Medication Summary
No effective medical therapies are known for neurofibromatosis type 2 (NF2). However, in rare instances in which surgical resection of symptomatic ependymomas is not possible, chemotherapy with lomustine, vincristine, and prednisone, or carboplatin and vincristine, following radiation therapy, may serve a palliative function.
As previously mentioned, therapeutic use of erlotinib has shown promise in the treatment of unresectable, progressive vestibular schwannomas, resulting not only in a decrease in tumor size but also in improvement in auditory function. Further clinical trials are in order before use of this oral chemotherapeutic agent can be recommended on a routine basis.[9] A trial of bevacizumab, an antivascular endothelial growth factor monoclonal antibody, also showed some efficacy in the shrinkage of vestibular schwannomas; the drug improved hearing in some patients with unresectable tumors.[10]
Early in vitro studies have suggested that Gleevec, a tyrosine kinase inhibitor, may be useful in the treatment of vestibular and spinal cord schwannomas in patients with NF2.[11]
Antineoplastic Agents
Class Summary
Antineoplastic agents act by inhibiting the key factors responsible for neoplastic transformation of cells.
Vincristine ( Vincasar PFS)
Vincristine inhibits tubulin polymerization; therefore, it targets dividing cells.
Carboplatin
This analog of cisplatin is used in treatment regimens for relapse.
Lomustine (CeeNU)
Lomustine inhibits RNA and DNA synthesis through alkylation of DNA and carbamylation of DNA polymerase, and alteration of RNA, enzymes, and proteins.
Corticosteroids
Class Summary
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.
Prednisone
Prednisone, a synthetic glucocorticoid analog, acts as a potent immunosuppressant. It may inhibit cyclooxygenase, which, in turn, inhibits prostaglandin biosynthesis. These effects may result in analgesic, antipyretic, and anti-inflammatory activities.
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Sestini R, Provenzano A, Bacci C, et al. NF2 mutation screening by denaturing high-performance liquid chromatography and high-resolution melting analysis. Genet Test. Jun 2008;12(2):311-8. [Medline].
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Plotkin SR, Singh MA, O'Donnell CC, et al. Audiologic and radiographic response of NF2-related vestibular schwannoma to erlotinib therapy. Nat Clin Pract Oncol. Aug 2008;5(8):487-91. [Medline].
Plotkin SR, Stemmer-Rachamimov AO, Barker FG 2nd, et al. Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med. Jul 23 2009;361(4):358-67. [Medline].
Mukherjee J, Kamnasaran D, Balasubramaniam A, et al. Human schwannomas express activated platelet-derived growth factor receptors and c-kit and are growth inhibited by Gleevec (Imatinib Mesylate). Cancer Res. Jun 15 2009;69(12):5099-107. [Medline]. [Full Text].
von Eckardstein KL, Beatty CW, Driscoll CL, Link MJ. Spontaneous regression of vestibular schwannomas after resection of contralateral tumor in neurofibromatosis Type 2. J Neurosurg. Jan 2010;112(1):158-62. [Medline]. [Full Text].
Safavi-Abbasi S, Bambakidis NC, Zabramski JM, et al. Nonvestibular schwannomas: an evaluation of functional outcome after radiosurgical and microsurgical management. Acta Neurochir (Wien). Jan 2010;152(1):35-46. [Medline].
Gerszten PC, Burton SA, Ozhasoglu C, McCue KJ, Quinn AE. Radiosurgery for benign intradural spinal tumors. Neurosurgery. Apr 2008;62(4):887-95; discussion 895-6. [Medline].
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Colletti V, Shannon R, Carner M, Veronese S, Colletti L. Outcomes in nontumor adults fitted with the auditory brainstem implant: 10 years' experience. Otol Neurotol. Aug 2009;30(5):614-8. [Medline].
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