Rapid Clinical and Radiological Response to Bevacizumab in a Patient with Schwannomatosis

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Conference Proceeding

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Limited evidence exists for optimal management of schwannomatosis, the least common neurofibromatosis syndrome characterized by multiple schwannomas and intractable pain in the absence of vestibular schwannomas and germline NF2 mutation. We present a case of a 32-year-old man with a family history of schwannomas who had been dealing with multiple paraspinal schwannomas throughout entire spine, neurological deficits and chronic severe pain since high school. He had multiple laminectomies with resection of schwannomas. By the time neurooncologist saw the patient he had been on daily narcotics for more than 10 years, was not ambulatory due to paraparesis and was dependent on chronic Foley catheter for urination. He was found to have SMARCB1 c.93G >A germline mutation that has not been reported before. Total spine MRI demonstrated further progression of multiple spinal schwannomas with cord compression. The patient started on bevacizumab 10 mg/kg IV every 4 weeks (the first dose 5 mg/kg) but had to have an 8-week interval between his second and third cycle because of slowly healing pressure ulcers over bilateral upper buttocks. The patient started to show improvement in leg movements and strength after 2 cycles, his urinary catheter was discontinued, and he started urinating on his own after 3 cycles of bevacizumab. MRI obtained after 3 cycles demonstrated decreasing size and enhancement of multiple schwannomas. The patient continued bevacizumab every 4 – 6 weeks and continued with clinical improvement of strength and mobility. He started to stand with support for several minutes after 6 cycles of bevacizumab and reported better control of pain. He started outpatient physical therapy sessions. The plan is to continue bevacizumab therapy long term. This case demonstrated efficacy of bevacizumab as an alternative treatment option to improve quality of life and avoid surgeries in patients with this rare debilitating condition.


The 28th Annual Meeting and Education Day of the Society for Neuro-Oncology, November 15-19, Vancouver, BC.

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