Imatinib Mesylate in Treating Children with Neurofibromatosis and Airway Tumors

Inclusion Criteria

• Diagnosis of neurofibromatosis type 1 (NF1)
• Presence of symptomatic airway plexiform neurofibromas; defined by abnormal sleep study or pulmonary function testing
• Patients must have measurable (>= 1.5 cm in two dimensions or able to assess a minimum of 3 slices) disease by magnetic resonance imaging (MRI)
• Patients must have a Karnofsky of >= 70% or Lansky of >= 50% and a life expectancy of >= 2 months
• Total bilirubin < 1.5 x upper limit of normal (ULN)
• Serum glutamic-oxaloacetic transaminase (SGOT) and serum glutamate pyruvate transaminase (SGPT) < 2.5 x UNL
• Creatinine < 1.5 x ULN
• Absolute neutrophil count (ANC) > 1.5 x 10^9/L
• Platelets > 100 x 10^9/L
• Patients must be able to swallow whole pills or crushed pills in a soft food such as pudding or apple sauce; or have other gastrointestinal (GI) access such as a gastrostomy (G)-tube
• Written, voluntary informed consent/assent

Exclusion Criteria

• Patient has received any other investigational agents within 14 days of first day of study drug dosing
• Patient is < 5 years free of another primary malignancy except: if the other primary malignancy is not currently clinically significant nor requiring active intervention, or if other primary malignancy is a basal cell skin cancer or a cervical carcinoma in situ. Existence of any other malignant disease is not allowed
• Patient with grade III/IV cardiac problems as defined by the New York Heart Association criteria. (i.e., congestive heart failure, myocardial infarction within 6 months of study)
• Patient has a severe and/or uncontrolled medical disease (i.e., uncontrolled diabetes, chronic renal disease, or active uncontrolled infection)
• Patient has a known brain metastasis. Non-specific central nervous system (CNS) changes on MRI/computed tomography (CT) characteristic of NF1 are allowed, but not known CNS malignancies requiring therapeutic intervention
• Patient has known chronic liver disease (i.e., chronic active hepatitis, and cirrhosis)
• Patient has a known diagnosis of human immunodeficiency virus (HIV) infection
• Patient received chemotherapy within 4 weeks (6 weeks for nitrosourea or mitomycin-C) prior to study entry
• Patient previously received radiotherapy to >= 25 % of the bone marrow
• Patient had a major surgery within 2 weeks prior to study entry
• Patient/parent with any significant history of non-compliance to medical regimens or with inability to grant reliable informed consent
• Patients who have or anticipate receiving permanent (or semi-permanent) metallic structures attached to their body. (e.g., braces on teeth, body piercings), which their physicians believe will interfere with the MRI
• Patient has an unstable airway requiring more urgent intervention or deemed unable to travel due to unstable airway by referring Doctor of Medicine (MD)