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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.

Methods

Participants were 6 patients with progressive VHL disease despite standard treatment. (Clinical details are available from the authors.) Patients were excluded if they had a neutrophil count of less than1500 cells/mm³, hemoglobin level less than 9.0 g/dL, platelet count of less than 100 000 cells/mm³, abnormal liver or renal function . . . [Full Text of this Article]

Srinivasan Madhusudan, MBBS, MRCP; Gaël Deplanque, MSc, MD, PhD; Jeremy P. Braybrooke, MRCP, PhD; Emma Cattell, MBBS, MRCP; Marian Taylor, BSc
Cancer Research UK Medical Oncology Unit
Oxford Radcliffe Hospitals
Oxford, England

Pat Price, MD, FRCR, FRCP
Academic Department of Radiation Oncology
Christie Hospital
Manchester, England

Marie D. Tsaloumas, FRCOphth
Department of Ophthalmology
Birmingham & Midland Eye Clinic
Birmingham, England

Niall Moore, FRCR
Department of Radiology

Susan M. Huson, MD, FRCP
Department of Clinical Genetics

Chris Adams, MA, MChir, FRCS
Department of Neurosurgery

Peggy Frith, FRCOphth
Department of Ophthalmology
Oxford Radcliffe Hospitals

Paul Scigalla, MD, PhD
SUGEN Inc
San Francisco, Calif

Adrian L. Harris, FRCP, DPhil
aharris.lab@cancer.org.uk
Cancer Research UK Medical Oncology Unit
Oxford Radcliffe Hospitals

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