Neurofibromatosis is a condition characterized by the growth of benign tumors along nerves in the body. The tumors can eventually grow large enough to compress surrounding nerves and other tissues, which causes various different symptoms depending on the tumor’s location. The main aim of treatment is to preserve function rather than to cure the condition.
Some of the approaches to managing neurofibromatosis type 2 are described below:
In cases of very slow growing or low-grade tumors, an approach called watchful waiting may be adopted. The patient is carefully monitored on a regular basis to assess tumor growth and check whether the condition is becoming symptomatic. The monitoring usually involves magnetic resonance imaging (MRI) scans, eye tests and hearing tests.
Early intervention with surgery
This approach is adopted by certain centres to eliminate the tumor early on, before symptoms have a chance to develop.
Vestibular schwannoma treatment
The tumors that develop along the nerves that control hearing and balance are called vestibular schwannomas. If the tumor is small, it may be removed without causing any damage to the hearing but if a tumor has grown, surgery can be complicated if nerves that affect the facial movement, balance and hearing are involved. An alternative to conventional surgery is radiosurgery which may be used to reduce the size of the tumor instead.
Several techniques can be used to preserve or improve the hearing including surgical hearing implants and speech and language therapy, for example. In cases of complete hearing loss due to damaged cochlear nerves, auditory brain stem implants may be used.
Some medications that may have beneficial effects in neurofibromatosis type 2 include lapatinib, a tyrosine kinase that inhibitis EGFR receptors; an immunosuppressant called everolimus and a monoclonal antibody called bevacizumab that inhibits vascular endothelial growth factor (VEGF) and therefore tumour angiogenesis (new blood vessel growth within tumors).
Reviewed by Sally Robertson, BSc