Intervention: Cediranib
Trial phase: Phase 2
ATC code: L01EK02
SMILES: COc1cc2c(Oc3ccc4[nH]c(C)cc4c3F)ncnc2cc1OCCCN1CCCC1
Sex: All
Age groups: Adult | Older Adult
Healthy volunteers: No
Participants at risk: 26
Group description: Patients receive 30 mg oral AZD2171 once daily on days 1-28. Treatment repeats every 28 days for 26 courses in the absence of disease progression or unacceptable toxicity. Patients with responding or stable disease may continue treatment beyond 26 courses in the absence of disease progression or unacceptable toxicity.
Eligibility criteria:
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