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Mi hermana tiene una enfermedad rara, oncológica, progresiva, altamente incapacitante e invalidante, llamada VHL (von Hippel-Lindau). Tiene 37 años, y desde que fue diagnosticada en 1995 (con 21) ha sufrido 4 neurocirugías, una cirugía por cáncer renal, ha perdido la visión de un ojo y la audición de un oído. La última neurocirugía

Sandra Kenworthy, 41, only discovered she had Von Hippel–Lindau (VHL) disease in 1996, while seven months pregnant with her third daughter...

Recruiting

Le professeur Stéphane Richard de la faculté de médecine de Paris propose son aide à cette famille.  

The 10th International VHL Medical Symposium, organized by the MD Anderson Cancer Center and the VHL Family Alliance, took place at the Hotel Zaza in Houston, Texas on January 26th to 29th, 2012.

The first day there were sections on metabolomics, cilia centrosome regulation, epigenetics, VHL proteostasis and non VHL hereditary renal disorders, as well as advances in current treatment options.

Abstract

Background Radiofrequency ablation (RFA) is accepted as a minimally invasive treatment of renal cell carcinoma (RCC). However, RFA is not fully evaluated for treating RCC in patients with von Hippel Lindau (VHL) disease who cannot undergo surgery due to serious postoperative morbidity or mortality. Purpose To evaluate the role of RFA of RCC in patients with VHL disease previously undergoing renal surgery. Material and Methods Percutaneous RFA...

European Journal of Human Genetics advance online publication, March 9, 2011. doi:10.1038/ejhg.2010.175

Authors: Eamonn R Maher, Hartmut PH Neumann and Stephane Richard (Source: European Journal of Human Genetics)

Abstract

The autosomal dominantly inherited disorder von Hippel–Lindau disease (VHL) is caused by germline mutations in the VHL tumour suppressor gene (TSG). VHL mutations predispose to the development of a variety of tumours (most commonly retinal and central nervous system haemangioblastomas, clear cell renal carcinoma and phaeochromocytomas). Here, we review the clinical and genetic features of VHL disease, briefly review the molecular pathogenesis and outline clinical management and tumour surveillance strategies.

Status: Recruiting, Condition Summary: Renal Cancer; Von Hippel Lindau

This study will examine the effectiveness of an investigational drug called ZD6474 (also known as vandetanib or ZACTIMA). Vandetanib is an experimental drug that is designed to prevent the growth and development of new blood vessels on tumors and to prevent the direct growth of cancer cells. It has been tested in a number of clinical trials on adults with cancer, but the U.S. Food and Drug Administration has not specifically approved it as a cancer treatment. The purpose of this investigational study is to better understand how vandetanib affects humans who have kidney cancer related to von Hippel-Lindau (VHL) disease, and to develop tests that may improve researchers' understanding of kidney cancer and its effects.

Volunteers must be at least 18 years old and must have been diagnosed with kidney cancer related to VHL. Candidates must have a life expectancy greater than three months and must have at least one measurable renal tumor for study purposes. Candidates may not be receiving any other investigational agents or have been treated with an investigational drug within the past four weeks. Candidates who have had surgery, chemotherapy, or radiotherapy within the past four weeks will be excluded from the study. Candidates will be screened with a physical examination and medical history.

During the study, participants will receive an oral dose of vandetanib once a day for 28 days (a treatment period known as a cycle). Participants will need to return to the National Institutes of Health every two weeks on the same day of the week as the first dose of vandetanib for a series of tests and procedures, including blood and urine tests and an electrocardiogram. Every 12 weeks, computerized tomography (CT) or magnetic resonance imaging (MRI) scans will be done to assess the size of participants' tumors. Participants whose tumors do not grow and who do not have unacceptable side effects may continue to receive vandetanib to maintain the current condition, until researchers conclude the study....

Abstract only 

BACKGROUND: To evaluate the efficacy and safety of probe ablative therapy as salvage treatment for renal tumor in von Hippel-Lindau (VHL) patients after previous partial nephrectomy (PN).