Preventing and Treating Hereditary Colorectal Cancer
Just like traits such as eye color or height, an increased risk to form certain types of cancers can also be genetic. Approximately 5% of all colorectal cancer cases are hereditary, and Dr. David Liska, Director of Cleveland Clinic's Sanford R. Weiss, MD Center for Hereditary Colorectal Neoplasia, joins Cancer Advances to discuss these cancers and how the multidisciplinary team at The Weiss Center is dedicated to preventing, treating, and educating patients and their families.
Preventing and Treating Hereditary Colorectal Cancer
Dale Shepard, MD, PhD: Cancer Advances at Cleveland Clinic Podcast for medical professionals, exploring the latest, innovative research and clinical advances in the field of oncology. Thank you for joining us for another episode of Cancer Advances. I'm your host, Dr. Dale Shepard, a medical oncologist here at Cleveland Clinic, overseeing our Taussig Phase I and Sarcoma Programs. Today I'm happy to be joined by Dr. David Liska, Director of the Sanford R. Weiss, MD, Center for Hereditary Colorectal Neoplasia. He's here today to talk to us about the Weiss Center and hereditary colon cancers. So welcome, David, tell me a little bit about your role here at Cleveland Clinic, as we get started.
David Liska, MD: Thank you so much for having me. So I'm a Colorectal Surgeon here at the Cleveland Clinic. I've been here now about seven years and I'm mostly the director of the Weiss Center, as you mentioned. The Weiss Center is dedicated to taking care of patients with hereditary colorectal cancers.
Dale Shepard, MD, PhD: Just as a backdrop, what kind of cancers would that include? So when you talk about hereditary colorectal cancers, it's normally one of the short list of questions that people ask me, is this hereditary? Tell me a little bit about what that would encompass?
David Liska, MD: Right? So in general, about 5% of all colorectal cancers are hereditary and what that means is that, these individuals have hereditary colorectal syndrome, usually because they inherited an abnormal gene from their parents and this gene predisposes them to develop colorectal cancer and frequently, this is also associated with other types of cancers, that can arise in different organs. And this is a little bit different of what we call, familial colorectal cancer.
We know that colorectal cancer in general, can run in families, even when you don't have a gene that predisposes you to that condition. However, what we're talking about when we're saying hereditary colorectal cancer, is those patients that have a known genetic syndrome, and those are at very high risk for colorectal cancer, even higher than the ones who have familial colorectal cancer.
Dale Shepard, MD, PhD: So these are typically people, who know that they have colon cancer, a very, very high risk. These aren't necessarily people who stumble into colon cancer. Is that correct?
David Liska, MD: It can be both, often, especially if they're part of a registry, like the one we have at the Weiss Center. Then these are families that know that they have this gene running through their families and they'll know ahead of time that they have the gene, so that we can prevent cancers from happening or detect them very early. However, there are some people who do kind of stumble into this, where they get a diagnosis of colorectal cancer, and then because of different things that are part of the family history or part of the cancer history, we suspect a hereditary cancer and then test them, have them see a genetic counselor and get genetic testing, which then confirms the diagnosis. So it can go both ways, in terms of knowing ahead of time, if you have hereditary syndrome or not.
Dale Shepard, MD, PhD: So we'll talk a little bit about the Weiss Center itself, here in a couple of minutes, but you mentioned other cancers and other sort of associated cancers. What sort of cancers would people be thinking about? They see a patient, they have other cancers, when would they think, "Hey, maybe this is hereditary, colon cancer."
David Liska, MD: A lot of times, some of the hereditary colorectal cancers are associated with polyposis, meaning that you developed lots and lots of polyps throughout your colon and other parts of your intestine. And if somebody has a colonoscopy that shows that, that would make us immediately think about hereditary colorectal cancer syndrome. Another thing would be, if they have multiple cancers in their colon, if you have colon cancer on the left side of the colon and on the right side of the colon, that would make us think maybe, you have hereditary colorectal cancer syndrome, or if you have a very strong family history of different cancers or different colon cancers that run in your family.
And lastly, like you said, if you have different cancer, for example, if you have uterine cancer, so cancer of the uterus, plus a colon cancer, not necessarily at the same time, but during your lifetime, that will make us think of a certain colorectal cancer syndrome and will prompt us to refer the patient to one of our genetic counselors to talk about getting genetic testing.
Dale Shepard, MD, PhD: So, we mentioned the Weiss Center. What is the Weiss Center? Give us a little background about what that is and what patients could expect through the Weiss Center?
David Liska, MD: Yeah, so the Weiss Center, it's made up of a large team of providers from different disciplines and specialties, and we're all focused on the care of patients and the families who have, or at risk for colorectal cancer syndrome. Patients and their families are enrolled in a hereditary registry, which was founded about 40 years ago by Dr. Jagelman, whom the registry is named after. And at this point, it's one of the largest registries of its kind, in the world.
We follow different conditions, like we discussed. Different conditions that are hereditary colorectal cancer syndromes, including polyposis and non-polyposis syndromes. And by being part of this registry, patients are being followed very closely, patients and the family, so we can detect cancer early or prevent cancer from happening, and not only focusing on one specific cancer, but focusing really on the patient as a whole. And our goal is to prevent cancer, while at the same time, optimizing the quality of life of patients.
And besides just our focus on providing the best patient care, we also focus on advancing the field of research and education, educating patients and physicians, because these are conditions that not everybody has expertise in. It is our privilege to be able to educate patients and other providers, how to best take care of these people.
Dale Shepard, MD, PhD: And these education efforts, are these primarily aimed at patients or families or their support groups or through conferences? What sort of education?
David Liska, MD: Well, a little bit of everything. So obviously, every time we meet a patient, the education part is very important, because many times these are individuals that are part of a family orientation, but they don't actually have a cancer yet. So there's a lot of education there, in terms of how can we sort of be part of the person's life and making sure, that without interfering with normal life, we can still make sure that we prevent cancer. And then we have a yearly Hereditary Health Day, where we have families come to hear from different families. We have shared medical appointments, where we have sometimes, multiple patients with a similar diagnosis, come together to allow for an interactive experience. And we have conferences that are aimed towards physicians, and we participate in many national and international conferences to be able to share our findings from the registry, with other centers, as well.
Dale Shepard, MD, PhD: Wow. So it's pretty broad. You mentioned multidisciplinary care. It's certainly, as with most cancers, this seems to be something that would be particularly important. Who's on the team? What sort of team members do you have in the Weiss Center?
David Liska, MD: Again, we are very fortunate to have a large team and I would be amiss to not say that, probably the most important team members are our coordinators. We have three dedicated coordinators, whose primary objective is really, just to make sure that this whole process is as seamless as possible for the patient, but also that nobody falls through the cracks. And we are very lucky, we have coordinators who have been with the registry a long time. They know each patient, like I just need to mention a name of a patient, they know exactly who they are, they know the entire family. And they develop relationships with our patients and really make sure that they get to see everyone they need to see.
And again, we have people coming from far away. We try to make sure they get to see all other members of the team that they need to see in the same day, or maybe within two days, to make it as easy of a process as possible. And the other members of the team, so we have several surgeons, both colorectal surgeons, upper GI surgeons, gynecologic surgeons, endocrine surgeons, neurologic surgeons. We have a large group of gastroenterologists to perform endoscopy and advanced endoscopy procedures. We have genetic counselors that are very important for the team, given that this is hereditary condition, and we have other medical specialties such as, breast cancer specialties, pulmonary specialists. And we are very fortunate to have a large team of experts who really have very specialized expertise for these conditions.
Dale Shepard, MD, PhD: You mentioned a couple of times, patients and families, and certainly with a hereditary condition, that's important. So when you say family, what kind of breadth are we talking? Are we talking immediate family? How extensive does that family reach go, in a condition like this?
David Liska, MD: As extensive as we can get. We start obviously, with the person that has a cancer, has been diagnosed with a cancer, and ideally, they would get genetically tested. But if they didn't or are not interested in that, then we can test the children as well. And then we go the first degree relative, so usually they're at highest risk, but we follow a family tree and different types of conditions are inherited in a different fashion, but we go as far as the gene carries, really. We have families, that we have cousins and nephews, but usually the first degree relatives, are the ones that are in immediate risk, at first.
Dale Shepard, MD, PhD: I'm going to take a step back here. So we've talked before about, you said something about registries. So we have a diverse group of people who listen in. What is a registry and what can you do with a registry? So registry versus study, how does that get defined?
David Liska, MD: The first colorectal cancer registry started actually, in the 1920s in England at a hospital at St. Marks. And one of our colorectal surgeons who trained there back in the '70s, he came back here to the States and started our own registry here back in 1979, that was Dr. Jagelman. And what a registry is, it allows us to prospectively, going forward, follow these patients and their families, making sure that they're part of a service that we provide, where we follow them and make sure they get all their testing done.
It's not just for research, which is different from a study. A study is for research, where we enroll patients for research and then study outcomes. This is a registry where we use it for clinical care as well. Yes, research is important too, but it's more for the clinical care of the patients because these patients are at risk for cancer in the future. So we can make sure they get the testing they need, at the time they need, so we can prevent any advanced cancer from forming.
Dale Shepard, MD, PhD: And I think it's important, you mentioned prospective. So you get to collect what you want to collect, which is different than a retrospective look, where you are at the mercy of what might've been collected?
David Liska, MD: Exactly, exactly. And the research component, that's a very important part of our mission at the Weiss Center. But again, these are rare conditions, so there are very few patients that have these certain conditions. And by being able to have all this data and information and learning which treatments are best, we have the opportunity to advance the field for all patients that have that condition. And that's another really important part of the registry, like you mentioned. That we can collect all this data, but also, have the opportunity to try new trials, new medications potentially, that can help us prevent cancer, as well.
Dale Shepard, MD, PhD: Thinking about scope, how big is the registry? So rare diseases, rare sorts of conditions, but you've been at it for a really long time. So how big is the registry?
David Liska, MD: Right. So in terms of overall patients and families, we have over 10,000 in the registry. Now, people with a confirmed genetic condition, about 4,000 to 5,000, at this point. Depending on which, again, some of them are rare, so certain diseases, we have maybe 60, 70 individuals and others we have, familial adenomatous polyposis, which is probably the one we have the highest number in our registry, we have close to 3,000.
Dale Shepard, MD, PhD: So when we think about rare diseases, outreach is probably something important, to try to capture as many cases as you can. How do people get to you? Is it mostly people that go on the web and they see about you? Is it doctor referrals? How do people come to the center and know about you?
David Liska, MD: So again, because we treat families, not just individuals. So there's some natural growth to it because family members keep getting added to the registry, just by being related to someone. So if you get diagnosed with a hereditary condition, like I said, part of it is the education and then we encourage our patients to reach out to their family members to get the appropriate testing. And then if they are interested, can also become part of our registry. But other than that, as being one of the few registries in the country, there's outreach to positions throughout the country and people who know us in the field and they'll refer to us those patients, obviously from regional centers, but also from the entire country and some, even international patients.
Dale Shepard, MD, PhD: So you're certainly following through the registry, a lot of people. What does this model look like in terms of, you mentioned the importance of the coordinators and keeping track of people along the way in their course. Are these primarily things, where people will get surveillance scans here, or is this more of a coordination of care out where people live and just making sure they get connected with the right resources? What does that look like?
David Liska, MD: It's a little bit of both, and it depends a little bit on how far you are. We have specialists here that are unique in their expertise and if you need a complicated endoscopy or complicated surgery, those would be the patients we would strongly encourage to come here to have those procedures done. Anything that can be done in an outside center and can then can be sent to us for review, we do that. And now, especially since the start of the pandemic, we've all gotten, and including us and our patient, we've all gotten very comfortable in doing virtual visits and we do a lot of these too.
So there's a lot of stuff we can do remotely and virtually. However, there are certain things that require special expertise for which our patients do come here. And again, like I said, we have our coordinators who really make sure, that if somebody does need to come here in person, that we make sure we do a one-stop shop, so to speak, where they can see all their specialists and have all the procedures done within the same day.
Dale Shepard, MD, PhD: Perfect. You mentioned the ability to follow people over time and see maybe, an intervention and what has happened and you also mentioned, trying to prevent cancer. So what kind of research questions have come from the center? What kind of things have you guys been able to come up with answers for, that have changed the field?
David Liska, MD: There've been a lot of advances in the surgical treatment and me as a colorectal surgeon, those are some of the questions I am very interested in. Because again, the goal is not only to prevent cancer, that's obviously, our most important goal is to prevent cancer and to cure cancer when it happens, but also to make sure we focus on the quality of life of the patients. So the question, for example, if somebody with familial adenomatous polyposis, meaning that the colon makes thousands of polyps, that all have a chance of turning into cancer is, how much do you have to remove? Do you have to remove all of it? Can you leave some of the rectum, if you do have to remove all of it, what is the best way of reconstructing it, so the patient still has an excellent quality of life?
And those are the questions that have been addressed and looked at for the last 20, 30 years. We've had some incredible advances in the field of surgery, in terms of minimally invasive surgery, but also, determining which patient can have what type of surgery to maximize both quality of life and minimize the risk for cancer? And there's still some ongoing questions there that we're looking at and that we're answering.
Besides the surgical care, there's also a lot of interest in chemoprophylaxis, meaning, what medication can we give our patients that are at high risk of colorectal cancer, to reduce the risk of colorectal cancer? And there's some very interesting and some encouraging trials out there, for different patients and different diseases, in terms of medications that can prevent cancer from forming. There's still a lot of research that needs to be done and again, because these are rare conditions, it can take some time.
Dale Shepard, MD, PhD: Is it possible that some patients may be spared surgery, as a result of those interventions?
David Liska, MD: That would be our goal. It depends again, on the type of underlying condition you have. And our goal would be ideally, to prevent the need for surgery.
Dale Shepard, MD, PhD: What's the biggest gap that you're waiting for that next big breakthrough?
David Liska, MD: Probably the biggest gap that would be chemo prevention for FAP patients. We're all looking for medications that can prevent the growth of polyps and prevent these polyps from turning into cancer. And they're having some encouraging trials, at this point though, still most of our patients do need surgery, but we are wondering if there are certain medications that we can develop that can reduce the amount of surgery they need, both in the lower GI tract and the upper GI tract and that would be something very important. As you know, Dr. Shepherd, desmoids are a major problem for familial adenomatous polyposis and medical treatment for those desmoids is still not as good as we would want it to be and that's definitely something we're also very interested in.
Dale Shepard, MD, PhD: Yeah, that's great. So what's the best way for people to reach out to you and be able to get their patients involved with the Weiss Center?
David Liska, MD: Yeah, so we have a website, go to the main, Cleveland Clinic website and Weiss Center and we have our one website. There's an appointment number that goes straight to our coordinators, which is 216.444.6470. There's an email address, [email protected] So there's many different ways. If you just call the general, Cleveland Clinic appointment line, you'll also get to us. So there's many different ways to get to us. And we really encourage everybody to reach out, even if they don't know if we are the right people to take care of them, we would be happy to work that out with anybody who calls us.
Dale Shepard, MD, PhD: Well, it sounds like you're doing some outstanding work and I appreciate your insight, any additional comments.
David Liska, MD: One other thing we're working on at the Weiss Center now is, there's been a lot of stuff in the media recently about early onset colorectal cancer, where it's not only hereditary conditions, but we find that even without hereditary conditions, young people more and more, are developing colorectal cancer for somewhat unknown reasons. And as part of the Weiss Center, we have now established a center, dedicated to the care of patients with early, onset colorectal cancer, and that's a topic in itself.
We're also dedicating ourselves to really provide care for these younger people, meaning younger than age 50 with colon cancer, which like I said, is really an alarming increase we've seen in these patients and we're trying to focus on the care of these patients with special needs they may have, and also focus on research, in terms of figuring out why this is happening and how it can be prevented?
Dale Shepard, MD, PhD: Yeah, that's a really, really important problem. The guidelines recently changed to 45 for screening, but that's really not going to help the 20 and 30 year olds that are coming into my clinic with metastatic disease. So I appreciate your efforts to try to sort out, what might be in play and try to identify those patients. Well, very good. Well, I appreciate your insight and thank you very much for joining us.
David Liska, MD: Thank you so much for having me.
Dale Shepard, MD, PhD: To learn more about the Weiss Center or to refer a patient, please call 216. 444.6470 or visit clevelandclinic.org/weisscenter. This concludes this episode of Cancer Advances. You will find additional podcast episodes on our website, clevelandclinic.org/canceradvancespodcast. Subscribe to the podcast on iTunes, Google Play, Spotify SoundCloud, or wherever you listen to podcasts. And don't forget, you can access real-time updates from Cleveland Clinics Cancer Center experts on our Consult QD website, at consultqd.clevelandclinic.org/cancer. Thank you for listening. Please join us again soon.