Letters Section Editor: Stephen J. Lurie,
MD, PhD, Senior Editor.
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.
Madhusudan S, Deplanque G, Braybrooke JP, Cattell E, Taylor M, Price P, Tsaloumas MD, Moore N, Huson SM, Adams C, Frith P, Scigalla P, Harris AL. Antiangiogenic Therapy for von Hippel-Lindau Disease. JAMA. 2004;291(8):943-944. doi:10.1001/jama.291.8.943