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To the Editor: The von Hippel-Lindau (VHL) disease is a rare hereditary cancer syndrome caused by mutation in the VHL tumor suppressor gene that results in enhanced transcription of several hypoxia-inducible genes, including the gene for vascular endothelial growth factor (VEGF). Patients with VHL disease often develop highly vascular hemangioblastomas and solid tumors1 with aberrant angiogenesis.2,3 The compound SU5416, which inhibits vascular endothelial growth factor receptor 2, has been reported to slow tumor angiogenesis and growth4 and has been investigated in patients with advanced cancer.5 We conducted a phase I/phase II study of SU5416 in patients with VHL disease.

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