Individuals currently living, over the age of 18 years and known carriers of a pathogenic variant in the VHL gene.

DNA extracted from periferal blood and CNS hemangioblastomas removed as part of the participants treatment for their condition.

Von Hippel-Lindau Disease

DNA from CNS hemangioblastomas and normal tissue (blood) will be analysed using whole exome sequencing.

Whole exome sequencing

Department of Cellular and Molecular Medicine

Odense University hospital, department of clinical genetics

Drivers of Hypoxia-induced Angiogenesis in Tumor Development

Marie Louise Binderup

Postdoc researcher

University of Copenhagen

Odense University Hospital

300 mg/day (starting dose) oral dose of vandetanib once a day for 28 days

Vandetanib in Participants With Kidney Cancer

Sex: Female, Male

Ethnicity (NIH/OMB)

Race (NIH/OMB)

Region of Enrollment

Overall response rate is defined as the percentage of participants with either a partial or complete response occurring at any time after initiation of therapy. Response is determined by the Response Evaluation Criteria in Solid Tumors (RECIST). Partial response (PR) is at least a 30% decrease in the sum of the longest diameter (LD) of target lesions, taking as reference the baseline sum LD. Complete response (CR) is a disappearance of all target lesions. Progressive disease (PD) is at least a 20% increase in the sum of the LD of target lesions, taking as reference the smallest sum LD recorded since the treatment started or the appearance of one or more new lesions. Stable disease (SD) is neither sufficient shrinkage to qualify for PR nor sufficient increase to qualify for PD (progressive disease), taking as reference the smallest sum LD since the treatment started.

Overall Response Rate.

Here is the number of participants with adverse events. For a detailed list of adverse events, see the adverse event module.

Number of Participants With Adverse Events

Time to progression is defined as the time from the start of treatment to progression as defined by the Response Evaluation Criteria in Solid Tumors (RECIST) criteria, with death being treated as a censored event. Partial response (PR) is at least a 30% decrease in the sum of the longest diameter (LD) of target lesions, taking as reference the baseline sum LD. Complete response (CR) is a disappearance of all target lesions. Progressive disease (PD) is at least a 20% increase in the sum of the LD of target lesions, taking as reference the smallest sum LD recorded since the treatment started, the appearance of one or more new lesions, developing a surgical size tumor that should be resected. Stable disease (SD) is neither sufficient shrinkage to qualify for PR nor sufficient increase to qualify for PD (progressive disease), taking as reference the smallest sum LD since the treatment started.

Time To Progression (TTP)

Progression free survival is defined as time from initiation of treatment to either progression as defined by Response Evaluation Criteria in Solid Tumors (RECIST) criteria or death. Partial response (PR) is at least a 30% decrease in the sum of the longest diameter (LD) of target lesions, taking as reference the baseline sum LD. Complete response (CR) is a disappearance of all target lesions. Progressive disease (PD) is at least a 20% increase in the sum of the LD of target lesions, taking as reference the smallest sum LD recorded since the treatment started or the appearance of one or more new lesions. Stable disease (SD) is neither sufficient shrinkage to qualify for PR nor sufficient increase to qualify for PD (progressive disease), taking as reference the smallest sum LD since the treatment started.

Progression Free Survival (PFS)

Peripheral blood was collected and analyzed by multiparametric flow cytometry for analysis of angiogenesis markers.

Effect of Vandetanib (ZD6474) on Endothelial Progenitor Cells

Peripheral blood was collected and analyzed by multiparametric flow cytometry.

Effect of Vandetanib (ZD6474) on Circulating Endothelial Cells (CEC)

Tumor measurements for non-renal tumors were assessed by the Response Evaluation Criteria in Solid Tumors (RECIST) before and after treatment. Tumor measurements for non-renal tumors were assessed by the Response Evaluation Criteria in Solid Tumors (RECIST) before and after treatment. Complete response (CR) is disappearance of all target lesions. Partial response (PR) is at least a 30% decrease in the sum of the longest diameter (LD) of target lesions, taking as reference the baseline sum LD. Progressive disease (PD) is at least a 20% increase in the sum of the LD of target lesions, taking as reference the smallest um LD recorded since treatment started or the appearance of one or more new lesions. Stable disease (SD) is neither sufficient shrinkage to qualify for PR nor sufficient increase to qualify for PD, taking as reference the smallest sum LD since the treatment started.

Response in Pancreatic Tumors/Cysts Associated With Von Hippel Lindau (VHL)

Tumor measurements for non-renal tumors were assessed by the Response Evaluation Criteria in Solid Tumors (RECIST) before and after treatment. Complete response (CR) is disappearance of all target lesions. Partial response (PR) is at least a 30% decrease in the sum of the longest diameter (LD) of target lesions, taking as reference the baseline sum LD. Progressive disease (PD) is at least a 20% increase in the sum of the LD of target lesions, taking as reference the smallest um LD recorded since treatment started or the appearance of one or more new lesions. Stable disease (SD) is neither sufficient shrinkage to qualify for PR nor sufficient increase to qualify for PD, taking as reference the smallest sum LD since the treatment started.

Response in Central Nervous System (CNS) Hemangioblastomas Associated With Von Hippel Lindau (VHL)

Response in Pheochromocytomas Associated With Von Hippel Lindau (VHL)

5-10cc venous blood was collected for evaluation of basal plasma levels of angiogenesis biomarkers VEGF and VEGFR-2.

Change in Plasma Biomarkers Vascular Endothelial Growth Factor (VEGF) and Soluble Vascular Endothelial Growth Factor Receptor 2 (VEGFR-2) Before and After Treatment With Vandetanib

hemangioblastoma/hypoxia

Drivers of Hypoxia-induced Angiogenesis in Tumor Development

Phase II Study of Vandetanib in Individuals With Kidney Cancer

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