Neurofibromatosis Type 2 Medication

Updated: Oct 06, 2016
  • Author: David T Hsieh, MD, FAAP; Chief Editor: Amy Kao, MD  more...
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Medication

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. [21] 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. [22]

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. [23]

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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.

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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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