Lawyer, Model Victoria Vesce Uses Her Platform for Brain Tumor Advocacy

Episode Transcript

Dr. Correa:
From the American Academy of Neurology, I'm Dr. Daniel Correa.

Dr. Nath:
And I'm Dr. Audrey Nath.

Dr. Correa:
This is the Brain & Life podcast.

Dr. Nath:
Daniel, we have a listener question. I'd like to read it. Is there anything for intense vertigo? From Emily. Thank you, Emily. That's a great question.

Dr. Correa:
Emily. That is a great question and something so many people deal with.

Dr. Nath:
Absolutely. Vertigo can be really debilitating. And when we talk about treatments, which is what you're asking about, the first thing we have to do is think about the cause, which is what neurologists love to do. So Daniel, tell us about where in the brain or outside of the brain we might have issues that can cause vertigo.

Dr. Correa:
Yeah, so the treatments as you were mentioning, Audrey, really end up depending a lot as to where is the problem, or what we do in neurology discusses as localizing the dysfunction or the lesion. And so there's the inner ear where not only you actually end up processing and bringing in and hearing, but also there's structures inside your ear that help you understand the position of your body and balance. And that sometimes those are the things that trigger that sense of spinning or falling or kind of like a rollercoaster sensation.
And then the inner ear connects through nerve fibers to the brain stem. And this is sort of the older parts of our brain that then integrates all that information, not just with your coordination of your body, but then of your eyes, and then coordinating how your arms and legs move. And then the back area of your brain there are areas that help coordinate your arms and legs and send the signals down through your spinal cord, so occasionally some people have a problem either in the spinal cord or in that area of your brain that does coordination, the cerebellum.

Dr. Nath:
Cerebellum. Exactly.

Dr. Correa:
That's right.

Dr. Nath:
And strokes of the cerebellum can present with vertigo for sure.

Dr. Correa:
And then sometimes it depends on what someone means by vertigo. It's become such a term that many people bunch in with dizzy and sometimes dizzy in other cultures might actually be description of just not walking right, or that they're just not feeling well or have a headache.

Dr. Nath:
Wait, wait. So like in Spanish, Daniel, I think dizzy is mareado, right?

Dr. Correa:
That's right.

Dr. Nath:
Does that also mean vertigo, or do y'all have another word for that?

Dr. Correa:
No, not really. And this is where different languages, there's lots of different dialects. And so in some areas you might have different determinations or descriptions of vertigo or spinning sensations, but there's not a consistent word that's used across South America, Caribbean, and Spain. So it's a perfect example. In other terms, it actually means that sense of spinning is what most people mean with vertigo, but when it's just not walking right, some people describe a sense of vertigo or dizziness when it's really actually the nerves out in their arms and legs that aren't telling their body what position they're in, or certain types of nerve damage that occurs in conditions like diabetes.
So Emily, unfortunately, there's no easy answer for intense vertigo overall. The first key thing is to work with a neurologist or even also an ENT doctor, an ear, nose and throat doctor in helping define and determine where is the problem that you're having and then working with medications. Occasionally there are some medicines that also cause a side effect of vertigo so that's another important place we have to look for.

Dr. Nath:
So essentially what you're saying about that whole process of seeing neurology and ENT and trying to get to the bottom of these symptoms is pretty much exactly what our guest went through. Victoria Vesce, so she was a Sports Illustrated model and an NBA dancer and in the best shape of her life and was having migraine-like headaches and dizziness just out of nowhere. And it ended up that she had a tumor. So we will speak with her all about how that came to be and how they figured it out and her journey and how she uses her massive platform to educate people about brain tumors and what it's like to go to radiation and also to advocate for more funding for research, which is awesome.

Dr. Correa:
It sounds like the perfect story to help us understand about really the importance of knowing what's causing the problem before just thinking about what's a treatment or an exercise someone can do.

Dr. Nath:
Exactly. For her it was not obvious.

Dr. Correa:
And then stay tuned. Upcoming in the following week, we will be featuring an episode where we interview US soccer goalkeeper Briana Scurry. So she's a legendary goalkeeper for all of you soccer fans. And if not, all you new fans stay tuned and learn more. That also is going to be in honor of Brain Injury Awareness Month. And after our discussion with Briana Scurry, we'll feature an interview and discussion with Dr. Michael Jaffee, the author of the Navigating Challenges of Concussion book out from the AAN in the Brain and Life book series.

Dr. Nath:
And Dr. Craig Stark will join us again on March 16th. Y'all may remember him from the Marilu Henner episode back in September. If you haven't listened then be sure to check it out. We will dive further into the topic of memory.
Today I have the honor of speaking with a true renaissance woman, Victoria Vesce. Many of you may have seen her work as a model in too many places to name, including Sports Illustrated and New York Fashion Week. She's also a professional dancer who is a dancer with the NBA for the Charlotte Hornets. And on top of all of that, she's a lawyer who got her JD from the Charleston School of Law, so clearly she's accomplished multiple lifetime level goals. But behind all of those accomplishments and all of the glitz and glamour, Victoria also battled with a brain tumor, specifically a paraganglioma and underwent surgeries and radiation. And she now functions with hearing loss. Thank you, Victoria, for joining us today to tell us about your story and your advocacy for brain tumor patients.

Victoria Vesce:
I'm so happy to be here. Thank you for having me.

Dr. Nath:
So I want to hear about this. I mean, I can't even imagine what your life looked like. You were doing so many different things that probably required a lot of traveling, a lot of physical activity when you were diagnosed with this brain tumor. So what happened? What was going on in your life?

Victoria Vesce:
I had a lot of things going on in my life. I was an NBA dancer for the Charlotte Hornets at the time. I had just graduated from NC State University. I was just living my best life. I had traveled to the Olympics with the NBA actually, in Brazil, Rio at the time. So I was doing a lot of heavy traveling, a lot of working out. I was super active, super healthy. So it was kind of crazy how all of this kind of came about.

Dr. Nath:
What did you notice first and what did you think was happening?

Victoria Vesce:
Well, I started having the migraines, but at the time, I will say I was going out a lot, so I was chalking it up to maybe some late nights out on the town. So I was chalking some symptoms up to that, and I was very tired. I had to take a lot of naps during the day for someone whose super active, it was very odd.

Dr. Nath:
So that was unusual, okay.

Victoria Vesce:
Yeah. It was just kind of unusual because I was very energetic and it felt like my energy was just going downhill very fast and started feeling nauseated a lot, dizzy. It was just very ongoing. The symptom that hit me the most was losing my hearing in my right ear.

Dr. Nath:
Oh, wow.

Victoria Vesce:
It was just a slow fade. And my friends would get so mad at me because they're like, "We're talking to you. Why aren't you listening?" I'm like, "I literally can't hear you." That was the trigger.

Dr. Nath:
They don't necessarily notice sometimes with unilateral or one-sided hearing loss. Sometimes people don't notice that so quickly. So you had enough people around you talking to you in different ways that you did pick up on that?

Victoria Vesce:
Yeah. I mean, it took a minute. I'm not going to lie. And I thought maybe my ear was just stopped up at the time.

Dr. Nath:
Which happens for a 20-year-old, that's more likely, right?

Victoria Vesce:
Yeah. The worst thing I'd ever gone through was a cold. So I mean, my brain isn't immediately going to, "Oh, this is a tumor." So I was like, "Oh, I just have an ear infection or something."

Dr. Nath:
So what got you to medical care from all of these symptoms that understandably you may have been chalking up to other things as a 23-year old who was traveling a ton?

Victoria Vesce:
Yeah. Well, I mean, I didn't really go to the doctor much. It was just kind of that was just me. And I started going to urgent care because everything was getting a little worse. And I'm like, "Okay, maybe I should go see the doctor." But I just went to basic urgent care and obviously it's something they've probably never even seen. So going in there, I kind of got misdiagnosed a bit, and then I just remember it was just a gradual process of me continually getting misdiagnosed then me getting even more sick. And I just remember my mom, who unfortunately has passed away, she was really like, "Victoria, come on, we got to go see a specialist. We got to figure this out because you're not yourself." My friends pinpointed it, my family, my mom especially. They're like, "You're just not yourself, so you need to go see a doctor, a specialist." And that's what prompted everything.

Dr. Nath:
Did you find a neurologist first thing, or who did you see, just logistically?

Victoria Vesce:
My ear was frustrating me because it was just like, "Why is it so stopped up all the time?" It felt really heavy on my right side. And so I immediately went to a ear, nose and throat doctor. And so that's who I saw.

Dr. Nath:
Okay, well, that makes sense. And then they got imaging. And how did you find out there was a tumor?

Victoria Vesce:
Actually, it took a minute. I remember going through a round of steroids, did not help. Then I went back for a hearing test and that's when I guess all the red flags came about with, they're like, "Oh, there's some nerve damage. That's not a good sign." So then we had a CAT scan and then they found the tumor and then referred me to Duke. So it was just like, that was probably a four-week process of just wondering what was wrong with me.

Dr. Nath:
Okay. You then go to Duke. Tell me what was going through your mind as all this happened being like this perfectly healthy 23 year old and now there's a tumor in your head that you have to go to a some specialist and your life is going to be in a medical gown, not on a catwalk? You know what I mean? That's just such a huge transition. How did you adapt to that?

Victoria Vesce:
I will say it was very dark. I had like Midas touch I feel like for the first 23 years of my life. Anything I did, I was pretty cocky, confident, and I hadn't had anything traumatic happen, drama here and there, but nothing as traumatic that hits you where you're like, "Is this the end?" So that was like, is this the end moment for me. And it humbled me real quick and grounded me. And I'm like, "I don't know what to do." My family didn't know what to do, my friends didn't know what to do. So it was like, who do I turn to? It was just a very dark period of time trying to get, I guess all the answers.

Dr. Nath:
I'm curious, I know now you work extensively with the National Brain Tumor Foundation. Were you in touch with any support groups for young people at that time? I'm just curious.

Victoria Vesce:
No, I was pretty prideful at that time. And I mean, I was just like, "We'll figure this out on our own." And also I was a little scared because I just didn't really, like, I wanted to know, but I also didn't want to know. It was kind of nice to be in a weird in between, but at the same time I was just on edge all the time and just didn't know what was going to happen to me.

Dr. Nath:
So walk us through a little bit about what did happen. There were surgeries, there was radiation. What happened next?

Victoria Vesce:
After I got referred to Duke, they did an MRI to get the extensiveness of it. It was literally the week of my birthday they found a tumor, my carotid body tumor, and it was just like, oh no, I had one, now I have two. How much more do I have?

Dr. Nath:
Oh, there were two?

Victoria Vesce:
Yeah, so it was in the brain and skull and then it was in my neck as well, and you can see my scar. I mean, if you could log onto my socials you can see my neck scar. I've never gotten that taken away because I think it's like my battle wound.

Dr. Nath:
It's part of your story.

Victoria Vesce:
People always ask about it when I'm out so it's kind of funny. I'm like, "Get ready for a long story."

Dr. Nath:
So for our audience, she's pointing to the right side of her neck where the carotid artery runs. And the carotid artery is extremely important to get blood to your brain. And if something were to cause the carotid artery to be disrupted or break that would be fatal essentially. So that's what Victoria was facing. Not only was it two brain tumors, but the location of them, which is often the big issue with brain tumors is not just the tumor, but where are they, were in a very dangerous spot right around her carotid artery. So that's what you were facing, which is huge. I think that would be hard for anyone to really wrap their heads around.

Victoria Vesce:
That was the first time I cried because the whole time I hadn't cried and I was like, "Okay, this is okay." You don't believe it's real. I remember he's like, "Are you okay?" I'm like, "Yeah, I'm great," and I remember getting in the car, I cried because I'm like, "How much more bad news can I take?" And it's okay to not be okay. It's not something you can take easily, especially when you never dealt with it before.
And then after that it was like go time we've got to schedule. It was hard because I was trying to finish out this NBA season, which I barely could. And then when they found the carotid tumor, they're like, "All right, we got to put a date in for surgery." And this was May 17th of that year. I was dreading the day. It was like doomsday because I was so scared.

Dr. Nath:
Were you scared that you wouldn't wake up from the surgery? I'm just curious what-

Victoria Vesce:
Yeah.

Dr. Nath:
Yeah, yeah.

Victoria Vesce:
At first you're okay till the day hits and then you're so nervous. And I remember they were doing the polymer beads through my groin so they could stop blood flow of the tumor. And that hurt because you have to be somewhat awake for that. And I just remember before getting wheeled back there, I was like, "How do I escape the hospital right now?" I was trying to plan my escape and I'm like, "Okay, now I'm scared." That was my whole body was shaking with nerves. And then after that got prepped and went to surgery. And I think I was in the hospital for like 10 days at that time. Some things came up and I had some spinal fluid leaking and then just like, it was just a whole thing after another and then an infection. I'm like, "Oh lord." That was a period of six weeks in and out, in and out, in and out of the hospital. And I mean, for that whole year I was in and out of the hospital.

Dr. Nath:
You had mentioned that you had been doing well. You had accomplished a lot, you had some pride, right? And looking kind of at your photos and things like that, you were clearly very skilled at looking perfect, right? I don't know anything about modeling, but clearly you did. And I'm just wondering if that played a role in how you took in this information and how you were able to express yourself. Do you think that your background and modeling and being this kind of quote perfection affected how you were able to express yourself?

Victoria Vesce:
They were planning to shave my whole head. I told the doctors, I'm like, "Look, I do not want to come out of here looking like my brother because we look very similar. I swear if I wake up and I'm bald, I'm going to get you. I'm going to haunt y'all." I told them that and they were dying laughing.

Dr. Nath:
Hair is a real issue with this. It comes up a lot.

Victoria Vesce:
Sometimes people say your hair is attached to womanhood, but for me that was like, I love my hair, I don't want to get rid of it That was my identity a little bit.

Dr. Nath:
So you bargained?

Victoria Vesce:
I bargained with them. They're like, "You've got a lot of hair." And they're like, "If we have to shave it, well, we're going to have to shave it." I'm like, "Okay." So they shaved half my head so I could put my hair over part of it. After that in terms of I guess appearance, because if you're thinking of modeling, it's more so appearance it was very humbling because I couldn't shower. I didn't shower for six weeks, I couldn't. It was really, really hard. I mean, they tried to bathe me and I would throw up. It was a very hard scenario of trying to balance being able to have just basic hygiene.
I couldn't go to the bathroom by myself. It was a very stark difference and very humbling and very mentally dark time because you're thinking to yourself, "Do I want to continue living like this?" Because I didn't know when it was going to end. I didn't know if I would get better quickly or not. But there was a period of time where I'm like, "I'm going to force myself to get better because I cannot live like this. I cannot." So that's when I really bucked up and I feel like my mind just changed and my mind really healed me.

Dr. Nath:
Yeah. You had that internal strength from somewhere, somewhere deep down.

Victoria Vesce:
People are like, "Oh, you probably always had that." Maybe I did. But it came out of nowhere when I was just like, "I can't live like this anymore." I was in so much pain.

Dr. Nath:
So you went through all of this and the radiation and you somehow clawed your way back to walking around and living independently. At what point did you think, "You know what? There's other people out there with this. I kind of want to reach out to the organizations and find other people and talk to other patients." When did that happen?

Victoria Vesce:
It was actually when I got better. It was at the six-week mark when I could actually take a shower and bathe myself. And I was ready to talk and I also was like, "How do I heal?" So after that six-week mark, and then when they told me, "Hey, you're going to start radiation treatment," because that took me for a loop. I didn't think I needed to do that, but I didn't know they left residual tumor because they had to.

Dr. Nath:
Because it was too dangerous to take out, right?

Victoria Vesce:
Yeah. It was too dangerous, yeah. And that's when I was like, "Hey, I want to reach out to someone because I'm going to be in the hospital again for another, like, a month and a half."

Dr. Nath:
You thought maybe you might be done, and then it was like, "Oh, just kidding. There's actually some still in there. You still have to do a whole other process."

Victoria Vesce:
Yeah, I thought I would maybe have to do like three rounds. That's what they were talking about. And then it went from 3 to 31 or 2.

Dr. Nath:
And wait, describe this to me. Radiation and each round of radiation taking like a week or something like that?

Victoria Vesce:
It would go Monday through Friday and I would go in the mornings.

Dr. Nath:
31 times?

Victoria Vesce:
Yeah, and it was during the summer when everyone's out having their best life and I'm sleeping 18 hours a day with my dog. So I was just sleeping a lot. I wouldn't say back to normal, but I felt more alive after the six-week mark because I really pushed myself. And then I'm like, "Great, I'm starting to feel better. I can do this." And then my doctor's like, "Just wait, it's going to hit you." And I'm like, "No, it's not. I'm going to be good." And then it hit me hard.

Dr. Nath:
And I did see on one of your socials that you showed pictures of the radiation process, which not a whole lot of people out there have huge followings and are showing what radiation looks like, but they made a mask for your face that's contoured to your face.

Victoria Vesce:
I still have it.

Dr. Nath:
You do?

Victoria Vesce:
I do. I'm trying to make it to work. I got to figure it out.

Dr. Nath:
Basically, yeah, that could contour to your face to keep you in the exact right spot to target the radiation to the exact right spot. And you did show this to people. What kinds of responses did you get to that? I'm just curious.

Victoria Vesce:
People would be like, "Are you claustrophobic?" I mean, because your head was in there and you cannot move. You can barely breathe. There's just a little nose hole and I really don't like tight spaces, but I knew I had to do it, so there was no getting out. And at first I didn't have any anxiety going in the little head case. But then when the days kept going on I started getting mad anxiety and you had to sit in there still for like 20, 30 minutes and breathe very soft because you cannot move.

Dr. Nath:
That's right.

Victoria Vesce:
It was pinpoint radiation that they were doing this study on me. And I mean, obviously it worked wonders.

Dr. Nath:
Because that's right, you can't take too deep of a breath because that would move your head slightly.

Victoria Vesce:
Yeah, and it had to be so exact. So it always made me pretty nervous. And if you see on my Instagram, maybe you go see the picture, it's always pinned on my Instagram. You may see a pretty face, but you don't know their story, so don't judge a book by its cover. And I always will keep that pin because that's part of my story.

Dr. Nath:
Yeah. So I would like to hear the transition from being in treatment every day and it's terrifying and you're exhausted and you're falling asleep with your dog. How did you transition from that to law school, which is extremely demanding and cognitively demanding? For one, what inspired you to do that? And then two, how did you get through law school?

Victoria Vesce:
I just needed something to look forward to honestly. I had to take all this time off, so I didn't really have anything on the horizon. I don't want to say, "Oh, law school was my last hope," but at the time that was what I had going. That was like the last thing I had done before I was in the hospital for months on end. So I had taken the LSAT and stuff and I had started applying to law schools, but I hadn't finished. And then I got hit with the brain tumor and carotid artery tumor diagnosis and I stopped applying because I didn't think I would do it. I had a scholarship to go, so why don't I go and try it out? So I tried it out for full-time for like a week. And then I told the dean, I was like, "Yep, I cannot do full-time." I was falling asleep in class. The lingering effects of radiation, it doesn't stop 24 hours later, it continues for a whole year. I was trying to get over those effects.

Dr. Nath:
Easily.

Victoria Vesce:
Yeah.

Dr. Nath:
That's why I was wondering. And there are part-time options for legal studies.

Victoria Vesce:
Yeah. So that was the thing I had to look forward to. And it's always nice when you're in one of those situations if you can, it doesn't have to be law school, but try to have something to look forward to because that helps you heal mentally faster at least. Because that was for me, I was like, "All right, if I have this on this day, I got to be somewhat to a point where I'm better."

Dr. Nath:
That's right. I mean, mindset and stress levels essentially, and how you view the future does affect healing in ways we don't completely understand. So essentially, I also wanted you to talk about that just for anyone out there listening to know that if there is a big goal that someone has that's now maybe dealing with some sort of disability, basically, that there may be ways to modify a study plan or an educational plan just so people can kind of hear that you are able to take a really, really daunting goal and do it with some modifications. And that's totally okay. You still got through it all.

Victoria Vesce:
Yeah. I think we have this idea that you have to do something a certain way, but there's always shortcuts or alternate routes. Even if you open Apple Maps there's alternate routes to your destination. So you don't have to go the fastest way, the quickest way or even the longest way, but you can find a way to make something happen for yourself. Super important to have some sort of goal. I was like, "In a year from now I'm going to be back to running three miles," and I was. So it was just these little goals you have to plan out or big goals you have to plan out. At least even if you don't get there and you get somewhere close, you're way better than where you started.

Dr. Nath:
So before we let you go, I want to hear a little bit about what you're doing with the National Brain Tumor Society and how people can find out more about your work with brain tumors.

Victoria Vesce:
Yeah. When I was going through radiation, I was like, "I need to reach out to someone." Somehow I found the Brain Tumor Society. I think maybe someone found me on Facebook and I just met a group of peers. It was actually two other athletes and after radiation treatment, we'd go eat or I'd try to eat and hang out, and we just talk about our tumors. And I was like, "This is nice to have someone who's another 24-25 year old going through the same thing and they can tell me how they healed." And it was just really nice so that time was very comforting to me. I just needed someone to talk to that went through the same thing.

Dr. Nath:
Absolutely.

Victoria Vesce:
And that's when I learned healing happens in community as well. And so having that community helped me heal. And when I was upset about something I could always reach out to them and they help a lot of patients and survivors and a lot of families. So I just love all the work that they do and that they helped me through a dark period in my life. So I've ran a fashion show benefit for them.

Dr. Nath:
That is so cool.

Victoria Vesce:
And then when my mom passed away ... My mom was super passionate about it as well. So instead of sending her flowers to the funeral home, we did like a fundraiser on Facebook, Instagram, social media, raised like 11K. But just raising awareness around these issues that there are communities that you can just reach out to because I didn't really know that until I was at radiation treatment or I didn't really look for it. But there's a lot of organizations out there. But for me, the National Brain Tumor Society helped me a lot and just raising awareness via social media.

Dr. Nath:
I just want to say thank you for using your platform that you have amassed from all the other things that you've done in your life to use for this cause and to give a voice to other young people with brain tumors that are going through what you went through and to fundraise. I mean, that is awesome and thank you so much for sharing your story with us here on Brain & Life.

Victoria Vesce:
I appreciate it. Thank you for having me on.

Dr. Nath:
Can't get enough of the Brain & Life Podcast? Keep the conversation going on social media when you follow @BrainandLifeMag or visit BrainandLife.org. As your hosts we would also like to hear from you on Twitter @NeuroDrCorrea and @AudreyNathMDPhD.
To learn more about nerve cell tumors, I caught up with an old friend from medical school, Justin Jordan, who is now an assistant professor of neurology at Harvard Medical School and the clinical director of the Massachusetts General Hospital Brain Tumor Center and the director of the Family Center for Neurofibromatosis. Welcome to the podcast.

Dr. Jordan:
Thanks so much for inviting me, Audrey. I'm happy to be here.

Dr. Nath:
Victoria told us about her journey with her tumor called a paraganglioma, which involves nerve cells and it was near her carotid artery, which is an artery that you don't want to have burst. And so it was a very, very precarious situation. What can you tell us about paragangliomas or nerve cell tumors in general?

Dr. Jordan:
Paragangliomas are a really rare type of tumor. They're not malignant, but clearly, as you can see here, the course may not be benign as was the case with Victoria. So these particular tumors arise from nerves in the autonomic nervous system, which control things like blood pressure and the function of your bowels and other things. I think this particular type of tumor really brings us back to remembering that there are so, so many different types of nervous system tumors, including the central nervous system in the brain and spine and the peripheral and autonomic nervous systems. All of those can unfortunately develop tumors from time to time.

Dr. Nath:
Just to make that clear, when we talk about the central nervous system or CNS, that refers to what Justin was saying the brain and the spinal cord. And then the nerves that come off of the spinal cord and into your arms and legs, those are peripheral nerves or the peripheral nervous system. And then there's also the autonomic nervous system, which is another set of nerves that helps regulate things like blood pressure. So essentially her tumor you were mentioning is non-malignant. The word I usually would say is benign. Is there a reason you don't use the word benign?

Dr. Jordan:
Benign is a pathologic term, which does mean that it's not technically a cancer, but it implies that things should be quick and easy for a patient. And as we know with Victoria and so many other patients that's not the case here. So the wording I tend to use is non-malignant, just to help demonstrate that the patients can still have their own experience, however benign it may or may not be.

Dr. Nath:
So basically non-malignant, like it's not going to spread and metastasize all over your body. However, if it's in the wrong place or a very inconvenient place, as was the case with Victoria, that still has a huge, huge impact on your life and can still be life-threatening.

Dr. Jordan:
Absolutely right.

Dr. Nath:
So essentially so many different types of neural tissue can grow abnormally and form a tumor. What are some other types of tumors that you see with neural tissue that aren't necessarily standard brain tumors?

Dr. Jordan:
Well, in the central nervous system, of course the most common types of brain tumors are things like astrocytomatas and glioblastomas. But really we think about non-neural tissues as well, such as the lining around the brain and think about meningiomas, which is the most common type of tumor to happen in the head. Peripherally, very common nervous system tumors include nerve sheath tumors like schwannomas and neurofibromas. And similarly, those are not malignant and they may or may not be particularly symptomatic for a given patient depending on where they arise.

Dr. Nath:
We had an episode with a neuro oncologist, Evan Noch, who himself experienced an acoustic neuroma and he talked to us about how yes, that particular tumor was not malignant, however it still needed to come out and he still sacrificed hearing in one side and there were still many, many ramifications from having that surgery from this non-malignant tumor. So to kind of tell our audience when cells grow out of control in ways that they're not supposed to and form tumors, those cells can still have the function that they had before, which is kind of weird to think about. And this can come up with what we call endocrine tumors. It always seemed strange to me when I was learning about this before because to me it seemed like, well, if they're growing out of control, how can they do anything useful, like make the thing they were making before? So sometimes people can present with symptoms that are resulting from the thing that that abnormal mass of cells is pumping out.

Dr. Jordan:
That's exactly right. So in the case of endocrine tumors like paragangliomas and their related malignant counterpart, pheochromocytomas, they can produce things like adrenaline, which is the usual day job of the cell of origin, the chromaffin cell that can go awry and create those chemicals even when they shouldn't be active. We see that with some types of brain tumors as well, where seizures may arise from tumors that integrate neurons for instance and can have overactivity of the electrical signaling in the neurons.

Dr. Nath:
What do patients normally tell you when they present with a neuroendocrine tumor? What sorts of things do they experience?

Dr. Jordan:
I'd say the most common things that we see for neuroendocrine tumors or paraganglioma specifically would be hot flashes that really can't be otherwise explained. That may present with flushing, with sweating, with palpitations, where the heart feels like it's racing in your chest. And patients often have periods of increased blood pressure as well.

Dr. Nath:
So those symptoms are fairly non-specific. There's a number of things that can cause those things. Does it make it harder to diagnose some of these entities or is there a delay to diagnosis that you see?

Dr. Jordan:
So there are some syndromes that predispose to these types of tumors and hereditary paraganglioma syndrome is one of those, as we've heard about. Another is neurofibromatosis type 1, which can have an increased risk of some of these tumors. So a physician examining a patient with one of those syndromes, of course, has to keep top of mind the risk of these endocrine tumors. More importantly for a patient who does not have a diagnosis of some underlying predisposition work like Victoria is doing to raise awareness is so important because it helps physicians keep top of mind these rare entities from time to time when patients have unexplained symptoms.

Dr. Nath:
So that we don't miss it because we think it's something else.

Dr. Jordan:
Absolutely.

Dr. Nath:
And we didn't absolutely put that on our differential diagnosis. So I have another question for you. I know you specifically study neurofibromatosis and that is a condition that can be inherited in families. What do you normally see when someone comes in with that?

Dr. Jordan:
So neurofibromatosis type 1 is a pretty common rare disease. It's about 1 in 3,000 individuals with the disease. It is a disease that presents over the course of the lifespan. So actually very early on children may have abnormal pigmentation on their skin where they have particular abnormal spots that are brown in color. They may have some abnormalities on their eyes and later in life may be at risk of developing non-malignant tumors on the surface of the skin, deep in the body. And unfortunately there are some malignancies associated with that condition as well. So it really spans the entire body and the entire lifespan.

Dr. Nath:
So what do you see in the future? I know this is not paraganglioma, but it's another type of inherited condition that includes tumors. What do you see on the horizon in terms of research and going forward, what may be available to these families either now or in the future?

Dr. Jordan:
It's actually a really exciting time to work in familial cancer syndromes. Our ability to understand the underlying biology that takes an abnormal gene all the way to the development of a tumor is just really in an altogether different place now. Actually in 2020, the FDA approved the very first drug specifically to treat neurofibromatosis type 1. The drug is called selumetinib, and it is a targeted agent that blocks a protein called MEK, which is responsible for the abnormal growth in some of the tumors associated with neurofibromatosis type 1.

Dr. Nath:
Wow, so they could give it to a kid with this condition theoretically?

Dr. Jordan:
Right, so the studies that have been done so far for this drug show that a particular tumor type called a plexiform neurofibroma, yet another nervous system tumor may shrink in 70% of individuals treated with this drug, so a really wonderful response rate. And not only is the FDA approval of this drug an important milestone in the history of neurofibromatosis type 1, but also it's really been a jumping off point for important, exciting new research for additional drug pathways that we can target and help patients.
And I think as this applies to Victoria as well, we know some of the pathways that predispose to paragangliomas. I told you that neurofibromatosis type 1 is one. There's also succinate dehydrogenase gene abnormalities that can predispose to paragangliomas. And so as we begin to understand the biology more and more, I think we'll have better opportunities for really targeted therapies.

Dr. Nath:
For anyone listening who may have an inherited condition, can you just kind of roughly tell us what happens when you sign up for a clinical trial or what sorts of different things are available?

Dr. Jordan:
Clinical trials are the way to advance science. So first of all, a huge thanks goes out every single time we talk about clinical trials for patients and family members being willing to sign up and join to help us advance science.

Dr. Nath:
Absolutely.

Dr. Jordan:
There are really sort of two big buckets of clinical trials. Those that are therapeutic in nature where we're trying a new treatment for a given disease and those that are non-therapeutic, where we're learning, we're observing, we're perhaps taking tissues from the operating room or blood samples and studying those in a laboratory. So in the non-therapeutic clinical trial space then I would say that many major medical institutions and the NIH actually have ongoing work to biobank samples from patients. And so whether you have no diseases or you have a very rare tumor predisposition syndrome, many entities, including the NIH are trying to get access to blood and saliva that they anonymize so that your privacy is protected, so that they can understand what's the difference between your blood and another person's blood who may be similar or different in terms of their overall health.
And for those patients who unfortunately develop tumors and may undergo surgery or biopsy, the willingness to share some of the tissue after the diagnosis is finalized is incredibly helpful for us to study in the lab and identify those pathways that make tumors grow and take that back for possible new therapies.

Dr. Nath:
Is there anything you'd like to tell any listener out there that's been told that they have a new tumor and it's something with a genetic basis and it's something that runs in their family? Is there something that you'd want them to know if you can't see them in your clinic?

Dr. Jordan:
I think the message that I always try to impart to my patients is the importance of a multidisciplinary team in caring for patients with rare diseases. In many aspects that includes a team of surgeons and medical doctors and genetic counselors or geneticists who come together to evaluate and determine treatments. I also think that patients should feel empowered to get a second opinion, especially when it comes to rare diseases.

Dr. Nath:
Absolutely, always. Yeah.

Dr. Jordan:
You can't expect that a given physician has seen an enormous number of whatever it is that you have, and so patients should feel comfortable to reach out to other doctors and even search online for doctors who may have particular expertise in their given entity.

Dr. Nath:
And what helps when doing that is if you can gather all the data that you've already had at one place, including scans and things like that and get copies and send it over, then a new fresh set of eyes can sometimes look at all of that data and put their thinking caps together and maybe come up with something different.

Dr. Jordan:
The other thing I'd like to say is Victoria has been such an amazing advocate for the National Brain Tumor Society, and I cannot applaud her enough or the other patients and family members who do this type of work. I think advocacy plays such an important role in advancing science and advancing research funding and really in paying it forward. Thankfully, Victoria is feeling well now, and I think that her advocacy and continued volunteerism with the National Brain Tumor Society helps other patients realize what they're going through will improve at some point, and she's really such a great ambassador for that population of patients.

Dr. Nath:
The vulnerability that she shows is something that really touched me. She showed herself getting her radiation and showing the mask and some of these little details that as physicians sometimes I don't necessarily talk about, but that are critical to the day to day operation for some of these patients. And for her to show that and for people to see it and think, "Okay, I can do that too," is just an awesome way for her to use her platform. Totally agree.

Dr. Jordan:
Yeah, absolutely. Just tremendous work on her part.

Dr. Nath:
Well, as a neuro oncologist for our listeners, can you tell us a couple of names of resources that you like to send patients to of national organizations that work with brain tumors?

Dr. Jordan:
Absolutely. So the National Brain Tumor Society is a tremendous organization that provides a lot of patient support as well as advocacy work for research dollars, and the American Brain Tumor Association is another one that patients can seek information and support from.

Dr. Nath:
We can put that in the show notes too. Thank you so much for joining us, Dr. Jordan.

Dr. Jordan:
Thank you very much for inviting me. This was a lot of fun.

Dr. Correa:
Thank you again for joining us on the Brain & Life Podcast. Follow and subscribe to this podcast so that you don't miss our weekly episodes. You can also sign up to receive the Brain & Life Magazine for free at brainandlife.org.

Dr. Nath:
Also for each episode, you can find out how to connect with us and our guests along with great resources in our show notes. You can also reach out by email at BLpodcast@brainandlife.org. And you can call in anytime and record a question at 612-928-6206.

Dr. Correa:
You can also follow the Brain & Life Magazine, Audrey and me on any of your preferred social media channels.

Dr. Nath:
A special thanks to the Brain & Life team, including-

Dr. Correa:
Andrea Weiss, Executive Editor for Education and News Publications.

Dr. Nath:
Nicole Lucier, Public Engagement Program Manager.

Dr. Correa:
Rachel Coleman, our Public Engagement Coordinator.

Dr. Nath:
Twin Cities Sound, our audio editing partner. We are your hosts.

Dr. Correa:
Daniel Correa. I'm joining you from New York City and online @NeuroDrCorrea.

Dr. Nath:
And Audrey Nath, beaming in from Texas and on Twitter @AudreyNathMDPhD.

Dr. Correa:
Thank you to our community members that trust us with their health and everyone with neurologic conditions. We hope together we can take steps to better brain health and each thrive with our own abilities every day.

Dr. Nath:
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Dr. Correa:
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Dr. Nath:
Thank you.

Dr. Correa:
Thanks again. See you next week.

Lawyer, Model Victoria Vesce Uses Her Platform for Brain Tumor Advocacy

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