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Ref Type Journal Article
PMID (22105611)
Authors Jonasch E, McCutcheon IE, Waguespack SG, Wen S, Davis DW, Smith LA, Tannir NM, Gombos DS, Fuller GN, Matin SF
Title Pilot trial of sunitinib therapy in patients with von Hippel-Lindau disease.
Journal Annals of oncology : official journal of the European Society for Medical Oncology
Vol 22
Issue 12
Date 2011 Dec
URL
Abstract Text Von Hippel-Lindau (VHL) disease induces vascular neoplasms in multiple organs. We evaluated the safety and efficacy of sunitinib in VHL patients and examined the expression of candidate receptors in archived tissue.Patients with VHL were given four cycles of 50 mg sunitinib daily for 28 days, followed by 14 days off. Primary end point was toxicity. Modified RECIST were used for efficacy assessment. We evaluated 20 archival renal cell carcinomas (RCCs) and 20 hemangioblastomas (HBs) for biomarker expression levels using laser-scanning cytometry (LSC).Fifteen patients were treated. Grade 3 toxicity included fatigue in five patients. Dose reductions were needed in 10 patients. Eighteen RCC and 21 HB lesions were evaluable. Six of the RCCs (33%) responded partially, versus none of the HBs (P = 0.014). LSC revealed that mean levels of phosphorylated vascular endothelial growth factor receptor-2 were lower in HB than in RCC endothelium (P = 0.003) and mean phosphorylated fibroblast growth factor receptor substrate-2 (pFRS2) levels were higher in HB (P = 0.003).Sunitinib treatment in VHL patients showed acceptable toxicity. Significant response was observed in RCC but not in HB. Greater expression of pFRS2 in HB tissue than in RCC raises the hypothesis that treatment with fibroblast growth factor pathway-blocking agents may benefit patients with HB.

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Molecular Profile Treatment Approach
VHL W88* VEGFR2 Inhibitor
VHL R69C VEGFR2 Inhibitor
VHL Y98N VEGFR2 Inhibitor
VHL W117C VEGFR2 Inhibitor
VHL L198* VEGFR2 Inhibitor
VHL L184P VEGFR2 Inhibitor
VHL R64P VEGFR2 Inhibitor
VHL Q132fs VEGFR2 Inhibitor
VHL E70* VEGFR2 Inhibitor
VHL loss VEGFR2 Inhibitor
VHL F148fs VEGFR2 Inhibitor
VHL N90I VEGFR2 Inhibitor
VHL L158fs VEGFR2 Inhibitor
VHL K171G VEGFR2 Inhibitor
VHL Y112H VEGFR2 Inhibitor
VHL S65L VEGFR2 Inhibitor
VHL Y98H VEGFR2 Inhibitor
VHL G114R VEGFR2 Inhibitor
VHL R167W VEGFR2 Inhibitor
VHL L158S VEGFR2 Inhibitor
VHL V155fs VEGFR2 Inhibitor
VHL del VEGFR2 Inhibitor
VHL F148Lfs*11 VEGFR2 Inhibitor
VHL P86H VEGFR2 Inhibitor
VHL negative VEGFR2 Inhibitor
VHL L158P VEGFR2 Inhibitor
VHL D126N VEGFR2 Inhibitor
VHL L163P VEGFR2 Inhibitor
VHL C77* VEGFR2 Inhibitor
VHL S65W VEGFR2 Inhibitor
VHL Q195* VEGFR2 Inhibitor
VHL Q96* VEGFR2 Inhibitor
VHL N131fs VEGFR2 Inhibitor
VHL inact mut VEGFR2 Inhibitor
VHL L116fs VEGFR2 Inhibitor
VHL P154L VEGFR2 Inhibitor
VHL S68W VEGFR2 Inhibitor
VHL Y112N VEGFR2 Inhibitor
VHL R161P VEGFR2 Inhibitor
VHL S111R VEGFR2 Inhibitor
VHL E52fs VEGFR2 Inhibitor
VHL V62fs VEGFR2 Inhibitor
VHL H115R VEGFR2 Inhibitor
VHL C162F VEGFR2 Inhibitor
VHL S65* VEGFR2 Inhibitor
VHL I206* VEGFR2 Inhibitor
VHL W117R VEGFR2 Inhibitor
VHL R167Q VEGFR2 Inhibitor
VHL S68fs VEGFR2 Inhibitor
VHL N78S VEGFR2 Inhibitor
VHL F136L VEGFR2 Inhibitor
VHL P81S VEGFR2 Inhibitor
VHL F119L VEGFR2 Inhibitor
VHL S183L VEGFR2 Inhibitor
VHL L188V VEGFR2 Inhibitor
VHL G144fs VEGFR2 Inhibitor
VHL T202* VEGFR2 Inhibitor
VHL S111N VEGFR2 Inhibitor
VHL S68* VEGFR2 Inhibitor
VHL R161G VEGFR2 Inhibitor
VHL R82P VEGFR2 Inhibitor
VHL H115Q VEGFR2 Inhibitor
VHL C162W VEGFR2 Inhibitor
Gene Name Source Synonyms Protein Domains Gene Description Gene Role
Therapy Name Drugs Efficacy Evidence Clinical Trials
Drug Name Trade Name Synonyms Drug Classes Drug Description
Gene Variant Impact Protein Effect Variant Description Associated with drug Resistance
Molecular Profile Indication/Tumor Type Response Type Therapy Name Approval Status Evidence Type Efficacy Evidence References
VHL mutant renal cell carcinoma predicted - sensitive Sunitinib Phase I Actionable In a Phase I trial, 33% (6/18) of renal cell carcinoma patients harboring a VHL mutation, as determined from archived patient specimens, demonstrated a partial response when treated with Sutent (sunitinib) (PMID: 22105611). 22105611