Dr. Jonasch, Professor, MD Anderson Cancer Center, discusses the incidence of Von Hippel-Lindau Disease among renal cell cancer patients and why it is important to know about it

Overview of Von Hippel Lindau Disease

VHL is a genetic mutation you may not know about that causes cancer.

Marston Linehan, MD, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, gives his opinion on the key kidney cancer data from this meeting. This interview took place at the 2020 Genitourinary Cancers Symposium, held in San Francisco, CA.

Eric Jonasch, MD, Professor, Department of Genitourinary Medical Oncology at MD Anderson Cancer Center, discusses data presented on MK-6482 for the treatment of renal cell carcinoma associated with von Hippel-Lindau disease.

Von Hippel-Lindau disease is a genetic disease characterized by the formation of tumors and cysts in many different parts of the body. The condition can result in a variety of noncancerous or cancerous tumors due to mutations in the VHL gene. People with von Hippel-Lindau disease are also at increased risk for developing other cancers, including renal cell carcinoma.

MK-6482 is an HIF-2α Inhibitor being investigated in a non-randomized phase 2 clinical trial to treat people with von Hippel-Lindau disease-associated renal cell carcinoma.

The primary endpoint of the study was the objective response rate in kidney masses. This study observed that 36% of patients who had confirmed responses with an additional 11% of patients having unconfirmed, partial responses. Furthermore, there were objective responses seen in some patients’ pancreatic lesions as well as stabilization or improvement in patients’ hemangioblastomas.
MK-6482 was recently given breakthrough therapy designation by the U.S. Food and Drug Administration (FDA). As Dr. Jonasch says, the psychological burden of von Hippel-Lindau disease on patients is tremendous and he hopes that this designation will lead to approval of MK-6482 in the next few years.

For more information about Von Hippel-Lindau disease and other rare cancers, visit checkrare.com/diseases/cancers/

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Join us for a lecture by Dr. Jeffrey Bruce on central nervous system tumors in von Hippel-Lindau Syndrome from the 2019 NYC VHL Family Education Day, sponsored by the VHL Comprehensive Clinical Care Center at NY Presbyterian/ Columbia University Medical Center.

Find out more about the NY Presbyterian/Columbia Comprehensive CCC at: vhl.org/nypcolumbia

At the 2016 NORD Breakthrough Summit in Arlington, Virgina, we spoke with Ilene Sussman, Executive Director of the VHL Alliance about Von Hippel-Lindau disease.