Brain Tumors in Neurofibromatosis

The neurofibromatoses are common neurocutaneous syndromes with multisystem involvement. These disorders place patients at increased risk for the development of malignancies. In particular, there is a predisposition to develop central nervous system and peripheral nervous system neoplasms. Distinct tumor types develop in association with neurofibromatosis type 1 (NF-1) different from those that are typically associated with neurofibromatosis type 2 (NF-2). In general, the tumors associated with NF-1 and NF-2 tend to demonstrate a more indolent course than similar tumors in patients without neurofibromatosis. An exception would be earlier presentation of tumors in each of these disorders. Management decisions are based on multiple factors. These include tumor location, presumed or known histology, and patient symptoms at time of diagnosis or evidence of progression either clinically or as demonstrated by neuroimaging. Once all of these factors have been weighed, therapeutic considerations include expectant observation, surgery, and radiation or chemotherapy. The overall philosophy of treatment is that of attempting to preserve neurologic function for as long as possible, because these are progressive disorders. At times, it may be preferable to keep interventions to a minimum in order to achieve this goal. A multidisciplinary approach is crucial in the care of these patients.