the changes do not meet the current criteria for radiological tumor response.


MLE = maximum longitudinal extent; MRC = Medical Research Council; NF2 = neurofibromatosis Type 2; VEGF = vascular endothelial growth factor; VEGFA = vascular endothelial growth factor A; VEGFR = VEGF receptor; bevacizumab; ependymoma; neurofibromatosis Type 2; oncology; spinal tumor

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