PRIMARY OBJECTIVE:~I. Evaluate safety and efficacy of treatment with pazopanib hydrochloride (pazopanib) for 6 months in patients with von Hippel-Lindau syndrome (VHL) who have a measurable VHL related lesion.~SECONDARY OBJECTIVES:~I. Evaluate rate of growth over time in target lesions before and after pazopanib treatment.~II. Evaluate need for surgical intervention over time in patients who receive pazopanib and compare to rate prior to receipt of drug.~III. Create an annotated tissue resource from patients with VHL for use in future research related to cancer.~PRECLINICAL OBJECTIVES:~I. Evaluate circulating factors in patients with VHL undergoing treatment with pazopanib.~II. Evaluate relationship between VHL genotype and response to pazopanib.~OUTLINE:~Patients receive pazopanib hydrochloride orally (PO) once daily (QD) on days 1-28. Treatment repeats every 4 weeks for up to 24 weeks in the absence of disease progression or unacceptable toxicity. Patients benefitting from treatment may continue pazopanib hydrochloride in the absence of disease progression.~After completion of study treatment, patients are followed up at 30 days and then every 3 months for up to 24 weeks.
This phase II trial studies the side effects and how well pazopanib hydrochloride works in treating patients with von Hippel-Lindau syndrome. Pazopanib hydrochloride may stop the growth of cancer cells by blocking some of the enzymes needed for cell growth.