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When to Suspect Sarcoma

Panelists:William D. Tap, MD, Memorial Sloan Kettering Cancer Center; Mark Agulnik, MD, Feinberg School of Medicine;George D. Demetri, MD, Dana-Farber Cancer Center;Martee L. Hensley, MD, Memorial Sloan Kettering Cancer Center; Shreyaskumar Patel, MD, The University of Texas MD Anderson Cancer Center;Damon Reed, MD, Moffitt Cancer Center
Published: Friday, Jul 29, 2016


Transcript:

William D. Tap, MD:
Hello, and thank you for joining us today for this OncLive Peer Exchange Panel Discussion on “Therapeutic Options in Recurrent Soft Tissue Sarcoma.” Surgery remains a standard of care for patients with localized soft tissue sarcoma. Advances in systemic therapy have improved survival, but also added to the complexity of treating this rare disease. Today, our panel of experts will present data regarding several newer therapeutic options and discuss important factors that can help guide treatment decisions.

My name is Dr. William Tap, and I am chief of the Sarcoma Medical Oncology Service at Memorial Sloan Kettering Cancer Center in New York, New York. Participating today on our distinguished panel is Dr. Mark Agulnik who’s an associate professor of medicine in the Division of Hematology/Oncology at the Feinberg School of Medicine, Northwestern University in Chicago, Illinois; Dr. George Demetri, who is a professor of medicine at Harvard Medical School and director of the Center for Sarcoma and Bone Oncology at Dana Farber Cancer Institute in Boston, Massachusetts; Dr. Martee Hensley who’s an attending physician of gynecological medical oncology at Memorial Sloan Kettering and a professor of medicine at Weill Cornell Medical College in New York; Dr. Shreyaskumar Patel, a Robert R. Herring Distinguished Professor and medical director in the Sarcoma Center at The University of Texas MD Anderson Center in Houston, Texas; and Dr. Damon Reed, the director of the Adolescents and Young Adult Program at the Moffitt Cancer Center. Thank you so much for joining.

So, to start, I think it would be important to discuss a little bit about soft tissue sarcoma and what that represents as a disease entity for us who see these diseases on a routine basis. I’d like to start off with Dr. Damon Reed to discuss a little bit about what are some of the challenges in soft tissue sarcomas.

Damon Reed, MD: I think there are a lot of challenges in managing soft tissue sarcomas. One of the challenges begins with a lack of suspicion of soft tissue sarcoma in many cases. Many times, a patient has a mass that is painless and may have been there for a while, and there’s no suspicion that this could be something malignant. Thus, a biopsy happens well outside of a cancer center, and only when the pathologist starts to look at this do they see there could be a bigger problem. Even when soft tissue sarcoma is suspected, the diagnosis is not exactly easy. And cooperative multidisciplinary tumor boards—whereby the radiology, the pathology, the clinical expertise, and the story behind the patient are all brought together—really helps maximize the chance of making the correct diagnosis.

William D. Tap, MD: I agree. I think one of the biggest challenges is that this is a very rare and heterogeneous disease, so it’s often difficult to get knowledge to patients out in the community or even to a community oncologist. Dr. Demetri, do you have any?

George D. Demetri, MD: Yes, I think one of the challenges is all of us work at academic referral centers. We’re used to seeing a lot of sarcomas. We forget sometimes that our brethren in the community see a lot more benign lumps than malignancies. I think Damon is right, that by the time we understand that something is there and it’s a problem, it may be that something has already happened. And so, very often, it’s referred to us after an initial operation. We try very hard to have our referring physicians always consider the diagnosis of sarcoma, even though we know the denominator of benign lumps is much more common.

Martee L. Hensley, MD: We have a bit of a special challenge in gynecologic sarcomas because it’s a little harder to get to the tissue, and there’s not presurgical imaging that definitely tells us whether cancer is present or not. So, the management of growing fibroids in a postmenopausal woman, for example, really ought to be very thoughtful and sometimes might need to be managed already by a gynecological oncologist rather than by a benign gynecologist, even before we have any histologic proof of cancer.

Mark Agulnik, MD: I also think we’re dealing with a younger population, and it’s a population where one doesn’t have primary care physicians. And often, people don’t think about malignancy in that 20-to-39-year range. And therefore, it’s not an area where they necessarily have access to medical care as easily as someone that might be 65 or older that’s seeing their primary care for hypertension and other illnesses.

Shreyaskumar Patel, MD: And one final comment. I think this may be more appropriate for the primary care physicians: that when the patient presents with a history of a growing mass, a diagnosis or possibility of malignancy has to be entertained. Because more often than not, we see these things that have been ignored for a long time as a lipoma, fibroma, hematoma. These things are not supposed to grow. If the patient is clearly telling us that this mass wasn’t there 6 months ago and started as a dime-size thing, and now it’s like a small orange, I think that’s a clear lead into further investigation, radiographically and histologically, to prove or disprove what this exactly is.

William D. Tap, MD: I think that’s a great point. Shreyas, why don’t you tell us a little bit about what this disease is that we call sarcoma? Because as we talk about sarcoma, we don’t look at this as one disease entity. We really look at it as multiple different cancers, each sometimes approached differently. And what are some of the more aggressive subtypes? What are some of the subtypes that you encounter more often, and how does that get you thinking about how to treat the patient?

Shreyaskumar Patel, MD: We’ve thrown the term around that this is a very heterogeneous disease and a rare disease. But just for the numbers to be out there, depending on how much of a stickler for details one is, there can be somewhere between 50 to 75 different types of soft tissue sarcomas. Clearly, they’re not all very common. But the more common ones tend to be liposarcomas, leiomyosarcomas, and what is now called undifferentiated pleomorphic sarcoma, but used to be called malignant fibrous histiocytoma in the past. The more common ones don’t necessarily always have to be the most aggressive ones, or vice versa. It’s clearly not true; there are plenty of aggressive ones that may not be that common. And from an aggressiveness standpoint, we’ll come back to the prognostic factor discussion a little bit later in the course of the session. But the more aggressive ones are going to present from a diagnostic standpoint as the faster growing ones, as the more rapidly changing ones.

William D. Tap, MD: I agree. Any other approaches when you think about these diseases and comments to some of the oncologists watching?

George D. Demetri, MD: Well, I think one thing is that we’ve effectively carved off the gastrointestinal stromal sarcomas or the gastrointestinal stromal tumors, the GISTs. They’re still sarcomas. They’re soft tissues, but they’re in their own bin because we understand them, and we have a very unique way of managing them. And I think it’s good if we just keep them in that bin and focus on all the other subtypes of soft tissue sarcomas. But what we’ve learned from GIST is what we’d like to do with all the other flavors of soft tissue sarcomas—really understand them, treat them uniquely for risk. The whole idea is that there’s a risk profiling that we put our GIST patients through after primary resection. What’s the risk of recurrence? It is just like we do in other solid tumors. So, that fits into an oncologist’s practice pattern very nicely, and I think that’s the kind of thing we’d like to do with all of these diseases. But we’re at specialty centers and we know how hard that is even for us to keep up with the literature. So, I don’t know how a community oncologist, who will see one of these cases every year, will handle that.

William D. Tap, MD: That’s exactly right. Martee?

Martee L. Hensley, MD: Similarly, in OB/GYN, I would carve out the entity called low-grade endometrial stromal sarcoma. Most of what we’ll talk about today are these higher grade sarcomas of differing histologies, but the low-grade endometrial sarcomas are hormonally sensitive tumors that women can live with, really for years. And to over treat them with cytotoxics is usually not the right choice, so that’s where histologic review and understanding that not all sarcomas are high-grade sarcomas comes in. It’s really important to make sure that our treatment fits the disease.

Mark Agulnik, MD: I think you just nailed it with two words: histologic review. When we all talk so freely about these histologies in this heterogeneous group, I do think a histological review is necessary, just to make sure that the diagnosis is appropriate before we start to discuss treatment options for these patients.

Transcript Edited for Clarity
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Transcript:

William D. Tap, MD:
Hello, and thank you for joining us today for this OncLive Peer Exchange Panel Discussion on “Therapeutic Options in Recurrent Soft Tissue Sarcoma.” Surgery remains a standard of care for patients with localized soft tissue sarcoma. Advances in systemic therapy have improved survival, but also added to the complexity of treating this rare disease. Today, our panel of experts will present data regarding several newer therapeutic options and discuss important factors that can help guide treatment decisions.

My name is Dr. William Tap, and I am chief of the Sarcoma Medical Oncology Service at Memorial Sloan Kettering Cancer Center in New York, New York. Participating today on our distinguished panel is Dr. Mark Agulnik who’s an associate professor of medicine in the Division of Hematology/Oncology at the Feinberg School of Medicine, Northwestern University in Chicago, Illinois; Dr. George Demetri, who is a professor of medicine at Harvard Medical School and director of the Center for Sarcoma and Bone Oncology at Dana Farber Cancer Institute in Boston, Massachusetts; Dr. Martee Hensley who’s an attending physician of gynecological medical oncology at Memorial Sloan Kettering and a professor of medicine at Weill Cornell Medical College in New York; Dr. Shreyaskumar Patel, a Robert R. Herring Distinguished Professor and medical director in the Sarcoma Center at The University of Texas MD Anderson Center in Houston, Texas; and Dr. Damon Reed, the director of the Adolescents and Young Adult Program at the Moffitt Cancer Center. Thank you so much for joining.

So, to start, I think it would be important to discuss a little bit about soft tissue sarcoma and what that represents as a disease entity for us who see these diseases on a routine basis. I’d like to start off with Dr. Damon Reed to discuss a little bit about what are some of the challenges in soft tissue sarcomas.

Damon Reed, MD: I think there are a lot of challenges in managing soft tissue sarcomas. One of the challenges begins with a lack of suspicion of soft tissue sarcoma in many cases. Many times, a patient has a mass that is painless and may have been there for a while, and there’s no suspicion that this could be something malignant. Thus, a biopsy happens well outside of a cancer center, and only when the pathologist starts to look at this do they see there could be a bigger problem. Even when soft tissue sarcoma is suspected, the diagnosis is not exactly easy. And cooperative multidisciplinary tumor boards—whereby the radiology, the pathology, the clinical expertise, and the story behind the patient are all brought together—really helps maximize the chance of making the correct diagnosis.

William D. Tap, MD: I agree. I think one of the biggest challenges is that this is a very rare and heterogeneous disease, so it’s often difficult to get knowledge to patients out in the community or even to a community oncologist. Dr. Demetri, do you have any?

George D. Demetri, MD: Yes, I think one of the challenges is all of us work at academic referral centers. We’re used to seeing a lot of sarcomas. We forget sometimes that our brethren in the community see a lot more benign lumps than malignancies. I think Damon is right, that by the time we understand that something is there and it’s a problem, it may be that something has already happened. And so, very often, it’s referred to us after an initial operation. We try very hard to have our referring physicians always consider the diagnosis of sarcoma, even though we know the denominator of benign lumps is much more common.

Martee L. Hensley, MD: We have a bit of a special challenge in gynecologic sarcomas because it’s a little harder to get to the tissue, and there’s not presurgical imaging that definitely tells us whether cancer is present or not. So, the management of growing fibroids in a postmenopausal woman, for example, really ought to be very thoughtful and sometimes might need to be managed already by a gynecological oncologist rather than by a benign gynecologist, even before we have any histologic proof of cancer.

Mark Agulnik, MD: I also think we’re dealing with a younger population, and it’s a population where one doesn’t have primary care physicians. And often, people don’t think about malignancy in that 20-to-39-year range. And therefore, it’s not an area where they necessarily have access to medical care as easily as someone that might be 65 or older that’s seeing their primary care for hypertension and other illnesses.

Shreyaskumar Patel, MD: And one final comment. I think this may be more appropriate for the primary care physicians: that when the patient presents with a history of a growing mass, a diagnosis or possibility of malignancy has to be entertained. Because more often than not, we see these things that have been ignored for a long time as a lipoma, fibroma, hematoma. These things are not supposed to grow. If the patient is clearly telling us that this mass wasn’t there 6 months ago and started as a dime-size thing, and now it’s like a small orange, I think that’s a clear lead into further investigation, radiographically and histologically, to prove or disprove what this exactly is.

William D. Tap, MD: I think that’s a great point. Shreyas, why don’t you tell us a little bit about what this disease is that we call sarcoma? Because as we talk about sarcoma, we don’t look at this as one disease entity. We really look at it as multiple different cancers, each sometimes approached differently. And what are some of the more aggressive subtypes? What are some of the subtypes that you encounter more often, and how does that get you thinking about how to treat the patient?

Shreyaskumar Patel, MD: We’ve thrown the term around that this is a very heterogeneous disease and a rare disease. But just for the numbers to be out there, depending on how much of a stickler for details one is, there can be somewhere between 50 to 75 different types of soft tissue sarcomas. Clearly, they’re not all very common. But the more common ones tend to be liposarcomas, leiomyosarcomas, and what is now called undifferentiated pleomorphic sarcoma, but used to be called malignant fibrous histiocytoma in the past. The more common ones don’t necessarily always have to be the most aggressive ones, or vice versa. It’s clearly not true; there are plenty of aggressive ones that may not be that common. And from an aggressiveness standpoint, we’ll come back to the prognostic factor discussion a little bit later in the course of the session. But the more aggressive ones are going to present from a diagnostic standpoint as the faster growing ones, as the more rapidly changing ones.

William D. Tap, MD: I agree. Any other approaches when you think about these diseases and comments to some of the oncologists watching?

George D. Demetri, MD: Well, I think one thing is that we’ve effectively carved off the gastrointestinal stromal sarcomas or the gastrointestinal stromal tumors, the GISTs. They’re still sarcomas. They’re soft tissues, but they’re in their own bin because we understand them, and we have a very unique way of managing them. And I think it’s good if we just keep them in that bin and focus on all the other subtypes of soft tissue sarcomas. But what we’ve learned from GIST is what we’d like to do with all the other flavors of soft tissue sarcomas—really understand them, treat them uniquely for risk. The whole idea is that there’s a risk profiling that we put our GIST patients through after primary resection. What’s the risk of recurrence? It is just like we do in other solid tumors. So, that fits into an oncologist’s practice pattern very nicely, and I think that’s the kind of thing we’d like to do with all of these diseases. But we’re at specialty centers and we know how hard that is even for us to keep up with the literature. So, I don’t know how a community oncologist, who will see one of these cases every year, will handle that.

William D. Tap, MD: That’s exactly right. Martee?

Martee L. Hensley, MD: Similarly, in OB/GYN, I would carve out the entity called low-grade endometrial stromal sarcoma. Most of what we’ll talk about today are these higher grade sarcomas of differing histologies, but the low-grade endometrial sarcomas are hormonally sensitive tumors that women can live with, really for years. And to over treat them with cytotoxics is usually not the right choice, so that’s where histologic review and understanding that not all sarcomas are high-grade sarcomas comes in. It’s really important to make sure that our treatment fits the disease.

Mark Agulnik, MD: I think you just nailed it with two words: histologic review. When we all talk so freely about these histologies in this heterogeneous group, I do think a histological review is necessary, just to make sure that the diagnosis is appropriate before we start to discuss treatment options for these patients.

Transcript Edited for Clarity
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