Schwannomatosis is a rare variant of neurofibromatosis( MF), affecting only around 1 in 40,000 individuals. it consists of multiple cutaneous schwannomas, central nervous system tumors, and other neurological complications, excluding hallmark signs of NF. Mainstays of treatment is surgery, pain management and more recently, radiosurgery. Multiple plexiform fibromas, Schwannomas and acoustic neuromas are a hallmark neurofibromatosis, which condition also presents with other findings and symptoms. It can appear as cafe-au-lait spots and bumps under the skin, or it can lead to complications that include blindness, scoliosis and disfigurement. Fibromas can cause pain and compromise other organs. Few treatments are available, although many novel agents are being studied.
Among these is Avastin. Information is limited and restricted to case reports. VEGF blockade with bevacizumab improved hearing in some, but not all, patients with neurofibromatosis type 2 and was associated with a reduction in the volume of most growing vestibular schwannomas(Plotkin). Mautner reported 2 cases of schwannoma regression on Avastin. Blakeley did a study in 14 patients and concluded that Bevacizumab treatment resulted in a durable hearing response in 36% of patients with NF2 and confirmed progressive VS-associated hearing loss. Imaging and plasma biomarkers showed promising associations with the response that should be validated in larger studies.
Therefore, this promising treatment should be still considered E/I.
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Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med. 2009 Jul 23;361(4):358-67.
Victor-Felix Mautner, Rosa Nguyen et al Bevacizumab induces regression of vestibular schwannomas in patients with neurofibromatosis type 2
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