A rare but serious form of cancer, sarcomas often have no symptoms or non-specific symptoms and can affect almost any part of the body.
Learn more about symptoms and the treatment options available from a UVA Cancer Center specialist in sarcomas.
Selected Podcast
Sarcomas: A Rare, Serious Cancer
Featured Speaker:
Dr. Michael G. Douvas
Dr. Michael G. Douvas is a board-certified pediatric hematologist/oncologist and adult oncologist who specializes in caring for patients with leukemia, lymphoma and sarcoma. Transcription:
Melanie Cole (Host): A rare but serious form of cancer, sarcomas often have no symptoms or non-specific symptoms and can affect almost any part of the body. My guest is Dr. Michael Douvas. He’s a board certified pediatric chemotologist-oncologist and adult hematologist who specializes in caring for patients with leukemia, lymphoma, and sarcoma. Welcome to the show, Dr. Douvas. So tell us, what is a sarcoma?
Dr. Michael Douvas (Guest): Well, in short, sarcoma is a cancer of connective tissue. We’re all used to thinking of cancers of organs, like breast, liver, lungs. Sarcomas are cancers of the material that keeps us together—muscles, bones, cartilage, fat.
Melanie: Okay. People don’t tend to think of those, as you say. Are there any risk factors? Because we hear about risk factors for breast cancer or colon cancer, but what about for sarcoma? Are there certain risk factors that we can control and some that we maybe can’t?
Dr. Douvas: Sarcomas affect roughly 12 to 13 thousand people in the United States annually. That’s the number that’s diagnosed. The vast majority of those patients do not have any identifiable risk factors for the development of sarcomas. The known ones are radiation, which can be either radiation that’s used a therapy—so patients who have had prior radiation for breast cancer are of very small risk for developing radiation in the radiation field. And similarly, patients who were treated with radiation for other cancers have small, defined risks for the development of sarcoma. Otherwise, there are a group of rare genetic disorders, such neurofibromatosis, Gardner’s syndrome, Li–Fraumeni syndrome, tuberous sclerosis, where there’s a risk for sarcoma. These are all pretty rare syndromes, and not everybody who has them develops the sarcoma, but they are associated.
Melanie: So you mentioned some hereditary, some rare ones, and you mentioned radiation. I’ve heard that maybe chemical exposure, and also people have heard about Kaposi’s sarcoma. That’s immune-deficient and such. How rare are these?
Dr. Douvas: Chemical exposure is one that’s pretty rare in terms of association with sarcoma. It’s often difficult to establish associations between chemical exposures to cancers. Kaposi’s sarcoma is, again, sort of rare sarcoma within a -- again, sarcomas are a relatively rare cancer. They are most often seen within patients with immune deficiency, most often acquired through HIV infection. Kaposi’s sarcoma was much more common in the 1980s and 1990s before the advent of highly effective therapies for HIV, and so, fortunately, it since has decreased significantly in the last 20 years with better treatments for HIV and AIDS.
Melanie: Dr. Douvas, are there any symptoms that people might come across that would send them to see you in the first place?
Dr. Douvas: The hard thing, as you mentioned in the introduction, is that sarcomas can occur really anywhere in the body—on the limbs, on the torso, in the head and neck. And because they’re cancers of the things that put us together, they can occur anywhere. As a consequence, they can cause different symptoms. The most common, however, are the development of a lump of some type and/or unusual pain that doesn’t go away in a particular area of the body.
Melanie: What would someone do when they found a lump or they found particular pain? They come to see you. You do some tests. What do you do?
Dr. Douvas: Most often, people will have been diagnosed before they see me. They’ll often go to a primary care physician or the emergency room, and in the workup of a lump or a pain, most often, a scan of some type discovers a tumor. And then they are often referred to a general surgeon or an orthopedist or a neurosurgeon for a biopsy. And then it’s after that point when a diagnosis is made that they often end up seeing me. It sometimes occurs that I’m involved in the evaluation of a tumor that’s suspected to be a sarcoma, but I’d say it’s more often that a diagnosis is established before people get to see me.
Melanie: Dr. Douvas, what treatment options are available once it is diagnosed as a sarcoma?
Dr. Douvas: In general, for cancer, there are three groups of treatment often, the first being surgical resection, the second being radiation, and the third being chemotherapy, which is just a large word that means medicine to treat cancer. Sometimes the chemotherapies fall within the bounds of things that people traditionally associate with chemotherapy—that is, medicines that go in through an IV and sometimes cause issues: nausea, vomiting, hair loss, the traditional chemotherapy that people think about. More often, recently, there are pills that are being used for cancer in general and sometimes for sarcomas that we call targeted therapy, where individual cancer has an identified genetic problem and a drug has been designed to specifically address that. A good example is -- there’s a tumor called a gastrointestinal stromal tumor, or a GIST, which is a sarcoma that most often arises in the stomach. It is a distinct entity from stomach cancer, and it very often has a particular molecular abnormality that a medicine called Imatinib or Glivec, that was initially developed to treat a chronic leukemia specifically addresses. These tumors used to be treated with traditional chemotherapy, and the effects were, I would say, less than optimal in terms of their ability to shrink tumors and control their growth. Imatinib, in a targeted way, attacks the tumor in a very different way by playing into its specific genetic abnormality and can be an extremely effective treatment for patients with tumors that are unresectable, or after their resection in order to prevent their recurrence. And so, again, in general, all of these modalities—surgery, radiation, traditional chemotherapy, and targeted therapies—are used to treat sarcomas.
Melanie: And Dr. Douvas, what advice do you have for listeners about coping, support, things that they should do while they’re going through any one of these treatments, whether they’ve had surgery, radiation, chemotherapy, as you’ve discussed? When they’re going through all of these things, what do you tell them to help them?
Dr. Douvas: Well, that’s a difficult question. There are a variety of support services that are available usually through the local treatment center. There are a lot of great support services that are through national foundations that are often specific to the type of disease that a patient has. And then, there are often a lot of services that can be found on the Internet on the web. I have a lot of patients who have found great support groups where they linked with other patients who have similar types of cancers and are going through similar types of treatments and have found these support groups extremely helpful in terms of understanding what others are experiencing and helping them to cope with their situation.
Melanie: And why should patients come to UVA Cancer Center for their sarcoma care?
Dr. Douvas: It’s a good question. I would say there are several reasons. Sarcoma is a pretty uncommon disease. I mentioned before that about 12 to 13 thousand people will be diagnosed with sarcoma in the United States in a year. To compare that, there are about 200 to 250 thousand cases of breast and lung cancer diagnosed annually in the United States. What this means is that hematologist-oncologists, blood and cancer doctors, are significantly less familiar with the treatment of sarcomas than they are with more common cancers. In addition, there are between 50 and a hundred different types of sarcomas, so they are a rare disease, but they’re also a very heterogeneous group. So it’s difficult even for people at major cancer centers to develop a lot of familiarity with all the individual types because they’re just, fortunately, aren’t that many of them out there. But the benefit of coming to a place like UVA is that although it’s a rare disease, we still see a significant proportion of patients diagnosed within the state and therefore are familiar with the treatments that are given, whether they’re specialized surgical techniques for resection and, potentially, reconstruction of limbs. We have dedicated orthopedic-oncologists for patients who have bone-based tumors or bone-based sarcomas. We have radiation oncologists who specialize in the latest techniques of really targeting radiation to specific areas. And, as medical oncologists, we have more familiarity with the regimens that are given for these rare tumors and how they work, what side effects to expect, how to prepare patients to go through it, and to know what is out there as far as new development, such as new targeted therapies for relatively rare tumors.
Melanie: Thank you so much, Dr. Michael Douvas. You’re listening to UVA Health Systems Radio. For more information, you can go to uvahealth.com. This is Melanie Cole. Thank you so much for listening, and have a great day.
Melanie Cole (Host): A rare but serious form of cancer, sarcomas often have no symptoms or non-specific symptoms and can affect almost any part of the body. My guest is Dr. Michael Douvas. He’s a board certified pediatric chemotologist-oncologist and adult hematologist who specializes in caring for patients with leukemia, lymphoma, and sarcoma. Welcome to the show, Dr. Douvas. So tell us, what is a sarcoma?
Dr. Michael Douvas (Guest): Well, in short, sarcoma is a cancer of connective tissue. We’re all used to thinking of cancers of organs, like breast, liver, lungs. Sarcomas are cancers of the material that keeps us together—muscles, bones, cartilage, fat.
Melanie: Okay. People don’t tend to think of those, as you say. Are there any risk factors? Because we hear about risk factors for breast cancer or colon cancer, but what about for sarcoma? Are there certain risk factors that we can control and some that we maybe can’t?
Dr. Douvas: Sarcomas affect roughly 12 to 13 thousand people in the United States annually. That’s the number that’s diagnosed. The vast majority of those patients do not have any identifiable risk factors for the development of sarcomas. The known ones are radiation, which can be either radiation that’s used a therapy—so patients who have had prior radiation for breast cancer are of very small risk for developing radiation in the radiation field. And similarly, patients who were treated with radiation for other cancers have small, defined risks for the development of sarcoma. Otherwise, there are a group of rare genetic disorders, such neurofibromatosis, Gardner’s syndrome, Li–Fraumeni syndrome, tuberous sclerosis, where there’s a risk for sarcoma. These are all pretty rare syndromes, and not everybody who has them develops the sarcoma, but they are associated.
Melanie: So you mentioned some hereditary, some rare ones, and you mentioned radiation. I’ve heard that maybe chemical exposure, and also people have heard about Kaposi’s sarcoma. That’s immune-deficient and such. How rare are these?
Dr. Douvas: Chemical exposure is one that’s pretty rare in terms of association with sarcoma. It’s often difficult to establish associations between chemical exposures to cancers. Kaposi’s sarcoma is, again, sort of rare sarcoma within a -- again, sarcomas are a relatively rare cancer. They are most often seen within patients with immune deficiency, most often acquired through HIV infection. Kaposi’s sarcoma was much more common in the 1980s and 1990s before the advent of highly effective therapies for HIV, and so, fortunately, it since has decreased significantly in the last 20 years with better treatments for HIV and AIDS.
Melanie: Dr. Douvas, are there any symptoms that people might come across that would send them to see you in the first place?
Dr. Douvas: The hard thing, as you mentioned in the introduction, is that sarcomas can occur really anywhere in the body—on the limbs, on the torso, in the head and neck. And because they’re cancers of the things that put us together, they can occur anywhere. As a consequence, they can cause different symptoms. The most common, however, are the development of a lump of some type and/or unusual pain that doesn’t go away in a particular area of the body.
Melanie: What would someone do when they found a lump or they found particular pain? They come to see you. You do some tests. What do you do?
Dr. Douvas: Most often, people will have been diagnosed before they see me. They’ll often go to a primary care physician or the emergency room, and in the workup of a lump or a pain, most often, a scan of some type discovers a tumor. And then they are often referred to a general surgeon or an orthopedist or a neurosurgeon for a biopsy. And then it’s after that point when a diagnosis is made that they often end up seeing me. It sometimes occurs that I’m involved in the evaluation of a tumor that’s suspected to be a sarcoma, but I’d say it’s more often that a diagnosis is established before people get to see me.
Melanie: Dr. Douvas, what treatment options are available once it is diagnosed as a sarcoma?
Dr. Douvas: In general, for cancer, there are three groups of treatment often, the first being surgical resection, the second being radiation, and the third being chemotherapy, which is just a large word that means medicine to treat cancer. Sometimes the chemotherapies fall within the bounds of things that people traditionally associate with chemotherapy—that is, medicines that go in through an IV and sometimes cause issues: nausea, vomiting, hair loss, the traditional chemotherapy that people think about. More often, recently, there are pills that are being used for cancer in general and sometimes for sarcomas that we call targeted therapy, where individual cancer has an identified genetic problem and a drug has been designed to specifically address that. A good example is -- there’s a tumor called a gastrointestinal stromal tumor, or a GIST, which is a sarcoma that most often arises in the stomach. It is a distinct entity from stomach cancer, and it very often has a particular molecular abnormality that a medicine called Imatinib or Glivec, that was initially developed to treat a chronic leukemia specifically addresses. These tumors used to be treated with traditional chemotherapy, and the effects were, I would say, less than optimal in terms of their ability to shrink tumors and control their growth. Imatinib, in a targeted way, attacks the tumor in a very different way by playing into its specific genetic abnormality and can be an extremely effective treatment for patients with tumors that are unresectable, or after their resection in order to prevent their recurrence. And so, again, in general, all of these modalities—surgery, radiation, traditional chemotherapy, and targeted therapies—are used to treat sarcomas.
Melanie: And Dr. Douvas, what advice do you have for listeners about coping, support, things that they should do while they’re going through any one of these treatments, whether they’ve had surgery, radiation, chemotherapy, as you’ve discussed? When they’re going through all of these things, what do you tell them to help them?
Dr. Douvas: Well, that’s a difficult question. There are a variety of support services that are available usually through the local treatment center. There are a lot of great support services that are through national foundations that are often specific to the type of disease that a patient has. And then, there are often a lot of services that can be found on the Internet on the web. I have a lot of patients who have found great support groups where they linked with other patients who have similar types of cancers and are going through similar types of treatments and have found these support groups extremely helpful in terms of understanding what others are experiencing and helping them to cope with their situation.
Melanie: And why should patients come to UVA Cancer Center for their sarcoma care?
Dr. Douvas: It’s a good question. I would say there are several reasons. Sarcoma is a pretty uncommon disease. I mentioned before that about 12 to 13 thousand people will be diagnosed with sarcoma in the United States in a year. To compare that, there are about 200 to 250 thousand cases of breast and lung cancer diagnosed annually in the United States. What this means is that hematologist-oncologists, blood and cancer doctors, are significantly less familiar with the treatment of sarcomas than they are with more common cancers. In addition, there are between 50 and a hundred different types of sarcomas, so they are a rare disease, but they’re also a very heterogeneous group. So it’s difficult even for people at major cancer centers to develop a lot of familiarity with all the individual types because they’re just, fortunately, aren’t that many of them out there. But the benefit of coming to a place like UVA is that although it’s a rare disease, we still see a significant proportion of patients diagnosed within the state and therefore are familiar with the treatments that are given, whether they’re specialized surgical techniques for resection and, potentially, reconstruction of limbs. We have dedicated orthopedic-oncologists for patients who have bone-based tumors or bone-based sarcomas. We have radiation oncologists who specialize in the latest techniques of really targeting radiation to specific areas. And, as medical oncologists, we have more familiarity with the regimens that are given for these rare tumors and how they work, what side effects to expect, how to prepare patients to go through it, and to know what is out there as far as new development, such as new targeted therapies for relatively rare tumors.
Melanie: Thank you so much, Dr. Michael Douvas. You’re listening to UVA Health Systems Radio. For more information, you can go to uvahealth.com. This is Melanie Cole. Thank you so much for listening, and have a great day.