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Bevacizumab treatment for symptomatic spinal ependymomas in neurofibromatosis type 2
Author(s) -
Farschtschi S.,
Merker V. L.,
Wolf D.,
Schuhmann M.,
Blakeley J.,
Plotkin S. R.,
Hagel C.,
Mautner V. F.
Publication year - 2016
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/ane.12490
Subject(s) - bevacizumab , medicine , neurofibromatosis type 2 , ependymoma , neurofibromatosis , immunohistochemistry , surgery , oncology , pathology , chemotherapy
Background Neurofibromatosis type 2 ( NF 2) is a tumor suppressor syndrome associated with vestibular schwannomas, meningiomas, and spinal ependymomas. There have been anecdotal reports of radiographic response of spinal ependymomas in NF 2 patients being treated for progressive vestibular schwannomas with bevacizumab, a monoclonal antibody against vascular endothelial growth factor ( VEGF ). Aims The aim of this study was to review the clinical effects of bevacizumab treatment for symptomatic, NF 2‐associated ependymomas Methods We conducted a retrospective review of all patients with NF 2 treated with bevacizumab for symptomatic ependymoma at three NF 2 specialty centers. Tumor size was evaluated by linear measurements; radiographic response was defined as >20% reduction in tumor size. We also performed immunohistochemical evaluation of NF 2‐associated symptomatic ependymomas from five patients, including two from this clinical series. Results Eight patients with NF 2 and symptomatic ependymoma were treated with bevacizumab. All patients had subjective clinical improvement with bevacizumab, although only five of eight patients evaluated had radiographic response. All tumors expressed VEGF ‐R2. Four of five evaluated ependymomas expressed VEGF ‐R1; one without VEGF ‐R1 expression was from a patient who showed clinical but not radiographic response. Conclusions Treatment using bevacizumab improved symptoms related to NF 2‐associated ependymomas, often without concurrent radiographic response. This treatment effect may be related to VEGF ‐R1 expression in NF 2‐associated ependymoma.