Neurofibromatoses are a complex set of genetic diseases with a wide spectrum of clinical manifestations. Life-threatening complications may develop as the result of tumor progression. Surgical intervention is the only effective means of treatment for progressive pain, disfigurement, functional compromise, and malignancy. In the future, molecular advances should allow for the development of targeted therapies to treat patients who have neurofibromatosis in addition to those who have sporadic tumors. Tumor profiling should allow us to guide therapies and predict responses.
- Malignant peripheral nerve sheath tumor
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