Transcription:
Sarcoma Awareness

Bill Klaproth (Host): Sarcoma is an uncommon group of cancers which arise in the bones and connective tissue such as fat and muscle. Treatment may include surgery, radiation and chemotherapy. Here to talk with us more about sarcoma is Dr. John Kane III, chief of the melanoma sarcoma service and chair of surgical oncology at Roswell Park Comprehensive Cancer Center. Dr. Kane, thank you so much for your time today. Can you go into a bit more detail of what sarcoma is and who is at risk for sarcoma?

Dr. John Kane (Guest): Sarcoma is actually a very interesting type of cancer. Sarcoma actually means “fish flesh” so that was the original description of the way it looked. A good way to think about sarcoma is that they are cancers of the glue of the body. As you had mentioned, they arise from muscle, bone, tendon, nerves, fat, connective tissue and all that things that hold people together. Because of that, those cells really aren’t dividing. It’s a very rare cancer, so it’ll probably be almost 13,000 sarcomas this year in the United States. They represent about 1% of cancers that adults get a little bit more common in children, mainly because children don’t tend to get breast cancer or colon cancer and things like that. The vast majority of them are actually bad luck. That one cell became abnormal, went almost from a normal cell to cancer in one step, probably 10% of them – a small subset of them – would be due to exposures in the environment and they're very specific exposures.

For example, high doses of radiation – that you would use to treat rectal cancer or breast cancer or prostate cancer – can occasionally cause a sarcoma. Certain toxic chemicals, primarily herbicides and pesticides, so people who are professional landscapers, gardeners, grew up on a far most of their life, Vietnam veterans that were sprayed with agent orange – those are all risk factors for getting a sarcoma, so it’s not any chemical – it’s certain types of chemicals. Probably another 10% or so are genetic, so there are certain known mutations – patients that have neurofibromatosis, von Recklinghausen disease when we talk about the elephant man – they're at very high risk for getting certain types of sarcomas. There's a mutation called li-fraumeni where you have a mutation in P53 – and that’s a gene in your body that corrects mistakes that cells make when they're dividing, tries to shut down the cell so it doesn’t become cancer – those patients are at much higher risk for getting soft tissue sarcomas. Unfortunately, for most of them, it’s just something changed and one cell became abnormal. For that reason, they affect any age group. We call it the cancer that does discriminate. There are six month old babies that can get sarcomas and up to 100 years old, so it’s not really an age related cancer. We see lots of patients in their 20s and 30s, but also in their 80s and 90s. It’s really thankfully rare, but a very unusual type of cancer.

Bill: Very rare, and does it usually strike a certain part of the body?

Dr. Kane: Because it comes from the tissues that hold you together, you can get a sarcoma anywhere in your body. Probably half of them occur on what we call the extremities – the arms or the legs – but then about 20% or 30% of them will occur inside the abdomen or even in the back of the abdomen – what we call the retroperitoneum. Think about you're in a furnished living room and you're underneath the wall to wall carpeting. That’s where the kidneys and the major blood vessels. People get them in their head and neck region. We resect them from the ribs and the chest wall. You can even get a primary sarcoma in a vital organ, so it can form in the blood vessels, in the liver, in the lung, so it’s one of those things wherever you have those types of tissues, you're at risk for getting sarcoma. We even get some sarcomas that form in the skin, so it’s not a skin cancer – it’s a sarcoma that just happened to be in the skin.

Bill: What are the symptoms generally?

Dr. Kane: Most of them will present as a painless mask. If you think about malignant cells, they're your own cells and by the cancer getting established, your immune system, your body hasn’t recognized it in a sense. Unless the tumor is invading into a nerve or pushing on a structure, most of the time, the patient will just notice a painless mass. ‘I don’t remember this gold ball in my calf’ or ‘I was in the shower and I realized I had this lump on my back.’ Many people will attribute it to a trauma, but there's isn't really compelling data that an injury or an accident will cause the sarcoma. What it probably does is it draws attention to the tumor, so you bump yourself and you hip is a little bit sore and when you reach there to say your hip is sore, you notice that you found this mass that wasn’t there before. As we use CAT scans and other imaging more and more, if you have a kidney stone, you can go to the emergency room with abdominal pain.

Some of them are actually picking up – what we call incidentally – that there weren’t any symptoms. The patient got the CAT scan for another reason and there's a football sized mass in the back of their abdomen. In some ways, what we like to change overtime – and we’ve seen this throughout the world – from the time the patient has symptoms – I’ve noticed the mass or there's this lump – to the diagnosis, the median time for half the patients is up to six months. I think some of that is because it’s rare and people don’t think about it. They don’t put it on the list of possibilities when the person has the mass – they think it’s a hematoma or a lipoma – a benign fatty tumor – things that are more common. Some of it too is the patient isn't always ‘I have this lump but it doesn’t hurt, I’ll just keep an eye on it for a while,’ and then it’s only overtime – over a few months – when it starts to grow that they become more concerned. We’re hoping to decreased that time in the future due to awareness for patient providers and things like that.

Bill: When you determine that it is sarcoma, how is it treated?

Dr. Kane: It would depend on the stage and people are very familiar when we talk about stage one, two, three, four, and interestingly in sarcoma, that’s not a good system. It’s because there's a lot of subtleties. For example, there's probably upwards of 50 different types of sarcomas and the type alone can sometimes behave a little bit differently. Some are much more likely to spend to distant organs that others. We have low grade and high grade growing slowly and growing quickly. The location on the body can actually help determine whether or not the sarcoma will spread. Sarcomas of the arms and legs tend to be less aggressive in terms of spreading to the distant organs than the same sarcoma inside your abdomen or in your chest. The age of the patient makes a difference. Interestingly, younger patients do better than older patients. It’s not due to their health – it’s just that somehow sarcoma has become somewhat more aggressive as patients get older. The size also is very important. A sarcoma the size of a golf ball, everything else being the same – the type, the grade, the location – will have a lower chance of spreading as compared to a sarcoma the size of a football.

When people talk about customized medicine in sarcomas, we can actually have computer algorithms where you punch in the age, the size of the tumor, the location, the type and you can get an individualized risk for it spreading. The best sarcomas may have a 90%+ chance of being cured, but then we have some sarcomas that will start to push a 50%, 60% or 70% chance of spreading to the distant organs in the first five years. For the primary tumor, the main goal is to try to remove it or surgically resect it with negative margins. Sarcomas have what's called a pseudo capsule – they grow like a mass or a lump – but there's little microscopic roots or tentacles that grow out past that sometimes ¾ of an inch. When you remove it, you would like to get a rim of normal tissue around it to make sure you got those roots to reduce the chance of it coming back. If we think that we can do that with surgery alone, surgery would be the primary treatment. If we feel that those roots might be close to something very important that we don’t want to take – for example, next to your femur bone in your thigh or your sciatic nerve – if we just did surgery and we leave some of that root, higher chance of it coming back and that may actually cost the patient their arm or their leg if it comes back. We will then combine it, surgery with radiation, and if we know what we’re going to do radiation, we do the radiation first. We have something to shoot at, the tumor is still there, it’s actually a smaller dose because radiation needs oxygen to work and the tumor being there has a better blood supply, better oxygenation, and it’s over a smaller area because we haven't done anything to it yet.

For primary sarcoma, high risk of coming back, we’ll give often five weeks or preoperative radiation, repeat all the scans and then do surgery. If we’re not sure they need it, we’ll often do surgery and then we can give post-operative radiation when the final results come back, we decide we’d like to reduce the risk of it coming back and add some radiation, but it tends to be a higher dose. A larger area takes longer – takes six to seven weeks to get it. For the very high risk ones in healthy people, we’ll think about either giving several cycles of chemotherapy before we even treat the tumor – what we call neoadjuvant – to say ‘let’s get chemotherapy into the person’s system as early as possible in case there's little cells hidden in the lung or liver, we can hopefully kill them before they have a chance to establish and grow,’ or if we didn’t do it before surgery for some of the high risk patients, we’ll give it after surgery so we may get four to six months or insurance chemotherapy. Many sarcomas will just be treated with surgery if they're low risk and many of them also will just require surgery and radiation. Not all sarcomas need chemotherapy like we use for some other cancers. For example, breast cancer – most women will get chemotherapy – that’s not the case with sarcoma.

Bill: What treatment types are offered to Roswell Park Cancer Institute and what sets Roswell Park Cancer Institute apart from other cancer centers when it comes to sarcoma treatment?

Dr. Kane: I think a couple of things. The most important thing is it’s very rare, so if you think about breast cancer, colon cancer, prostate cancer, common cancer – many people are familiar with caring for those types of cancers in patients with those cancers. Sarcomas, we see patients from all over the state, from other states in the US, even from out of the country, because they're so rare and each type has a subtle difference to it – some behave differently than others, some response to chemo, some don’t. There's an expertise here. I'm the main soft tissue sarcoma surgeon. We have an orthopedic oncologist who treats the bony sarcomas and some other soft tissue tumors. We have a radiation oncologist who just treats sarcomas. We have a medical oncologist who just gives chemotherapy to sarcomas.

We have a group of people who have significant experience with what would otherwise be a very rare tumor. We actually have a sarcoma conference every week, so all the new patients that I see, we present them in a room, the radiologists put up the image, we actually have pathologists here who have expertise in sarcomas – including doing molecular testing looking for gene mutations that may either define what type of sarcoma it is, or also to help guide treatment based on certain mutations – a specific drug that may work. Every Monday for one and a half to two hours, we actually sit in a room and we go through these patients, we talk about them, we come up with ‘is this patient going to get preoperative radiation,’ in which case we send them to the sarcoma radiation oncologist. The patients are getting the best possible plan. Usually, your first shot at treating a sarcoma is going to be your best shot, so you don’t even want to say ‘I wish we would have actually done this first in this situation.’ We actually talk about it ahead of time.

One of the other things I think that really helps the patients is that we have an approach to make the diagnosis. Years ago, when I first came to Roswell, we would see a lot of sarcomas that had already been removed. Somebody completely cut out the tumor, it had positive margins – that meant that we couldn’t give preoperative radiation or we couldn’t give preoperative chemotherapy – and then see was the chemotherapy actually working because the tumor was still there – a marker for whether it was responding or not. There are also some benign tumors that in the past were treated with surgery, but now we don’t do surgery – we do observation. We may treat them with very simple medications. What we've tried to do is move everything upstream. We’ll see patients with a suspicious mass. They don’t have to have a sarcoma diagnosis or any kind of cancer diagnosis – if they're concerned about the mass, if their primary care doctor is concerned, we’ll see them, we’ll make sure we get the most cost effective imaging. There's a lot of things we don’t need to get – sometimes a CAT scan is what we need, sometimes just an MRI.

We actually have a process where we’ll do the biopsy and make the diagnosis, and that almost always done with what's called a core needle biopsy. People are more familiar with breast tumors, so for years and years, a woman with a breast mass went to a surgeon, he or she surgically removed the mass, if it came back benign, the woman was happy, but she had a scar on her breast for the rest of her life and she had had surgery. If it was breast cancer, it wasn’t the cancer surgery, so she saw to go have a second operation. Now that same woman would get a mammogram or an ultrasound and using a special needle that takes little pencil point sized pieces – they would do biopsies of the tumor in the breast and make the diagnosis with basically an incision the size of a grain of rice. Very low risk of bleeding, very low risk of infection. We can do the same thing with sarcomas at almost any location in the body. Instead of the patient having to have surgery and cutting it open and the risk of bleeding and infection, we have our radiologists under image guidance take five or six little pencil point sized pieces. The pathologist can do everything with those that you could do with the size of a marble or even a bigger piece that we would cut out surgically, including the genetic testing and the mutations. About 90% to 95% of the time with basically a 10-minute outpatient under local anesthesia, we can make the diagnosis – including the type of sarcoma, the grade – and that allows us, without having really done anything to the tumor other than putting a needle into it, to make the most appropriate plan. I think those are some of the unique things that really comes to the fact that it’s such a rare tumor and many of them are being filtered here because they're so uncommon that we have a significant experience with all the different types of sarcomas.

We also even have when they built the new hospital in the late 1990s, we actually had them build a lead lined operating room where we can give interoperative radiation, so there are very few facilities, even in the country, that have that. For some of the sarcomas where we gave them pre-op radiation or we wanted to give them postop radiation but were concerned about one particular area more than others while I have the patient in the OR after the tumor’s been removed, the radiation oncologist can come in and give a 10 or 15-minute high dose boost – I almost call it spot welding – a very high dose radiation to that particular one spot with radiation going to virtually none of the other tissues. Instead of having to push it through the patient’s normal tissues when they're awake, we can do all of this with the normal tissue pushed out of the way – it’s called interoperative brachytherapy. We have the ability to additional target areas where we think there might be some of those microscopic roots and give even more radiation there without hurting the normal tissues. That’s one thing that’s very unique about our operating room here at Roswell Park.

Bill: Very throughout, systematic approach. Dr. Kane, thank you so much for talking with us today about sarcoma. For more information, visit roswellpark.org. That’s roswellpark.org. you're listening to Cancer Talk with Roswell Park Comprehensive Cancer Center. I'm Bill Klaproth. Thanks for listening.