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Pathophysiology
Nerve tumors can form in the peripheral nerve network anywhere in the body. These tumors often affect the function of the nerve, causing pain and disability. A large majority of peripheral nerve tumors are benign. Some are caused by neurofibromatosis, or schwannomatosis. Malignant Peripheral Nerve Sheath Tumors are very aggressive tumors and should be managed by a multi-disciplinary team.
Disease statistics
In Canada, cancer incidence among children aged 0 to 14, 2006?2010 was average number of cases per year-905, annual incidence rate (per million children)-161, percent of childhood cancer cases-100.
Disease treatment
There are several factors in deciding to surgically remove these tumors. Some, like schwannomas are slow-growing and can be watched using imaging studies over time. Others, like malignant nerve sheath tumors need to be removed immediately, as they are very aggressive.
Major research
Current research in on understanding the molecular mechanisms that normally act to arrest tumor growth as well as those that promote nerve tumor, currently comparing and contrasting the molecular profile of growing and non-growing tumors in patients with NF1.