P16.07 BEVACIZUMAB AS PALLIATIVE TREATMENT OF FAMILIAL SCHWANNOMATOSIS

• Published in: Neuro-oncology (Charlottesville, Va.) Vol. 16; no. suppl 2; p. ii79
• Main Authors: Clement, P. M., Blockmans, D., Bechter, O. E., Van Calenbergh, F., Legius, E.
• Format: Journal Article
• Language: English
• Published: 01.09.2014
• ISSN: 1522-8517; 1523-5866
• DOI: 10.1093/neuonc/nou174.304

Familial schwannomatosis is a rare genetic disorder characterized by multiple schwannomas and chronic pain. There are, except for the schwannomas, no characteristic findings of neurofibromatosis type II. The only established treatments involve repeated surgery, radiotherapy including gamma knife radiosurgery, and analgesics. We describe a 42-year old female patient with familial schwannomatosis with numerous schwannomas. She underwent 12 resections for symptomatic schwannomas in the past two decades, and was treated with external beam radiotherapy at the skull base and the pelvic region in 2006 and 2010, respectively. Based on reports in neurofibromatosis type II and the expression of VEGF, the patient was treated after written informed consent with bevacizumab at a dose of 5 mg/kg every two weeks. She started treatment in November 2013. The treatment was well tolerated, and the patient observed a decreasing volume of a cervical schwannoma. The patient is free of pain, three months after the start of treatment. CT imaging confirmed an average volume reduction of about 25% in all lesions. In irradiated areas, the volume reduction seemed less pronounced than in previously untreated lesions. To our knowledge, this is the first report of a response to systemic treatment, observed in an adult patient with familial schwannomatosis. Treatment with bevacizumab could be considered as a palliative treatment in patients with this rare but debilitating disease.