Advances in Appendiceal Cancers Webcast April 20, 2009 Andrew Lowy, M.D., F.A.C.S. Jennifer Ambrose. Introduction

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1 Advances in Appendiceal Cancers Webcast April 20, 2009 Andrew Lowy, M.D., F.A.C.S. Jennifer Ambrose Please remember the opinions expressed on Patient Power are not necessarily the views of UC San Diego Medical Center, its staff, our sponsors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. Please have this discussion you re your own doctor, that s how you ll get care that s most appropriate for you. Introduction There are common cancers like colon, lung of course, and prostate, breast, and there are very rare cancers. A one-in-a-million cancer diagnosis, if you will, is cancer of the appendix. You re about to meet someone who experienced that and a physician at UC San Diego Medical Center who specializes in it, and you ll get the latest information all coming up next on Patient Power. Hello. I m Andrew Schorr. Welcome to Patient Power sponsored by UC San Diego Medical Center, UCSD. One of the really rare cancers is cancer of the appendix. It s said it can even be a one-in-a-million cancer diagnosis. Well, at UCSD Medical Center they have experts who specialize in the treatment of it, and that can really be lifesaving getting to the right expert and getting the appropriate care for this very rare condition. Jennifer s Story As always we like to begin with someone who s experienced that. Jennifer Ambrose is 35-years old, lives nowhere near San Diego. Jennifer, you live in New Lenox, Illinois. Is that right? Yes it is. About 30 miles south of Chicago I believe, and so you live there with John, and going back to April 2007 you had young Ryan who was what, about 7 or so? Yes. Okay, but something started going on in your health. What was that? 1

2 I just wasn t feeling too good. My grandfather was dying, and the stress was really taking over me, and so I was urged by my husband who doesn t really like doctors too much to go see what was going on. So I had gone to my primary. She sent me for an ultrasound, and they found that I had some fluid in my abdomen, which was very not normal. So they said they wanted to take a deeper look. They went and sent me for a CAT scan. At that point they had found a mass in the lower right quadrant of my abdominal cavity, but they weren t exactly sure where it was. So I went in for surgery on April 20 th of 2007 with my general surgeon, and he did kind of like an exploratory thing to see what was going on and take a sample of the fluid. When he had gotten in there he had noticed that there was a tumor on my appendix, and the tumor now had ruptured, not my appendix, just the tumor had ruptured, and it was a mucin-producing tumor, which caused my whole entire abdominal cavity to fill with this mucin, and this mucin kind of just reproduces and grows and gets more volume as time goes on. What did they tell you they could do for that? My surgeon wasn t too familiar with it because it s so rare. He had heard of it, but he really didn t know what direction to point me in. He gave me a name, and I had kind of researched a little bit, but I was not too sure. Let s mention that you have a bit of a medical background. You were trained as a phlebotomist, somebody who, all those blood tests. So you started searching, and you found out that at the University of Cincinnati at the time there was an expert, right, Dr. Andrew Lowy. Yes. So you went looking for Dr. Lowy only to find that he was moving to California. To California. Cincinnati is not too far Chicago, and California is quite a ways, a plane ride. But in the end you connected with Dr. Lowy, and he thought he could help as a sort of super-subspecialist, a surgical oncologist. So you went out there with the whole family and grandma too, right? 2

3 Grandma, my mom, my husband, and my little boy, we all went out there. I know you ve told me previously, the story s incredible, is that when grandma got to the airport she fell and broke her hip. Absolutely. So she was in the hospital and, as we ll hear, you were in the hospital On the same morning, actually at the same time, we were both having surgery; her on her hip and me on my abdominal cavity. And we should just mention, and San Diego s a very hospitable place, but as you arrived there that the place that you thought you d be staying, a house that you were renting for the whole family, wasn t anything like you thought, and you ended up sleeping where? In a WalMart parking lot our very first night. My poor little boy. He said, Mom, I don t like it in California. Can we please go back home to Chicago? But we should say that while it got off to a rocky start things changed dramatically. Dr. Lowy, who we re about to meet on this broadcast, did do surgery that he ll describe. So with this cancer throughout your abdomen and now it s spring of 2009, how are you today? I m absolutely wonderful. I am just delighted and healthy and happy, and I have a 9-month-old little boy to celebrate. Right, so let s see if we understand. So you had this abdominal surgery, and what happened two months later? I got pregnant. 3

4 Oh my, well, it s an amazing story. So that s 9-month-old Cole. That s 9-month-old Cole. That s correct. What a great story. Well I think it s probably appropriate now to meet this eminent surgeon that you were chasing after from Ohio to California and points in between, and that s Dr. Andrew Lowy. Yay. Yay. Dr. Lowy is a surgical oncologist. More than that he s a Professor of Surgery, and he s Chief of Surgical Oncology at UC San Diego Medical Center. Dr. Lowy, it must just make you feel great to hear that a woman who is a powerful patient was able to connect with you, and the result is so good. Yes, her story is obviously very inspiring, and she has a great family, and we were delighted we could help her. Let s understand now. So I have never done a program on cancer of the appendix before, so it really is quite rare, isn t it? Yes it is. As Jennifer mentioned her physician hadn t heard of it, and that s actually pretty common because the government keeps statistics on certainly all the common cancers, but the statistics on appendix cancer because it s rare and more elusive, and most of the estimates are that there is roughly an incidence of one in a million persons, but that number is, it s hard to know how accurate that is. Suffice it to say it s rare enough that many physicians never do see a case of it either in training or during their career. Diagnosing and Treating Appendiceal Cancer Wow, all right, well let s talk about that. So she had this fluid, and it sounded like mucus on her abdomen. I ve read about it a little, and it said that that s been referred maybe in some of the medical textbooks or other articles written referring to it as like jelly belly. Is that it? It s just the cancer, like she talked about it, sort of bursting in a way and spreading. What s it like? 4

5 I think it s important for the audience to, you know, everybody has heard of the appendix because appendicitis is so common, but many people don t really know sort of what the appendix is, where is it, etc. It s basically a little structure, and it s about the size of your index finger in most people, and it hangs off of the first part of our large intestine. It has no recognizable function or purpose in us at this point, and so really the main thing it can do for us is cause problems, and the most common problem is appendicitis. But like any other organ or tissue in our body it is also susceptible to becoming cancerous. Many cancers of the appendix tend to form from, the best way I think to understand it is a structure that s like a polyp, which is what many people are familiar with in the colon. It s a little growth that starts out as cells that are growing but not cancerous, meaning they can t spread or invade into other tissues, but many of these polyp-like structures in the appendix tend to overproduce mucus. Now our whole gastrointestinal tract from our stomach all the way down through the small and large intestine produces mucus. It s normal. It helps coat the lining of the bowel, helps our food slide down easier, but these cells for whatever reason, reasons we don t understand very well, sort of become little mucin, mucus factories. They overproduce mucus and secrete it outside of the cell and essentially form what you can think of it s almost like a water balloon type of structure where the cells are lining what would be the balloon, and the inside is filled with this mucus or jelly-like material. What happens is Jennifer really well described, eventually it s gets big enough, tight enough, and just like a water balloon it can burst. What happens when it bursts is all that mucus and the cells get spilled into our abdominal cavity. In some patients those cells have a capacity to live in the little bit of fluid that s normally in our abdominal cavity, and they sort of get swept along like they were seaweed or something on the ocean and wash around the abdominal cavity, and they can continue to divide and continue to produce mucus, and that s sort of how they cause problems. All right. Let s understand what you do about it. So Jennifer came to you, and she had surgery, and I know the surgery was at least nine hours. What do you do? Now we ll discuss other patients, and I know people vary, but in Jennifer s case what did you do? I don t think her surgery was quite that long, but that is about the average length of the operations that we do for this condition. So as I said, the tumor cells get into the abdominal cavity, and essentially once they do that and have quite a bit of time to wash around they can basically set up shop anywhere on any surface in the abdominal cavity, and our abdominal cavity obviously has all of our abdominal 5

6 organs; our liver, pancreas, stomach, all our bowel; and so there s a large surface area. There are a lot of places for those tumor cells to sit, to hide. So in doing the operation the goal of the operation is to eradicate all of these cells. That obviously can be a very tall order because in some patients there are literally billions of cells present. So the first thing we have to do is we have to be able to see the whole abdominal cavity, which extends from the diaphragm all the way to the pelvis. So we make an incision which goes essentially from the lower part of the breastbone to the top of the pubic bone, which allows us to open the abdomen up. This is not what we call minimally invasive or some of the surgeries we can do now through a laparoscope. Essentially these operations have to be done more the old-fashioned way with a large incision where we can access the whole abdominal cavity. What we do, and what we did in Jennifer s case, is we systematically examined all the areas of the abdomen looking for evidence of the tumor as well as the mucus. Then the goal is once we find it to get rid of it, and depending on which structures and surfaces are involved that will dictate what operation or operations we are actually doing. All right, so in some cases, and fortunately it wasn t in Jennifer s case, there might be some structures as you say or organ you d have to remove. Correct, and that s very common that we would have to remove segments of intestine, large or small intestine, other organs like the gallbladder or our spleen may have to be removed, and what s very common is that the lining of the abdominal cavity; we all have a wallpaper that sort of covers the muscular part of our abdominal wall. A muscle is what holds all our organs where they are, but on the inside there s actually a lining over that muscle which literally is sort of like wallpaper, and that s called the peritoneum. That lining often gets studded with these tumor implants, and the best way to get rid of the tumor is to actually just sort of strip that wallpaper off. That s the way I usually describe it to patients because it s the easiest to understand. The problem with if you try to scrape the tumor cells off, just like if you were trying to get paint off wallpaper, you d always leave some stuff behind, and our goal in trying to cure patients is to not leave anything behind. So the easiest thing and the best thing to do is to essentially strip the wallpaper off because then all the tumor comes along with it. Now there s another part of the procedure too. Correct. 6

7 And that is, and people fighting cancer understand this, is some sort of cancer killing drug therapy. Most people are used to it thinking that you have it as an IV over many months, or sometimes there are oral cancer medicines, or even now there are some injectable ones, but you use chemotherapy during surgery. Tell us about that. Correct. So the idea here is that obviously we can see tumor that s of a certain size with the limits of our vision, usually a few millimeters, and so we can take out all the visible tumor, but obviously we can t see cells themselves that are floating around. You can only see that with a microscope. So even when we remove all the visible tumor pretty much in every patient there is going to be some amount of invisible cells that are still remaining in the abdominal cavity. In order to try to get at that tumor that we can t see we use chemotherapy. Now the problem with this tumor as we said is that it often just sort of floats around, and if it floats around and can survive in floating fluid, then it s not attached to our blood vessels in any way. So if we gave chemotherapy through the vein as is typically given for most cancers, those floating cells won t ever see the drug, and so it doesn t really work. So the idea here is to essentially pour the chemotherapy right into the abdominal cavity where it will contact all those cells and all the surfaces of the abdominal cavity. It can seep into those cells very efficiently when the tumors are very small or when there s only cells and get to the tumor cell that way. In conjunction along with the chemotherapy we use heat to heat up the fluid that the chemo is in because it s been known for really over a hundred years that cancer cells are more sensitive to heat than are our own body cells. They seem to have a defect in their ability to repair themselves after they get injured with heat. So by using chemotherapy that s in fluid heated to a high temperature, usually around 105 to 110 degrees, we can more effectively get at killing those cells, and as you mentioned, the advantage is that this is done all while the patient s asleep under anesthesia, and so when they wake up they ve had both their surgery and their chemotherapy all done at once. Wow, this is quite a story. We re going to take a quick break, and when we come back we re going to talk more with Jennifer Ambrose about her recovery from this procedure and also with Dr. Andrew Lowy from UCSD Medical Center about how approaches vary by patient and how people can connect with him as well given that there are so few centers where there are experts such as Dr. Lowy and his team who treat it. We ll be back with much more of Patient Power sponsored by University of California San Diego Medical Center right after this. 7

8 Recovering from Appendiceal Cancer Surgery Welcome back to Patient Power; I m Andrew Schorr; as we continue our discussion with Jennifer Ambrose from Chicago who found out she was diagnosed with a cancer she d never heard of, and that was a cancer of the appendix, and then she went to great lengths to get expert care that has proved right for her, and she ended up at UC San Diego Medical Center and with a renowned expert in the field, Dr. Andrew Lowy, who is a surgical oncologist. He s also Chief of Surgical Oncology at UC San Diego Medical Center. Jennifer, so Dr. Lowy in our last segment described the surgery and the chemotherapy during the surgery, and all this while you re asleep. So you wake up. Tell us about your recovery because it sounds like it s a pretty big deal, but how was the recovery? How quick was it? How did you feel? How long did it take you get to enjoying the sights of San Diego, if you will? Actually I feel very blessed and very fortunate because I m one of the patients that happened to heal very quickly. My recovery was very quick. It was very tender and painful at first; I m not going to lie to anybody; but I recovered quite quickly. I was only in the hospital about six days. I was in ICU for about hours, I believe, which is uncommon. Dr. Lowy, I believe, said usually it s 1-2 days, and I remember the following morning I was sitting up in my bed, and I had a tube in my throat, and I was waving trying to say hello, and he said to me, he goes, You re not going to be here very long are you? And I said, No. And I got moved to the floor, and I remember people telling me that when you have surgery like this you start walking as soon as you can. So I did. I got up, and I started walking, and within six days I was released. A couple of hours before I was released, the last thing to come out of me was I had a chest tube in place, and that was the biggest sigh of relief. She said, Wait about two hours, and you should be able to go. And I was on my way. Let s talk about being on your way. You had your whole family with you, and I know that they all wanted to get out and about San Diego. Did you go down to the beach? We did. Actually I was actually in the ocean swimming ten days after my surgery. I had had my staples removed, and that day we went down to the ocean, and I was actually in the water. Now mind you, my back was to the waves, not the front, but I was in the ocean, and it was amazing. We did some sightseeing. And you had a decent place to stay? No more WalMart parking lots? 8

9 We did. We were at the one of the Marriott Hotels. It was more of like a studio apartment type thing, and they took very, very good care of us. I think the medical center helped arrange your accommodations too. They did, they did. They put us in contact with people at the Marriott, and they were just absolutely wonderful. So how do you feel now? You said earlier you feel great? I do. I feel really good. I m back to normal. Okay, let s talk to Dr. Lowy more about it. Dr. Lowy, so Jennifer s story obviously is a great one, and we know in cancer what the treatment approach is varies by someone s situation and also their recovery may vary as well or quite frankly in cancer whether they re able to recover. So help us understand beyond Jennifer s situation. It sounds like there are times when there are things you need to remove or the cancer may be more advanced, and I know maybe there are even different versions, if you will, of appendix cancer. Help us understand the variety of it. Yes, so there are definitely, as there are in all cancers, different varieties, different stages, etc., of tumors. The less aggressive the cell type is the better the outcome is, and that s pretty much true for any cancer you can name, and Jennifer certainly had a favorable cell type, and her disease was caught relatively early, which made it easier for us to eradicate all the disease, and it made the surgery somewhat less invasive, but nonetheless it s still a big operation no matter how you slice it; she can certainly attest to that; but certainly other patients present with either tumors that are more aggressive or tumors that have been present longer and present with larger amounts of disease, and in those cases each patient has to be individualized, and we have to assess what in particular about their situation dictates whether they should have surgery, whether they should have surgery first. There are times when we do chemotherapy first, more traditional chemotherapy, if we think that an operation may not be able to be performed or be able to be performed adequately. Our main goal and the main thing that affects a patient s prognosis is our ability to remove surgically all of the visible tumor. It s very clear that those patients have a much better outcome, and the thing that generally determines that is how much of the small intestine is involved by the tumor because the small intestine is a vital organ in that we can t remove all of it. We can remove parts of it, even significant 9

10 parts of it, but you can t get rid of all of it because that s what absorbs our nutrients. So in some patients, particularly in tumors that are a bit more aggressive, the small intestine may be involved and may be involved extensively. If that s the case that s where we become limited in our ability to deal with it surgically, and we have to use other tools like chemotherapy to try to shrink the tumor or control it, and sometimes we will do that ahead of surgery and then if we re successful in shrinking it a bit we can go in and do an operation. As I said, each operation is different. It s always dictated by what s there, and it varies quite a lot about how much we may or may not have to remove. Is there any genetic connection or any cause for this that we know about? That obviously something that every patient asks particularly patients like Jennifer who have children or young children who want to know is my child going to be at risk for this? One of the limitations of our knowledge comes from the fact that it s a rare disease. So we don t have big studies to look at that question, but the fact that it s a rare disease alone suggests that there probably is no clear genetic link or predisposition. So as far as we know there is no genetic abnormality that predisposes patients to this tumor. There have been some recent suggestions by some researchers that perhaps its related to a bacterial infection, something called Helicobacter, which is associated with the risk of stomach cancer, but that is at this point a theory and is definitely unproven, and so really we just need research to try to understand these things, but the short answer is at this point no clear evidence of any genetic predisposition to appendiceal cancer. All right. Now we recognize it s rare, so somebody may well decide, and I would recommend it, to go see someone like yourself skilled in it and who is experienced in it. So do you at UCSD welcome people coming from afar like Jennifer did? Yes, a large number of our patients come from outside the State of California, and so we re happy to see anybody who wants to come and see us for an opinion. We also sometimes do consultations essentially by phone these days because it s sometimes hard for people to travel, and we don t want patients to have to travel here and then to find out that they re not eligible for treatment and then they ve incurred a lot of cost and time, so we will even review records and x-rays if that s what works out best for the patient. Let me mention for anyone who wants more information on Dr. Lowy s practice and surgery for appendix cancer or even any of the services at UCSD Medical Center. Here s the phone number to call: UCSD (8273). 10

11 Signs and Symptoms of Appendiceal Cancer Okay, let s back up for a minute then. What are the signs and symptoms that someone may have? We heard what Jennifer described, but how would you even know? For appendix cancer it is as many other abdominal cancers are, it can be very subtle. The most common presentations tend to be either pain on the right side of the abdomen, which seems to be a sign that somebody has appendicitis, and so it s often misdiagnosed initially as appendicitis. The patient will have an operation presuming that their appendix has to come out because it s inflamed and then the tumor is found. That s a common presentation. So obviously if you have abdominal pain and certainly if that s anything severe that s not going away that needs to be evaluated. Another common presentation, particularly in women and particularly women who are a little bit older than Jennifer is just the increasing size of the abdomen, sort of the girth is increasing, and that obviously happens in men too, but in women it will often get evaluated oftentimes by a gynecologist since they do a lot of primary care for women, and there s often a suspicion that this is ovarian cancer, and that s because ovarian cancer is more common than appendix cancer and also because it also tends to produce fluid and metastasize/spread into the abdominal cavity. Also appendix cancer when it spreads sometimes will spread to the ovaries, and so when the evaluation happens they will see something on the ovary and it s often misdiagnosed as ovarian cancer and then found subsequently to be appendix cancer. So those are the more common presentations. I actually have several women, younger women, who ve undergone a fertility workup with they re having difficulty conceiving a child and get a laparoscopy where a scope is put in to look at the ovaries, and lo and behold they see that there s tumor. So that s been another way that this has been diagnosed, but I would say pain and increasing abdominal girth or the size of the abdomen are probably the most common ways that this presents. Risk of Recurrence All right, one other question just related to Jennifer from you her doctor is I know she s asked you millions of times. She s feeling good. So the fear though is for any cancer patient; I m a leukemia survivor; that for any of us is that it could come back. So you used the C word, and I mean cure at one place, so tell us about how whether someone like Jennifer can be cured or someone s worry about it coming back. 11

12 Sure, well that obviously is always the concern, and it s hard to, you know, nothing we say can convince anybody or we can never be absolutely sure; however, in a circumstance like Jennifer s we know from other patients that have been treated in the past that there s a very good chance of cure and that we expect that somebody like her has more than an 85% chance of being free of disease five years after her operation, and since she s already past a year from the time of her operation, about a year, then the risk of recurrence has gone down even more. So there s even probably a greater than 90% chance that in five years she ll be doing just fine. But we always have to watch. We always have to be vigilant. I always tell patients unfortunately the cancer cells don t sign any contract that after a year or two years they re never going to come back, so we have to watch and particularly in patients who have slow-growing tumors we actually have to watch longer because if the tumors grow slow it may take them a little longer to show up, so we tend to watch instead of just for five year we tend to watch out to at least about ten years, but as the time passes the risk of it coming back continues to go down, and we have to look less and less, and I think as I m sure you know that the more time that passes the less patients tend to think about this, and it just sort of comes up when you have to have your doctor visit, but I fully expect Jennifer is going to do very well, and I do expect her to be cured. Yay, that s good news, huh Jennifer? Absolutely. Closing Comments Jennifer, so for patients, so you connected with Dr. Lowy. What would you recommend to listeners so that they could be well informed? Where did you look for information you found was good information? I started my search on the Internet. I mean it s the most resourceful information people have these days. The only other thing that people have is word of mouth. People are powerful people, and when you speak you speak in volumes, and one of Dr. Lowy s patients actually spoke to me and speaking through her is how I really felt empowered to go along with what I was doing and researching and following up and learning as much as I can and not giving up and just keep pushing forward. Right, you re a powerful patient right now for so many others. Jennifer, what s your outlook on the future? 12

13 I just want to enjoy my family, and I just feel like I have a second chance at life. I feel like Dr. Lowy is an angel to me. I feel I ve been given life again by him and through him for doing what he does and for him being there for me, and I just feel as though my life is just going to continue on and just be even better now because we also have a new baby. Do you want to say thanks publically here? I m indebted to you. I don t even have words to express my feelings to you how my family and I feel about you. We love you. Wow Dr. Lowy, that s gotta make your day. It certainly does. It s what it s all about. I should mention that part of Jennifer s address is livinlife. I really love that Jennifer. So Jennifer, when you look at little Cole, and he has joined your family after all this when as this started you didn t know whether there would be an after, that must just be a reminder of this second chance at life. Absolutely. It s funny you mention that. It s like the other day my husband and I were lying down, and we were just looking at him, and, Are you really ours? Am I just baby-sitting you? It just hits you in so many different ways, and for years I never thought I d be able to have another child, and then surprise, surprise. Well congratulations to you Jennifer. We want to wish you all the best. Thank you so much. And you are a powerful patient. I want to thank you for sharing your story here today. Thank you. 13

14 And Dr. Andrew Lowy at UC San Diego. So the country tilted and you moved down to California, and it was a great addition there, and we want to thank you for all the work you do and being at a major research institution now and also with your specialization in appendix cancers, I know it will draw people from far and wide, and it sounds like it s working out well for you, and I want to thank you for being there, Dr. Andrew Lowy who is Chief of the Division of Surgical Oncology at UC San Diego Medical Center, and I m delighted that we could do this program. I m Andrew Schorr. Thanks to UC San Diego Medical Center for helping make it possible. Remember, knowledge can be the best medicine of all. Thanks for joining us. Please remember the opinions expressed on Patient Power are not necessarily the views of UC San Diego Medical Center, its staff, our sponsors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. Please have this discussion you re your own doctor, that s how you ll get care that s most appropriate for you. 14