A Sneak Peek Into Von Hippel-Lindau Disease Treatment Advances With Eric Jonasch, MD

At this year’s American Society of Clinical Oncology (ASCO) Annual Meeting, in Chicago, Illinois, Eric Jonasch, MD, Professor of Medicine at the University of Texas MD Anderson Cancer Center, sat down to discuss i3 Health’s upcoming live webinar events he will be chairing, entitled Comprehensive Management of VHL Disease: Genetic and Oncological Perspectives. In addition, he shares recent updates in treatment for VHL disease and why it is imperative that clinicians attend these webinars to maintain an updated understanding of the VHL treatment landscape.

Oncology Data Advisor: Welcome to Oncology Data Advisor. Today, we’re here live at the ASCO Annual Meeting in Chicago, and I’m joined by Dr. Eric Jonasch. Thanks so much for coming on today.

Eric Jonasch, MD: Thanks. It’s great to be here.

Oncology Data Advisor: To start off, would you like to introduce yourself and share a little bit about what you do?

Dr. Jonasch: I’m a Professor of Medicine in the Department of Genitourinary (GU) Medical Oncology at the University of Texas MD Anderson Cancer Center, and I run a VHL clinic. Also, I see patients with advanced renal cell carcinoma, I have a lab, and I also run several research consortia.

Oncology Data Advisor: Awesome. We’re really looking forward to the webinars that you’re chairing with i3 Health in June and July, entitled Comprehensive Management of VHL Disease, Genetic and Oncological Perspectives. What are the things that you’re looking forward to discussing during these webinars?

Dr. Jonasch: I think it’s really important with VHL disease to understand the biological underpinnings. Why does it happen? How does it affect individuals? And what can we do about it? So, those are the three guiding principles of the presentation.

Oncology Data Advisor: Absolutely. I think it’ll be a really educational talk. Is there any notable VHL research that’s being presented at ASCO this year?

Dr. Jonasch: At ASCO this year, no. One thing that we did present that is of some interest is we looked retrospectively at a claims database to see whether or not we can get an idea of what the impact of having a VHL diagnosis with hemangioblastomas is on these individuals. One of the challenges in looking at a claims database is identifying who truly has VHL disease, and we came up with an algorithm to identify those individuals, and then we asked the question of whether or not these individuals used a different amount of pain medication compared to control populations, whether they had an increase in imaging compared to control populations, and also whether they had an increase in provider appointments compared to control populations. The answer was yes, they did. This basically shows that, indeed, individuals with VHL disease and hemangioblastomas definitely have to consume more healthcare resources than the average person.

Oncology Data Advisor: Absolutely. In light of these results that you found in this study, is there anything that clinicians should counsel their patients about or any implications for these results?

Dr. Jonasch: I think it’s more from a standpoint of looking at the overall burden of this disease on an individual. Also thinking about potential alternatives, we do have belzutifan that’s now approved, and it raises the question of whether or not—at least in some of these parameters—by using belzutifan earlier, could we decrease, for example, chronic pain because we’re not going to have to have as many surgeries for these patients? Could it also change the other healthcare provider visit frequency because they don’t need them as much?

Oncology Data Advisor: Absolutely. Speaking of belzutifan, would you like to tell us a little bit about some of the recent data that has been published about it?

Dr. Jonasch: Yes. Belzutifan is a HIF-2 alpha inhibitor, and basically what happens is in the renal cell carcinoma cell, you have broken VHL that results in upregulation of HIF-2 alpha, which results in transcription of a whole bunch of things that are good for the cells, but not necessarily good for the person. Belzutifan will block HIF-2 alpha’s interaction with HIF-1 beta, and therefore it can’t act as a transcription factor—you get decreased transcription. That’s how the drug works, and we’ve demonstrated with publications and follow-up publications for von Hippel-Lindau disease that we have consistent effects on renal cell carcinomas, pancreatic neuroendocrine tumors, and hemangioblastomas. More recently, in advanced renal cell carcinoma, publications have come out both in the phase 1 setting, as well as now in a registrational setting, showing that this drug is superior to control agents like everolimus, and it was FDA-approved in December of 2023.

Oncology Data Advisor: Very exciting that it received approval. So, since this webinar is also going to be directed towards genetic counselors, would like to talk a little bit about the role of genetic counselors for patients with VHL disease?

Dr. Jonasch: Yes, it’s critical. Number one, for an individual to undergo genetic testing, they really need to know what the implications of the testing are, and the genetic counselors are very well-equipped to provide that information. Number two, genetic counselors are very good at recognizing patterns within patients and their families to determine what the right testing is. And then the third thing is if the testing is positive, it’s critical then to look at other family members to determine what type of reflex testing should occur. So, these are just three of the many other things that genetic counselors do for the patients, and they’re a critical part of the overall package here.

Oncology Data Advisor: Absolutely, definitely a very critical role. So, for these two webinars that are occurring on June 26th and July 11th, why should listeners attend and what are some of the things that they can look forward to hearing about?

Dr. Jonasch: Well, if they’re interested in the biology of VHL, interested in how to manage VHL, or interested in what new therapies are available for VHL disease, then these webinars really are going to be helpful.

Oncology Data Advisor: Wonderful. We’re really looking forward to hearing more during them as well. Thank you for stopping by to talk about this with us today and looking forward to hearing more in the webinars.

Dr. Jonasch: Thank you.

About Dr. Jonasch

Eric Jonasch, MD, is a Professor in the Department of Genitourinary Medical Oncology, Division of Cancer Medicine, at the University of Texas MD Anderson Cancer Center in Houston, Texas. Dr. Jonasch’s research expertise focuses on improving outcomes for von Hippel-Lindau disease, advanced renal cell carcinoma, and kidney disease. He has been the Director of the von Hippel-Lindau Clinical Center in Houston since 2003.

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