Patients with neurofibromatosis type 1 (NF1) commonly develop non-cancerous tumors called
plexiform neurofibromas. These tumors can be defined as "high-risk" when they result in
severe pain, physical disability, organ dysfunction and/or become life-threatening.
Presently, there is no effective medical therapy to offer patients with "high-risk" plexiform
neurofibromas, and surgery does not provide lasting help. This study will evaluate the
effectiveness of two treatment combinations in patients with "high-risk" plexiform
neurofibromas.