Understanding the risks of rare bile duct cancer

Cholangiocarcinoma, also known as bile duct cancer, is a rare and aggressive form of cancer. Doctors say it is also extremely difficult to diagnose. Most bile duct cancer patients are 65 and older, and they often face a very poor prognosis. For more on bile duct cancer, we are now joined by Doctor Alexei Novikov. Doctor Novikov is an assistant professor at the University of Florida's Division of Gastroenterology. Doctor Novikov, thank you so much for joining us today. Thank you. It's my pleasure to be here today. Doctor, can you first explain what is bile duct cancer? About the cancer is essentially a malignancy. It's a neoplasm of the bile duct. It is a it's somewhat related to, pancreatic and gallbladder cancer. And yet it is distinct in a sense that it has a distinctly bad prognosis. The average survival of the diagnosis being of, gallbladder kilo of the cholangiocarcinoma is about 5 to 7 months. That's that's a terrible prognosis. Why is this, such a difficult disease to diagnose and to detect? Partly it has to do with the biology of, the bile duct cancer, cholangiocarcinoma. And, because of just because of how it is biologically different than special, it doesn't quite lend itself to, being seen very well in cross-section imaging, such as MRI and CT scans. You can still see it, but not all the cancers will be seen. And certainly early stages of cancer might not be seen as well. The other problem is that when we, you know, for us to effectively treat cholangiocarcinoma, we need to have, a sample that has cancer in it. And that is what I do for a living, an international endoscopy. And, we employ different endoscopic methods, such as endoscopic ultrasounds in the RCP. When we get inside the bile duct to diagnose this cancer of the bile ducts, normal standard sampling methods sometimes, oftentimes are insufficient. And again, it has to do with the fact that, cancer itself is pretty, and it's quite hard to explain. But this particular case, it doesn't have a lot of cancer cells in it. It has a lot of inflammation that surrounds it. It has a lot of, tissue that is made by inflammatory cells. But oftentimes what happens, the that the biopsies from inside the bile ducts, the brushes will come out non diagnostic or inconclusive. And that makes things hard because then the patients would need to do for additional, sampling procedures which delays the time from onset of symptoms or onset of concern to until, until the time that the effective therapy can be provided for this patient. Can you? That is two different reasons why it's hard. Excuse me. Can you explain what is the bile duct? For those that don't know what the bile duct is and what it does, bile ducts are it's a it's kind of hard to explain, but bile ducts. So you have a liver, right. And the liver is a, very important organ in metabolism in, recycling of, used red blood cells in, and one of the things that among many, many, many things that the liver does, it makes bile. Okay, once the bile is made, bile has to somehow be transported into the small bowel, because bile, bile itself actually plays a number of really important roles in digestion, in maintenance of your normal bodily functions. But the way to transport it and we're going to get to that is, in fact, through the bile ducts. And so inside the liver, right there is something called the biliary tree. And this tree, that's in it's exactly what it is. It looks like a little tree. It has little branches that kind of come together to form bigger and bigger, bigger ducts until you get to something called a, main duct or, common hepatic, that common bile duct and that, duct actually enters into the duodenum. And, that structure, the biliary tree is what, enables the liver to basically send the bile into the small bile where it's going to be used for digestion. Hopefully that explains it. It certainly does. Now, we know the, most of those diagnosed are over the age of 65. What is the link between older age and this particular cancer? Well, we find this is an interesting kind of, conversation. So let's just step back for a second because, you know, cholangiocarcinoma can be sub classified into different types. There's intrahepatic there's pre Cuyler and there's straight body cholangiocarcinoma. And there are basically kind of designated based on where the cancer happens either as it happens in the bile ducts inside the liver, right outside the liver, or really outside the liver. So, we generally speaking, you're right. It's the cancer of older folks. And it has to do something. We don't really know what happens and what causes cholangiocarcinoma. We think about sometime we we suspect that might be some environmental exposures. But, you know, generally speaking, cancer is a disease of older folks. And we do know that, recently we've been, there's, there's interesting data that, probability of having cholangiocarcinoma has been increasing on younger population, but it has been there is data only for the, intrahepatic cholangiocarcinoma that is, getting progressively younger, so to say. I guess we don't know what it is. It could be obesity, it could be environmental exposures. But we don't have a clear trigger or environmental trigger at this point. Is there something that we can do to reduce our risk of getting this kind of cancer? Not at this time. So there are people who need to be aware, you know, there is no such thing as screening for cholangiocarcinoma in the general population. And cancer remains to be, a relatively rare cancer. We will, you know, the, we think that we diagnosed about, 10,000 cases of cholangiocarcinoma in there. It's this per year. It sounds like a lot, but you have to remember that, the country has close to about 350 million people in it. And in the same time period, as you all diagnose 10,000 cases of cholangiocarcinoma, you will diagnose close to a million cases of colon cancer. So it is a rare cancer. That being said, there are specific population groups that will have a higher risk. Example would be people with primary sclerosing cholangitis, which is not the main disease of the liver, characterized by inflammation, chronic inflammation of the bile ducts. So these guys have a much higher risk of developing, cholangiocarcinoma, about 10% risk in a lifetime. But for general population, I would say that you have to pay attention to your liver health, pay attention to your liver numbers. And if your liver numbers also, we call them lfts are, elevated, that would be a time to be evaluated, by a specialist who can, adequately explain what's going on and if there is a concern to get appropriate high quality cross-sectional imaging, that can lead to an earlier diagnosis. So there's no specific symptom that would alert one that they could potentially have this. There are no specific symptoms unless it's longer or more. You know, the one thing that we always talk about is kind of like jaundice or the yellowing of the skin, yellowing of the eyes, in dark urine, that happens. And that is certainly a sign to be evaluated by a doctor. But it's not a it's not a specific sign, meaning that many diseases can give that thing, and, it does happen with cholangiocarcinoma, but it happens relatively, a later in the course of the disease. We don't have a very good way to detect this thing early. Doctor, is there a genetic predisposition to this? Is that something that has been studied? There are certain genes that there there are, genetic mutations that predispose one to cholangiocarcinoma. There are not, to my knowledge. And again, this is this is, something that I can't quite tell you right now. I don't think that there are specific genetic, markers. And I don't think that there is a familial association of cholangiocarcinoma, if that's what you're asking. But the cholangiocarcinoma does have a very specific molecular signature. And so one of the things that we do from our interventional perspective, when we go inside the bile duct and we sample the bile, that one of the things that we do at, you know, Florida is that we look at not just the how the cells look under the microscope. We also look at the genetic composition, of what's inside the cells to detect mutations associate of cholangiocarcinoma, even if, the actual cytology or sampling the cellular sampling from inside, that might not be conclusive. So that's one of the things that we do here that, you know, we not everyone does very interesting information. Where can we go if we want to learn more about keeping our livers healthy and and be cancer free? So, you know, specifically I think, there is a nonprofit called Cholangiocarcinoma Foundation. This is a great place to learn about the disease, about the community. It has lots of different, resources for survivors and for folks who have family members affected by the disease. It, it collects the money for research purposes. So I think it's a it's a great place to start to learn about cholangiocarcinoma in regards to figuring out, or to make sure that your liver is healthy, I suggest your best friend is your primary care doctor. Or a gastroenterologist or a hepatologist. And if you have abnormal liver numbers, that is something that needs to be looked at and carefully evaluated. If there is a suspicion for cholangiocarcinoma, I recommend going to a specialty to a center with a, special knowledge and expertise in treatment and diagnosis of cholangiocarcinoma. And there are several in the state of Florida. Because in order for you to get good diagnosis, you need to have a team of doctors who are used to dealing with this rare disease. Thank you so much for your expertise, your time, and joining us today, Doctor Novikov. It's been my pleasure. Thank you so much for inviting me.