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Disease State: Advanced Soft Tissue Sarcoma

Insights From: Mark Agulnik, MD, Northwestern University Feinberg School of Medicine;Brian Van Tine, MD, PhD, Washington University
Published: Tuesday, Nov 08, 2016


Transcript:

Mark Agulnik, MD:
Soft tissue sarcomas are considered to be rare tumors. The interesting thing is when you go see a sarcoma specialist—this is what they do every day—and so the rarity of it is not really rare for them. That’s the benefit of having certain academic physicians treat a lot of tumors. But, generally, for a population as a whole, it is a very rare tumor, with less than 20,000 cases coming in per year. And really, when you look at the cases, what are they? Are all soft tissue sarcomas the same? Well, obviously not. There are a great number of histologies. They’re really a heterogeneous group of tumors, and that’s what makes it challenging to treat. It makes it interesting to treat, and it also makes it very challenging when you’re trying to derive what is the best treatment options for patients, knowing that each histology is going to behave a little differently.

If we look at the literature and look to see what is the time period from the time that a tumor develops in a person’s body—and that they’re aware of a change to the time of diagnosis to the time of treatment—unfortunately for soft tissue sarcomas, it seems that there is a challenge to getting a patient diagnosed accurately, properly, and treated in a timely manner. The question always becomes, why does that happen? From my own personal experience in my own practice, I see a lot of patients who present with masses that are either deemed, or thought to be, benign conditions, whether it’s a lipoma, whether or not it’s a hematoma, whether or not they think they have a pulled muscle.

Often, it’s a young patient, and the first thing we don’t think about in young patients is malignancy. And so, this becomes very difficult. I see a number of patients who get referred to physical therapy, whether it’s acupuncture, whether or not they’re sent on antibiotics, whether or not they’re just told to watch it. I do think it becomes difficult because it’s an unexpected diagnosis. Once you do have a diagnosis though, it is very important to pursue the right place. You want to make sure that pathology is correct, you want to make sure the diagnosis is correct, and you want to make sure the workup and treatment options are correct.

Brian Van Tine, MD PhD: I’m often asked what the unmet needs are in soft tissue sarcoma, and I think there’s actually quite a lot. First, I think you have to realize we’re not talking about one disease. The parallel word to sarcoma is actually carcinoma. And so, when you really get into this, you realize we study somewhere between 50 and 100 separate diseases that all have independent biology. It’s very rare to find a therapy that comes through that is sort of a one-size-fits-all. It’s like asking for a drug that works in every carcinoma, and that isn’t true. There are a lot of commonalities amongst the soft tissue tumors where certain drugs seem to be having an effect, such as our baseline drug, doxorubicin, which works on a good majority of the soft tissue sarcomas. But I think what’s really missing, what the unmet needs are, comes down to a better understanding of what these diseases are, how they’re different, what their underlying biology is, and how best to build specific therapies for them.

Transcript Edited for Clarity
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Transcript:

Mark Agulnik, MD:
Soft tissue sarcomas are considered to be rare tumors. The interesting thing is when you go see a sarcoma specialist—this is what they do every day—and so the rarity of it is not really rare for them. That’s the benefit of having certain academic physicians treat a lot of tumors. But, generally, for a population as a whole, it is a very rare tumor, with less than 20,000 cases coming in per year. And really, when you look at the cases, what are they? Are all soft tissue sarcomas the same? Well, obviously not. There are a great number of histologies. They’re really a heterogeneous group of tumors, and that’s what makes it challenging to treat. It makes it interesting to treat, and it also makes it very challenging when you’re trying to derive what is the best treatment options for patients, knowing that each histology is going to behave a little differently.

If we look at the literature and look to see what is the time period from the time that a tumor develops in a person’s body—and that they’re aware of a change to the time of diagnosis to the time of treatment—unfortunately for soft tissue sarcomas, it seems that there is a challenge to getting a patient diagnosed accurately, properly, and treated in a timely manner. The question always becomes, why does that happen? From my own personal experience in my own practice, I see a lot of patients who present with masses that are either deemed, or thought to be, benign conditions, whether it’s a lipoma, whether or not it’s a hematoma, whether or not they think they have a pulled muscle.

Often, it’s a young patient, and the first thing we don’t think about in young patients is malignancy. And so, this becomes very difficult. I see a number of patients who get referred to physical therapy, whether it’s acupuncture, whether or not they’re sent on antibiotics, whether or not they’re just told to watch it. I do think it becomes difficult because it’s an unexpected diagnosis. Once you do have a diagnosis though, it is very important to pursue the right place. You want to make sure that pathology is correct, you want to make sure the diagnosis is correct, and you want to make sure the workup and treatment options are correct.

Brian Van Tine, MD PhD: I’m often asked what the unmet needs are in soft tissue sarcoma, and I think there’s actually quite a lot. First, I think you have to realize we’re not talking about one disease. The parallel word to sarcoma is actually carcinoma. And so, when you really get into this, you realize we study somewhere between 50 and 100 separate diseases that all have independent biology. It’s very rare to find a therapy that comes through that is sort of a one-size-fits-all. It’s like asking for a drug that works in every carcinoma, and that isn’t true. There are a lot of commonalities amongst the soft tissue tumors where certain drugs seem to be having an effect, such as our baseline drug, doxorubicin, which works on a good majority of the soft tissue sarcomas. But I think what’s really missing, what the unmet needs are, comes down to a better understanding of what these diseases are, how they’re different, what their underlying biology is, and how best to build specific therapies for them.

Transcript Edited for Clarity
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