AZD2171 in Treating Patients With Neurofibromatosis Type 1 and Plexiform Neurofibroma and/or Neurofibroma Near the Spine

Inclusion Criteria: Diagnosis* of neurofibromatosis type 1 (NF1) and extensive plexiform and/or paraspinal neurofibromasproducing pain (not controlled by use of over-the-counter medications), progressive neurologic deficit, or significant neurologic consequenceswith continuous tumor growth Extensive paraspinal neurofibroma defined as a neurofibroma that involves multiple neural roots at ≥ 3 spinal levels with connection between the levels or extending laterally along the nerves Symptomatic neurofibromas at < 3 spinal levels, but surgical treatment is not possible, allowed Meets ≥ 2 diagnostic criteria for NF1, including the following: Six or more café-au-lait spots (≥ 1.5 cm in postpubertal patients) Freckling in the axilla or groin Optic glioma Two or more Lisch nodules Distinctive bony lesion (dysplasia of the sphenoid bone or dysplasia orthinning of long-bone cortex) First-degree relative with NF1 Patients with documented mutation in neurofibromin gene with onlysymptomatic plexiform and/or paraspinal neurofibroma who do not fulfill the above clinical criteria are eligible Measurable disease, defined as ≥ 1 lesion whose longest diameter can beaccurately measured as 8.0 cm^3 with 3-dimensional (3D) MRI Skin lesions are consideredmeasurable (e.g., plexiform neurofibromas), but MRI imaging still required for 3D measurement Patients with symptomatic neurofibroma, in whom surgery is not feasible, who refuse surgery or are not goodsurgical candidates due to high risk of damage to vital structures or spinal cordinjury are eligible No evidence of progressive optic glioma, malignant glioma, malignant peripheralnerve sheath tumor, or other cancer requiring treatment with chemotherapy orradiotherapy ECOG performance status 0-3 WBC ≥ 3,000/mm^3 Absolute neutrophil count ≥ 1,500/mm^3 Platelet count ≥ 100,000/mm^3 Hemoglobin ≥ 8.0 g/dL Bilirubin normal (patients with Gilbert's syndrome allowed despite elevated bilirubin) Alkaline phosphatase normal AST and ALT ≤ 2.5 times upper limit of normal Thyroid-stimulating hormone and free thyroxin normal Creatinine normal OR creatinine clearance ≥ 60 mL/min Ejection fraction ≥ 50% by echocardiogram Not pregnant or nursing Negative pregnancy test Fertile patients must use effective contraception No other uncontrolled, serious medical condition that would preclude study participation, including any of the following: Cardiac arrhythmia Diabetes Serious infection Significant cardiac, pulmonary, hepatic, or other organ dysfunction No psychiatric illness or social situation that would preclude study compliance No history of allergic reactions attributed to compounds of similar chemical orbiologic composition to AZD2171 No New York Heart Association class III or IV disease Class II disease controlled with treatment and increased monitoring allowed No systolic blood pressure (BP) > 130 mm Hg and diastolic BP > 90 mm Hg No history of familial long QT syndrome Mean QTc ≤ 470 msec (with Bazett's correction) by EKG QTc prolongation ≤ 500 msec No other significant ECG abnormality within the past 14 days See Disease Characteristics More than 30 days since prior investigational agents More than 4 weeks since prior radiotherapy, chemotherapy, hormonal therapy directed at thetumor, immunotherapy, biologic therapy (e.g., interferon), or majorsurgery No concurrent medication that may markedly affect renal function (e.g., vancomycin, amphotericin, or pentamidine) No concurrent CYP interactive medications No concurrent combination antiretroviral therapy for HIV-positive patients No concurrent enzyme-inducing anticonvulsants (e.g., phenytoin, carbamazepine, or phenobarbital) No concurrent use of drugs or biologics with proarrhythmic potential