Medication Summary
No curative medical therapies are known for neurofibromatosis type 2 (NF2). However, in instances in which full 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, bevacizumab may be recommended to radiographically reduce the size or prolong tumor stability in patients without surgical options, and can improve hearing or prolong time to hearing loss. [33, 32] Lapatinib may also be considered for use in reducing the size of vestibular schwannomas and to improve hearing, and there is some evidence that lapatinib may inhibit the growth of meningiomas in NF2. [34, 35]
Antineoplastic Agents
Class Summary
Antineoplastic agents act by inhibiting the key factors responsible for neoplastic transformation of cells.
Vincristine ( Vincasar PFS)
Vincristine is a vinca alkaloid that is cell cycle–specific (M phase). The mitotic apparatus is arrested in metaphase via disruption of the microtubules. Absorption of vincristine through the GI tract is variable; therefore, administer the drug intravenously. It is metabolized extensively in the liver and excreted primarily via bile. Neurotoxicity is the limiting factor during therapy. Peripheral neuropathy is vincristine's most common adverse effect at usual doses.
Carboplatin
Analog of cisplatin used in treatment regimens for relapse. This is a heavy metal coordination complex that exerts its cytotoxic effect by platination of DNA, a mechanism analogous to alkylation, leading to interstrand and intrastrand DNA crosslinks and inhibition of DNA replication. Binds to protein and other compounds containing SH group. Cytotoxicity can occur at any stage of the cell cycle, but cell is most vulnerable to action of these drugs in G1 and S phase.
Lomustine
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 (Rayos)
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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Subcutaneous and cutaneous lesions in a young man with neurofibromatosis type 2; note paucity of cafe-au-lait spots.
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Right neck mass in a patient with neurofibromatosis type 2.
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Facial asymmetry, OS proptosis, and exotropia, as well as several subcutaneous lesions on the forehead and face, in a 20-year-old man with neurofibromatosis type 2.
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Posterior cervical scar from cord lesion resection, thoracic scoliosis, and subcutaneous masses in a young adult with neurofibromatosis type 2.
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Meningioma to the left of midline in a patient with neurofibromatosis type 2.
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Multiple meningiomas (on the left) on the surface of the brain in a patient with neurofibromatosis type 2.
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Bilateral acoustic neuromas in a patient with neurofibromatosis type 2.
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Bilateral acoustic neuromas and a left-sided meningioma in a patient with neurofibromatosis type 2.
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Small ependymoma in a patient with neurofibromatosis type 2.
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Multiple meningiomas in a patient with neurofibromatosis type 2.
