Transcript:William D. Tap, MD: Damon, one of the things we have to stress is that these are not just adult diseases. These are pediatric diseases, as well, and we’re going to spend a lot of time talking about the adult aspects—but pediatric sarcomas tend to have very well-defined treatment paradigms. How do you approach a patient when they come in, and how is it a little different from what we spoke about?

Damon Reed, MD: I do think that the strengths of the pediatric oncology sarcoma community and the strengths of the adult sarcoma oncology community work well together. And when we have an unusual patient—like, say, a 61-year-old patient who has a neck mass and is ultimately found to have alveolar rhabdomyosarcoma, a disease that peaks in the teenage years—in those kind of cases, one could be dogmatic and say, “This is how we would treat a teenager.” However, as one who primarily treats teenagers, they don’t have a really good understanding of how to treat a 61-year-old and how toxicities and tolerabilities scale to that age range.

And so when something like that happens, when there’s a pediatric cancer happening in an advanced age adult and not a young adult, I certainly would have multiple members of our team involved. But this is when the diagnosis really does matter a ton, and this started with a small round undifferentiated cancer. And we’ve talked about how heterogeneous the diagnoses are. But then, once you’ve done all that work, once you’ve found that small bit of fat that made it a dedifferentiated liposarcoma and not an undifferentiated sarcoma, and once you’ve cinched the diagnosis, why do some that are 2 cm have metastases, grow quickly, and progress rapidly, and others are 47 cm and recur 12 years later after a surgery that was marginal? The heterogeneity goes beyond the naming of the diagnosis.

But within pediatric kinds of cancers, yes, I do think that the collaborative spirit of pediatric oncology, the idea that all centers will treat the same way through the Children’s Oncology Group, did lead to significant findings, or, at least, cohesive and more formulaic treatment of pediatric rhabdomyosarcomas than perhaps the way some soft tissue sarcomas are managed in adults. We have learned things through that method, but I see the same collaborative spirit among sarcoma adult oncologists, and as we continue to break them down into their subtypes, I think the same things will be found.

William D. Tap, MD: And I think that’s an important point that we’ll bring throughout: that the pediatric sarcomas, even when we see them in young adults or adults, they have very well-defined treatment strategies. And it’s very important to make sure that you understand that and tap into those. One of the things that we need to get across is, what do patients with rare diseases face? What are some of the challenges that they face that, say, patients who have breast cancer or colorectal cancer don’t face?

Mark Agulnik, MD: It’s very complex, and I think you can break our patients down into probably two core groups. We focus a lot, at least at my center, on the patients under 39, and the reason is that I think we really need to push toward an adolescent and young adult group. They’re walking into a waiting room with a lot of 65+ patients. They’re not seeing anybody that looks like them. They’re being treated in rooms with patients that don’t look like them. If they go at age 26 to the children’s hospital, they’re sitting there in waiting rooms with blocks, games, and toys and everything. So, I do think it’s a very complex group that does need the social workers, the therapist, and psychosocial oncology involved to really get them through. They’re also at a point of life where they could be finishing up college, starting their first job. They’re dealing with issues of fertility that a lot of the 65-year-old-and-over patients don’t deal with; they’re dealing with issues of what do they do with respect to their families, getting married, things like that; young children that probably an older population doesn’t have to deal with.

And then for the older population, I think there really is a difficult understanding. If you have a tumor in the lung, you think it’s lung cancer. If you have a tumor in the liver, you think it’s liver cancer. And there really is this difficult concept to grasp: what is a sarcoma? No one else they know ever has this, and to show up and have something very rare is very scary. Even if the prognosis is very good, it still is this nonentity. They’re referred often in to an academic center because they’re being told by someone they don’t know what this is— “I’ve never treated this; I don’t know that.”

When we round on patients, everyone said, “Oh, this is such an exciting case.” I would never want to be an exciting case, because it’s really only exciting for the physicians and never exciting for the patient. There are really a lot of things they have to deal with. And then they show up in a cancer center. I don’t know that we have a ribbon. I don’t know that we’re giving out cookies. I don’t know that we have walks. I don’t think we have these things, and they’re left in this area where they don’t fit in to very much.

William D. Tap, MD: I agree, and there’s no information out there, so patients come in often with 6 months of missed diagnoses and confusion. And it can be a very difficult journey before you can start on treatment.

George D. Demetri, MD: Or worse, they’ve been Googling it, and then they get scared by what they read because it’s not in context. And they’ve been in a place where the doctor may say, “You’re the only one of these I’ve seen, and I’m going to call a center, or I’m not going to call.” Then it’s up to the patient to help navigate their own service, and it’s up to us, then, to reach out to the doctors in our catchment areas and to our community brethren to say, “Let’s work together. We can help you define a plan of action together for this patient.”

Martee L. Hensley, MD: The uterine sarcoma world has actually gotten together digitally. There are online support groups and chat rooms of women with leiomyosarcoma, particularly, but other kinds of sarcomas. And though that kind of information can be dangerous or incorrect, at least they are connected. And there does seem to be a common thread where they’d say to each other, “You need to be in a specialized center. You need to be cared for by someone that knows what they’re doing.” And that means the referral to academic centers, like we’re in, is increased, and that gives us the opportunity to get the correct information out there.

But it’s not uncommon that people still arrive in my office, I will sit down to talk to them, and the first thing they’ll say back to me is, “I already know chemotherapy doesn’t work in this disease.” And that’s very common that people think that of uterine sarcomas, but not really all sarcomas. Yet, later on, I’m going to be able to say, “But we have so many choices now because there are drugs that work in these cancers, sometimes curably so.” And so, it’s important that people get to where they can hear the right thing.

William D. Tap, MD: I agree, because once you start on the path, you can empower patients, you can bring in hope, and you can really then allow them to take control again.

Damon Reed, MD: I think it’s important to have peers, just like you just said, who have had similar cancers, especially in the young-adult range; patients who are in that age where they do rely so much on peer support. I’ve had patients who say, “Not only do I want somebody with my diagnosis, but also in the left arm, proximal, and preferably between 8 cm to 10 cm.” They’re really detailed. But you can find them, and there are organizations that exist around the country to do things like that. And there are certain things that even the best social worker just can’t do because they haven’t been there. None of us have tried out these medicines that we give our patients, recreationally. So, it takes someone who’s been there.

Transcript Edited for Clarity