Neurofibromatosis Type 2 Medication

Updated: Apr 12, 2022
  • Author: David T Hsieh, MD, FAAP; Chief Editor: George I Jallo, MD  more...
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Medication

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]

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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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