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Tumor

CURE’s carcinoid cancer page features the latest cancer news and updates on carcinoid cancer Through articles, videos, podcasts, and more, CURE brings readers the insights of experts, cancer survivors, patients, and advocates to ensure everyone is up to date in carcinoid cancer.

A recent phase 2 study showed promising survival improvements with neoadjuvant Libtayo in patients with cutaneous squamous cell carcinoma after a year and a half follow up.

Libtayo Shows Survival Benefit In CSCC After Year Follow Up

The PAN Foundation today opened a new patient assistance program for people living with carcinoid syndrome.

The PAN Foundation Opens New Carcinoid Syndrome Patient Assistance Fund

Carcinoid Cancer Foundation: Spreading the Word About an Often Overlooked Disease

NORD was founded in 1983 by parents of children with rare diseases. Over the past 30 years, it has become a powerful advocacy voice for all patients with rare diseases, including cancer.

NORD Offers Information and Support for Patients of Rare Cancers

What is multidisciplinary care, and where can patients get it? 

Meetings of the Minds

 Maryann, you talk to a lot of patients. Do you think they are aware of all the treatment options, or do you feel like you have to educate them quite a lot, at least in the beginning?

 I think that they still need a lot of education out there. There’s still a lot of unknown for the patients. And if they’re using a local doctor, a local GI doctor, they might not have all the updated information of which scans to have done and which tests need to be done, what blood work and stuff like that. So, yes, the patients usually are the ones who bring them back the information. We send out the information packet and a lot of times they’ll call us up and say, “Can you send us another one? I brought it to the doctor and he’s very interested in the information you sent.” So, we’ll send them out to the doctors’ offices as well.

 That’s very interesting. So, Larry, tell me, what advice do you have for patients who are trying to manage their diseases since you’ve been managing it for now six years? We need some advice here.

 Well, obviously don’t get it on the first place.

 I don’t think that’s a choice, unfortunately.

 That would be my best advice. But recognize as early as you can after the diagnosis that it isn’t a death sentence, that it isn’t something that has to change the character of your life traumatically for the rest of it. Because it really doesn’t, at least in my experience. If you manage it properly, if you have very good medical care, if you follow the care and follow the treatment plans and do you part as a patient, then I’m optimistic about living a long, fruitful life with a disease. And it sounds as if Maryann is, too.

 Maryann, you’ve given advice to a lot of patients. So, what is your main advice that you think is really important for patients to know for trying to manage their disease? What do you tell them?

 The first thing you tell them is to take a deep breath, because I think everybody goes into a complete panic.

 Absolutely. Take a deep breath and just relax, because the more stress that you get, the more you’re going to have syndrome. So, we encourage them to relax. But we tell them, you need to find the right doctor for you. Not every doctor is the correct doctor for you, you need to find the right doctor. You need to find the right treatment and follow your treatment plan, making sure you get your colonoscopies, whatever you need to get done. Make sure you go and have your blood work done. Make sure you follow a plan. And with you following a plan and reaching out and getting support, you can survive. And it’s not even surviving, you live with this, you live with this disease. And you can live a good quality of life if you take care of yourself. And if you’re not feeling good, take the time to rest, but don’t stay in bed and lay around. Get up every day, get dressed, and try to be productive. Because if you lay down and you let this disease take over, those are the patients I see that lose their lives earlier, because they don’t want to fight. You need to get up and fight.

 Amazing. Dr. Morse, I’ll give you the last word here. For the patients out there, it’s a unique disease and you treat this unique disease. So, what do you think you would tell the patients out there?

 Well, we’ve actually heard that more eloquently than I can say it, but I’ll repeat what has been said, which is, it’s important for people to be educated about the disease to find the highest quality information, to bring that information to their other healthcare providers, and to stay up on the developments. Cancer therapies, in general, their development is accelerating and treatments that weren’t available even last year are, of course, available now. We’ll have some others available in the new year.

 Yes. We talked earlier about the lutetium-177 DOTATATE that we hope will be available as a therapy in the new year. This year we saw Xermelo, or telotristat—which you started taking and you started taking this year as well&mdash;that helped with the diarrhea. And if you read about things from the past, it doesn’t mean what the future is going to be like. You read that the survivals were five years or less, that was really based on old data. It has continued to improve and it will continue to improve. So, whatever you hear and read about today, it will be better as long you can keep yourself in good shape, and there will be other options coming down the line to help stay in good shape for as long as possible.

 So, educate, educate, educate, and advocate, right?

 OK. This has been a great discussion for the management of carcinoid tumors. Thank you, Dr. Morse, Larry, and Maryann for joining us today and providing your perspectives. And to our viewers here at 

Side Effect Management

Awareness and Neuroendocrine Tumors

 Larry, tell me a little bit—we talked about this before&mdash;about your triggers. Wine was a trigger and you said some cheeses. How did you go about eliminating your triggers? Did you go, “Oh, this cheese, not good; this cheese, good”?

 Process of elimination. I’d try something and if it triggered a flushing episode, I wouldn’t try it again. And that’s basically the way it worked.

 So, what all are your triggers? You said some wine, some cheese.

 Wine; alcohol in general. In fact, I’m very sensitive apparently to that as a trigger.

 No tequila, no vodka in the bottle. I don’t even use mouthwash with alcohol.

 Because I try to stay completely away from it. The most difficult part for me is the spicy food. I’ve noticed that I flush often after I eat spicy food, and I love spicy food.

 I do. So, that has been the part that has been very difficult to manage.

 Stress-related things—exercised-induced, emotional. I do not drink wine or alcohol. I will have a beer occasionally, that doesn’t seem to bother me as much. I do occasionally use the rescue shot if I want to have a glass of wine or champagne; at my children’s wedding, I did. But otherwise, I really don’t drink alcohol. Cheeses, I don’t really have that much of a problem. Sometimes I do from tomatoes, so I try to stay away from tomato sauces and things like that.

 Very interesting. You did the same thing, a process of elimination?

 Yes, process of elimination. And you know what, with this disease, you never know exactly what’s going to set it off, each person is so different. One thing I do have to stay away from is turkey.

 So, no Thanksgiving, huh? What do you have on Thanksgiving?

 We have pot roast. So, two years in a row, before my diagnosis, I ended up in the ER with severe abdominal pain and diarrhea and flushing and everything else. And we never put two and two together. And they said, “Oh you must have passed a kidney stone,” and I’m like, “I only pass a kidney stone on Thanksgiving? Kind of weird but OK.” And then later on, I found out that the turkey with the tryptophan turns into serotonin, which set me off. And so, I do not eat turkey unless I have a rescue shot.

 So, what do you think, Dr. Morse? Does your team work with patients like Larry, like Maryann about self-management of symptoms? Because I think that seems to be a really important link in this disease.

 Yes, absolutely. It’s important that people be their own advocates, as we’ve been discussing, and part of that is understanding the cancer, understanding how it affects them and what the triggers are, and what they can do for themselves. And, in fact, we rely on that because I can’t pretend to know what some of these symptoms are going to be. It’s actually fairly common for people to downplay them—I think we all do that. And certainly, when we go to see our healthcare professionals, we don’t want to bother people with our issues. Say everything is OK, that last treatment you tried worked great, many people probably don’t bring up the severity of their symptoms, and so we just assume everything is OK.

So, if somebody doesn’t bring up certain things, usually I go through my mind a list of side effects and symptoms that we want to discuss with people. And it’s also important that people’s other physicians know a little bit about their disease. You can self-educate and you can educate the other healthcare providers. For example, we talked about carcinoid crisis before. It’s absolutely critical if somebody is going to have surgery and has carcinoid syndrome that the surgeons and the anesthesiologists know that.

Most of the time, if you have a major operation and people have taken a detailed health history, they will probably find that out. But what if you go to the dentist and they haven’t necessarily taken a health history, or you haven’t even bothered to mention certain things when you go? So, it’s important to have that so people aren’t given treatments or have stimuli that might bring on a carcinoid crisis.

 Maryann, I know you talk to a lot of patients and everything. Do you think patients are reluctant to talk to their doctors about their symptoms or not tell them everything because they’re like…?

 I do, I do. I always try to encourage them to open up and tell everything to the doctor. I think that they really should keep a diary of what’s going on, because when you get into that room, the doctor walks in and he only has that three or four minutes. Even with the nurses, they’re afraid to open up and say what’s going on. So, I always tell them to take a diary, keep track of how many times you go to the bathroom. Because I think what they think about is what happened in the last 48 hours, not what happened during the whole month. And so, that’s really critical. Like I said, if I had maybe mentioned earlier to my doctor about the fevers being in the afternoon…

 It was actually flushing. But after several years of having fevers, you don’t take your temperature anymore. You go into the closet and you take a Tylenol. And so, maybe if I had said something to him, it would have been picked up a lot earlier. So, I was encouraged then. And the other thing I always encourage patients to do is also to be mindful of your body. If you feel something’s not right, make sure you mention it to your doctor because a lot of the time they’ll assume that it’s something to do with carcinoid when it might not have anything to do with it.

 It might not have anything to do with carcinoid.

 And they might see something else going on. I’ve had patients where they assume that everything was their carcinoid, and then later on they find out that they had a secondary cancer and it actually was colon cancer. So it’s very important that you tell them everything. Is there any change in your diarrhea? Are you having flushing? Or, are you having heart palpitations? Are you making sure that you go for your echocardiogram? We’ve had patients where they’ve had 2 heart valve replacements before they were diagnosed with the carcinoid. So, it’s very important that you tell the doctors ahead of time and also be an advocate when you’re going for surgery. Make sure everybody knows the history and that they properly medicate you.

 I try, but I’m not very good at it. I tried doing that early on and I settled into a routine where I just try, frankly, to remember the symptoms that I encounter. Now if I encounter something unusual, then I’ll make sure that I make a note of it.

 And by this time, Dr. Morse probably knows what to ask, in a way.

 Well, what is helpful though about having a diary, or at least somebody who can put things together, is individual events are very difficult to put together in a story and to know really how somebody is doing. And a diary or just somebody who can say, “This is what has happened over the month,” allows me to understand better the course of the disease and so I know when we do need to intervene and when we don’t need to really make any changes.

 So, what you’re looking for is a pattern, essentially, of things that may keep on happening.

 I think that’s the way humans think; when we can group things, when we can find patterns, we can understand them better.

Carcinoid Syndrome: Symptom Triggers

 Let’s move on to our next segment, which is really what we’ve talked about: taking ownership of your disease. Maryann, Larry, both of you took the disease with both hands and ran with it. So, how important—Maryann, I’ll come to you first&mdash;is it to take ownership of your disease, be an advocate for the disease, maybe join the Neuroendocrine Cancer Awareness Network?

 Right. I think that, in the beginning, a lot of patients are scared, but I always tell the patients that they need to ask questions. The doctors might not know everything, try to get as much material as possible and bring the information to them. Each patient is different, each doctor is different. Find a doctor who will work with you closely, and open communication is the most important thing.

It wasn’t until after my diagnosis that I went back to my hematologist. I run fevers at night from the lupus. And I started running fevers during the day, or which I believed were fevers when I actually was having a flushing. Now had I mentioned that to the doctor when he would ask, “How are your fevers?” And I’d say, “I still had them.” Had I said to him, “Well, they’re starting to come during the day,” maybe I would have picked it up a little sooner. He said, “Why didn’t you tell me? I could have noticed.”

 It’s very important. And a lot of patients think, “Well, I have this disease, I don’t have to worry about anything else.” Just as any other person in the world, you’re still prone to prostate cancer, breast cancer and everything else. Just because you have this cancer, it does not discriminate you from having any other cancer. And most important is that you have to be your own advocate. Make sure you go for your blood work, go for your CAT scans, go for your MIs, go for your Gallium scans. Make sure you stay on top of it. Don’t let it go.

I met a patient who had a Whipple procedure and her doctors told her she was cured, and I kept on saying, “You really need to be followed up.” And she’s like, “Nah, everybody said I’m cured.” She went and it turned out that she had another tumor, it was originally slow growing and it turned into a faster growing tumor. Had she not gone for her scans, she might have not been here. So, it’s important to stay on top of it, don’t wait five years to have the scans done. Go.

 What’s the regular follow-up for patients, Dr. Morse? Everybody’s different but do you have a path that you follow? I know every doctor is different in that, too.

 The patients who have had primary surgeries and the cancer is thought to be gone, I agree, still need some follow-up; we would say early on. And there are some guidelines out there that can be used but it’s six-month intervals—CT scans or MRI scans, particularly of the area where they have the tumor and the liver. As time goes on, you can stretch it out to yearly. Because they’re so slow growing, some guidelines would suggest following people for 10 years, perhaps longer if you have reason to believe they could still relapse that far out. Somebody with known metastatic disease spread to the liver or elsewhere and with off-and-on therapy, if we’re actively treating, if the disease is changing, we may image it three to four months. If people are stable, it’s typically about 6-month intervals. And again, we prefer, because of their simplicity, CT scans or MRI.

You did bring up a new test, the Gallium-68 DOTATATE test. It’s important that people know about this test because it’s done as a CAT scan and as a PET scan together, but it’s not the typical PET scan that most people have heard of. And I think we’ve had scenarios where people came to see us and they said, “Well, I’ve had a PET scan,” and we’re trying to understand what PET scan did they have? They probably had the more typical one, which is called the FDG-PET scan. Those will only light up in the high-grade poorly differentiated tumors. They’re generally negative in the lower grade tumors.

The older test that had been done for lower-grade neuroendocrine tumors was called OctreoScan. The problem with OctreoScan is it has very poor resolution and you can miss lesions with it. Certain parts of the body, such as the liver and spleen, will light up on that test anyway and it’s a little bit difficult to differentiate tumor from background. Of course, it’s excreted into the urine so the kidneys and the bladder will have high uptake. With this newer type of PET scan, Gallium-68 DOTATATE, it’s very specific for neuroendocrine tumors and it’s very sensitive as well.

On the other hand, because it’s new, many insurance companies don’t seem to understand what it is, and there have been some challenges. And I think this is definitely where a person needs to be an advocate and if you get one denial, it’s try again. I had a recent scenario where we’re finding very often now that insurance companies are now hiring other companies to assess whether they should pay for the imaging. You and I spend hours a day dealing with this sort of thing. And it’s important to say, “This is new, it doesn’t go into the usual rubrics of the type of tests—the PET scans, the CTs&mdash;it’s different. And if they don’t pay for it today, if they get enough education, they’ll probably start paying for these because it’s a better test.”

And the hope is that in each person, their scenario is going to be different, but that we find a regimen that’s affordable, that’s easy for the person to comply with. It gives us the most information. Because if we can find it early recurring, as you pointed out, you can jump on it early and hopefully when it’s a smaller volume of disease.

 So, it’s care for individual patients. It’s not one-size-fits-all.

Follow-Up Care for Neuroendocrine Tumors

 One of the things we talked about—maybe Larry and you could talk about&mdash;is that these doctors who talk about it are specialists, like Dr. Morse, in this disease. They spend a good amount of time figuring about this disease, what it is, how the symptoms are, what are the specific symptoms, syndromes, etc. So, how important do you think it is to see a specialist? Like you’ve got in with Dr. Morse within a week. How important do you think it is for patients to find somebody who is a specialist in this disease?

 Well, I believe it’s critical, I really do, in rural areas especially. Many general practitioners don’t have the experience to deal with this particular type of cancer; I know this, I live in a rural area. So, if you really want to get the treatment that you need to try to control this disease and maintain your quality of life, you need to be able to reach out to a place and to people who can offer you world-class care.

 I think it’s true. I think a lot of patients don’t realize that sometimes you can go to the specialists and they will work with your local oncologist. I think that every patient at some point in their life needs to go and see a specialist, somebody that treats this disease, because local oncologists don’t have the time. It’s not their fault. They’re treating dozens of different diseases and we are rare. And a lot of times there may be only one or two patients who they’ve ever seen in their lifetime and so they really don’t have the time to dedicate it. So, at one point during your lifespan, or in the journey, you should see a specialist and then have them work with your local oncologist and get the treatment that you deserve.

 As Larry said, he probably knew more about his disease than some of the doctors he saw. Now Dr. Morse, that brings up a very important point. Not everybody can go to Duke. Not everybody can just get up and say, “I’m driving to Duke.” They could be living hours away, not in the same state, because there are not many doctors like you around. So, do you communicate with a community oncologist? Is that fairly common? How do you deal with this situation for this special disease?

 Right. There are many opportunities for us to help out local oncologists. Often people will come, even if it’s for 1 visit, so we can understand their unique case and what issues are critical to focus on in them. But many times, people take the rest of their care until there’s a new juncture in their disease with their local oncologist.

I think there are some areas that we can, in particular, point out that people aren’t always aware of. Some of the symptoms are long-term complications, like carcinoid heart disease for example. It’s not something that people necessarily think about. We focus on this as being a GI tract disease with prominent diarrhea and the flushing. But silently, there can be valve damage going on.

 Yes, and I’ve even seen people near the time of diagnosis who already had some elements of valvular damage and didn’t know it, and they weren’t quite symptomatic enough where somebody would think about their heart. So, that’s a scenario where we can make a recommendation. We’ll make sure the person has an echocardiogram at baseline, and make sure it’s checked periodically.

 And to check on the heart, check on the bowels. What kind of symptoms do people have if they have this heart valve disease from this disease?

 Well, first of all, we hope people don’t get to the point of being symptomatic. Symptomatic means, typically, the valve is on the right side of the heart. The tricuspid and the pulmonic valve are the ones that are affected. And if they’re severely damaged and scarred up, you have what’s called valvular regurgitation—blood goes in the wrong direction when the heart is pumping. And so, typically people have exercise intolerance. They just can’t do the activities that they normally could. They start swelling because the blood’s not being pumped forward as efficiently.

 It’s in the legs, in the belly, or around the lungs. The point is people don’t get adequate forward blood flow and so it backs up, so to speak. And it can severely affect quality of life and eventually can be lethal if the right side of the heart eventually fails. We obviously want to get to people before that happens. In some cases, people need valve replacements. And if somebody has had a valve replacement, it’s important to protect that valve so they don’t need another valve replacement in the future. So, what we currently do is try to get an understanding of how much serotonin the person is making. We talked earlier that although you can measure serotonin levels in the blood stream it can be…

 It can fluctuate quite a bit. And the better test is the metabolic breakdown product of serotonin 5HIAA, typically done in the urine. There is a way of doing it in the blood now, but most people have it done in the urine. And if we find people have particularly high levels, and even if they’re already on a somatostatin analog at that point, we take that very seriously because the very high levels are associated with a greater risk of having carcinoid valve damage. And fortunately, we can measure that. We can do the echocardiograms and decide if we need to intervene to try to control their serotonin more or make sure they’re seeing a cardiologist to understand when valve replacement may be an issue.

 So, a lot of little nuances. It seems like this is true, every patient is unique in this. Larry was unique, Maryann, you were unique in your symptoms, so…

 That’s one of the reasons why we chose the zebra as our logo and as our awareness, because no two zebras have the same stripes. And a lot of people believe that in medical school, the doctors are taught that when hearing hoofbeats, think of horses not zebras. Look at the common, not the uncommon. It’s very easy for the doctors to assume that when you’re having a lot of diarrhea or anything like that, the first thing they say is IBS; it’s easy. But we’re uncommon.

And the second reason is because no two zebras have the same stripes. There are no two NET patients or carcinoid patients who are exactly the same. We all respond differently; we all have different symptoms. It’s not a cut-and-dried situation. Like most cancers, after five years, you’re either gone or you’re released from your oncologist. You are a lifetime patient with this. And there are many of us who are out there 15, 20, or 30 years. And so, a lot of times, it’s treated as a chronic disease. But there are so many facets to this disease. It’s very difficult, it’s very frustrating sometimes for the patient as well as for the doctor.

 So, when they call you at night, is it mostly patients who’ve been diagnosed or somebody who’s having the symptoms and they go, “Well, I’m not really sure what I have”?

 I would say 90 percent of our calls are from patients who are already diagnosed and are concerned. And I would say 10 percent of the time we do get calls from people who say, “My doctors have exhausted everything and they’re saying they’re not really sure, could you give me a list of what bloodworks I should be getting done? Maybe we can pick up something soon.” And so, we do. I always encourage patients that if they’re going for the colonoscopy, go ahead and get a pediatric scope, because…

 Do the big one but do the small one, definitely.

 Yes. I’m very grateful for my GI doctor because if he had not, I really don’t know where I would be today. And since he has found mine, he has found six other patients using the pediatric scope in the last 16 years. So, it does work.

Neuroendocrine Tumors: Expert Care

Tell me a little bit about your advocacy about. You told me before you were organizing conferences. Tell me about the growth of the organization, I’m fascinated.

Carcinoid Cancer

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