Outsmarting Sarcoma: How MSK’s Experts Are Advancing Treatment and Research Progress

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Medical oncologist Bill Tap examines a patient

Bill Tap, Chief of MSK's Sarcoma Medical Oncology Service, gives every person as much attention as possible. "Patients are always on your mind," he says.

9:54 am: It’s an unusual Tuesday in Bill Tap’s sarcoma clinic. He’s been reviewing his patient list, along with notes on each person, for the better part of the morning. The schedule, 17 people, is a light day for him. “This is quiet,” he says. “I’d usually have double this amount.”

Indeed, more than 100 people with a variety of sarcomas are seen at the clinic at Memorial Sloan Kettering’s Rockefeller Outpatient Pavilion on 53rd Street on an average day. Sarcoma, a potentially deadly group of cancers,  accounts for just about 1% of all cancers. As a result, it isn’t widely known, and many doctors have never diagnosed or treated it. Unlike tumors that occur in a particular organ (the prostate or lung, for example), sarcomas can appear almost anywhere in the body. There are more than 50 different types of sarcoma, all wholly unique.

This level of complexity, of both the disease and its treatment, is what brings so many people to Dr. Tap and his colleagues. “MSK offers people with sarcoma a depth of experience that few other hospitals in the world can match,” he says. “This expertise extends across all age groups, from sarcomas that affect babies and young children to those that are primarily found in older adults.”

Beyond adult sarcomas, MSK experts have extensive experience in diagnosing and treating pediatric sarcomas of all types. We care for about 200 children, adolescents, and young adults with this disease each year.
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10:25 am: Medical oncologist Sandra D’Angelo drops in to ask Dr. Tap about a patient who has taken part in a study of pembrolizumab (Keytruda®), an immunotherapy treatment.

Pembrolizumab is a checkpoint inhibitor, a class of drugs that take the brakes off the immune system and allow it to target cancer. Originally used to treat melanoma, these drugs are showing promising results for a number of different cancers, including many types of sarcoma. Doctors and researchers at MSK are leading the way in developing and testing immunotherapy treatments for sarcomas.

Drs. Tap and D’Angelo discuss treatment options for the patient and decide to have her seen by a colleague in radiation oncology. Radiation therapy, given before or after surgery, is part of the standard treatment for many types of sarcoma, and is also commonly used for people who have metastatic disease to complement other therapies. It may boost the effectiveness of checkpoint inhibitors by making tumor cells more visible to the immune system.

Getting the Right Diagnosis

10:35 am: High school student Clare Nimura is shadowing Dr. Tap this morning as part of a program the sarcoma team has created for budding medical professionals. To give Ms. Nimura the full picture of the disease, Dr. Tap suggests she visit the lab of pathologist Cristina Antonescu, who has specialized in diagnosing sarcomas for more than 20 years at MSK.

“Many different types of sarcoma can look similar under a microscope,” says Dr. Tap. “Molecular pathology is important not just for categorizing each tumor but also for learning about the molecular changes that drive it and eventually find ways to stop that growth. It can even change a diagnosis entirely.”

“When samples are sent to us from other hospitals for a second opinion, I would estimate that we change the diagnosis 10 to 15% of the time,” says Dr. Antonescu. “This can have big implications. Because some of these tumors are so rare, many pathologists don’t have the necessary experience to identify the nuances. But you have to know what’s driving a tumor to identify the best treatment.”

She and investigator Marc Ladanyi, Chief of the Molecular Diagnostics Service, have focused their careers on sarcoma and have discovered many of the genetic changes that drive its formation and spread.

Pathologist Cristina Antonescu with fellows Andrew Golden and Chuanyong Yu

Pathologist Cristina Antonescu, with fellows Andrew Golden (left) and Chuanyong Yu, specializes in diagnosing sarcoma — a necessary expertise for a complicated disease. (Photo: Michael Prince)

11:15 am: Jason Chan, one of Dr. Tap’s two medical oncology fellows, pokes his head in the doorway. (Dr. Tap has a general open-door policy.) He’s just met with a patient and wants to give Dr. Tap the rundown. The person is considering leaving her primary oncologist, near her home, and coming to MSK.

The woman has been diagnosed with gastrointestinal stromal tumor (GIST), a rare cancer that develops in the digestive tract. Dr. Tap’s visit with the family lasts nearly 30 minutes. He explains that MSK’s extensive expertise offers the best hope for a positive outcome, but also suggests he can coordinate care with her local oncologist. “What she really needs is to be treated here on a clinical trial,” he says after he leaves her consultation room. But all he can offer today is guidance.

“Whenever I meet a prospective patient for the first time,” Dr. Tap says, “they set the tone. Are they anxious? Upset? Lost? Then my job is to decide what is the best role we can play to help this person and their family. That’s the key.”

Though GIST is uncommon, it can be treated with targeted therapies, including imatinib (Gleevec®). MSK investigators led much of the work that resulted in the US Food and Drug Administration’s approval of imatinib for GIST in 2002. Dr. Tap led the development of another targeted sarcoma drug, olaratumab (Lartruvo), which the FDA approved in 2016.

(Editor’s note: On April 25, 2019, Eli Lilly, the company that makes olaratumab (Lartruvo®), announced that it was withdrawing the drug from the market. This move came after results from a phase III clinical trial found the drug did not improve survival for people with soft tissue sarcoma. The company said it was establishing a program that would allow people who have benefited from the drug to continue to have access to it.)

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Offering a Surgical Solution

12:17 pm: Back in his office, Dr. Tap is greeted by surgeon-scientist Samuel Singer, with whom he collaborates on a daily basis. Dr. Singer arrives with two surgical fellows to talk through a patient’s treatment options.

When samples are sent to us from other hospitals for a second opinion … we change the diagnosis 10 to 15% of the time. This can have big implications.
Cristina R. Antonescu pathologist

Surgery is the primary treatment for many soft tissue sarcomas. For people with early-stage, less-aggressive cancers, surgery may be the only treatment they need. “We do about 650 sarcoma surgeries a year at MSK, and many of our surgeons have dedicated their entire practice to sarcoma,” Dr. Singer says. “They have the know-how to do operations that have the best chance of removing all the cancer while at the same time preserving as much function as possible.”

MSK surgeons have developed a number of specialized approaches for removing sarcomas. These include laparoscopic or robotic surgeries so people can recover faster and limb-sparing procedures for tumors in the arms and legs. MSK’s plastic and reconstructive surgeons help people regain both their appearance and function after the tumor is removed.

For many people, though, surgery alone is not enough. Sarcomas may come back afterward and may spread to other parts of the body, including vital organs like the liver and lungs. Or their location in the body may make surgery too difficult. This is where Dr. Tap and his colleagues in medical oncology and radiation oncology come in, looking for other ways to eliminate these tumors.

12:44 pm: Dr. Tap walks past a platter of pastries on a desk in the hallway on the way back from another patient visit and offers them to anyone around. He chooses one, takes a bite, and makes a face. “I hoped this was blueberry,” he says. “It isn’t.” He eats it anyway.

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Understanding the Biology of Sarcoma

2:14 pm: Dr. Tap takes a moment in between patient visits to check his email. He’s gotten some notes that relate to ongoing sarcoma research in labs at MSK. This kind of research is vital to increasing the understanding of sarcoma.

Most sarcomas begin in tissues that form the mesoderm, the part of a developing embryo that creates the body’s supportive structures. By contrast, the majority of common cancers arise from tissues that begin in the ectoderm (like skin, breast, lung, and colon cancers) or in organs that develop from the endoderm (like liver, pancreatic, and thyroid cancers).

Surgeon Samuel Singer

Surgeon Samuel Singer is the principal investigator of the sarcoma SPORE grant at MSK. SPORE (Specialized Programs of Research Excellence) is a designation from the National Cancer Institute that recognizes interdisciplinary research programs for a variety of different cancer types. MSK is currently the only single institution in the country to receive SPORE funding for sarcoma research. “Research for sarcoma has been historically underfunded. That’s part of what makes this recognition so significant,” Dr. Singer says. Photo: Michael Prince

This makes understanding developmental biology, the study of how embryos develop from a single fertilized egg into all of the tissues in the body, an important focus of sarcoma research. “We tap into areas of basic science as well as clinical research and drug development,” Dr. Tap explains. “By understanding at the molecular level how these cells and tissues develop normally, as well as how they transform into cancer, we can create better treatments.”

One major resource Dr. Tap and others use is a database of more than 10,000 people treated for sarcoma at MSK. It was started more than 20 years ago by pioneering sarcoma surgeon Murray Brennan and includes clinical records and medical histories, images of pathology samples, and, now, details about the genetic and other molecular changes driving tumor growth.

“This database helps us understand the clinical nuances of the disease, so we can then begin to figure out which treatments may benefit which patients,” Dr. Singer explains. “It also helps us to identify areas for new research.”

One such area of focus is on pediatric sarcomas. Although they are very rare, sarcomas are relatively more common in children than they are in adults, making up about 15 percent of all childhood cancers. MSK’s Pediatric Translational Medicine Program is focused on translating discoveries made in the lab into the clinic for sarcomas as well as other pediatric cancers.

Because of the nature of the disease, it's a really small international community of people who treat it. Fostering relationships between other experts is vital to advancing progress and caring for as many people as possible.
William D. Tap medical oncologist
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A Global Team Effort

2:44 pmDr. Tap emerges from a consultation room along with Reema Patel, a clinical observer who is beginning a sarcoma program at the University of Kentucky. Dr. Patel is working with Dr. Tap for a month to learn about how MSK operates so she can take those lessons back to her patients. Throughout the course of the year, Dr. Tap says, doctors from Europe, South America, Asia, and across the United States visit MSK’s sarcoma clinic.

“Because of the nature of the disease, it’s a really small international community of people who treat it,” he adds. “Fostering relationships between other experts is vital to advancing progress and caring for as many people as possible.”

MSK treats more people with sarcoma than most other places in the world, including about 70% of those with the disease who live in the New York City area. But not everyone wants to or can come to MSK for treatment. Thus many efforts are focused on setting the standards for care and sharing the findings made in MSK’s labs and clinics widely.

The team gets emails from people across the United States and around the world asking for advice and gauging whether patients should travel to MSK for treatment. “Your first thought is to say, yeah, of course, come here,” he adds. “We try to fit everyone in. But you have to be wise about the decision and do what you think is really best for the patient.”

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Care with a Personal Touch

3:00 pm: A long-time patient of Dr. Tap’s injects some levity into the day, joking with the team and ribbing Dr. Tap about his baby face. (He’s a youthful 47.) When developing relationships with patients, “there are two things you need to do,” Dr. Tap says. “You have to really pay attention to people and pick out the subtle things, both physically and mentally, that the disease is doing to them — affecting their mood or their outlook. Then it’s the conversations, the nuances. You enter medicine and you learn how to interact with people to the best of your ability, and also how not to interact.”

With a disease like sarcoma, doctors must also learn how to give potentially devastating information. “Giving bad news is not easy,” he adds. “If you have frustration, it can transfer to the patient. At the same time, if you bring in a lot of hope and it’s not warranted, that’s not always the best thing either.”

This particular interaction was a happy one. After this, there are several more people to see, more rounds of support to provide, options to offer, and advice to give. But Dr. Tap’s kind bedside manner never wavers. And this was a light day.

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Comments

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While I opted to have my retroperitoneal sarcoma treated elsewhere, I remain still very impressed by Dr. Singer and his team when I visited with them 2 years ago on 20 December, 2016. The aura of professionalism, the atmosphere of compassion, and the environment of expertise which emanated from Singer and team, could have literally encircled the island of Manhattan and reached up to the ivy covered grounds of Singer's alma mater in Cambridge.

Dr. Tap told my wife Carole she had leiomyosarcoma. This was 8 years ago. She needs a new medication and sent an email to Dr. Tap for his advice. He answered in two days. He is truly amazing. Can't afford to live in New York. We are in Nevada!

Dear Gary, thank you for your sharing your experiences with MSK.

I encourage all sarcoma patients to get to a high volume sarcoma center like MSKCC. Research shows that it can make difference in survival. Dr. Tap and his staff are on the top of my list of places I recommend!

Sharon Anderson
President, Leiomyosarcoma Support & Direct Research Foundation (LMSDR)
www.LMSDR.org

I have lms. I would like an appointment. Is it possible to have a phone consult once I get all my records to you?

Dear Laurie, we’re sorry to hear about your diagnosis. We recommend that you call our Patient Access Service to discuss this. They can be reached at 800-525-2225. Thank you for your comment and best wishes to you.

The National Leiomyosarcoma Foundation is proud to have Dr. W. Tap and Dr. S. D'Angelo support its new International Leiomyosarcoma Research Roundtable that holds promise for potential cross-over to other sarcoma subtypes. Drs. Tap and D'Angelo are part of the Foundation's Medical Advisory Council.

I would like to know what clinical trials exist for Sarcomas. I have a Giant Cell Sarcoma Undifferentiated Soft Parts. I have had Radiation, Surgery, post surgery radiation, and now it has spread to my lungs so I am getting Doxorubicin. By far, the most promising new drug to emerge for sarcoma over the last several decades is Lartruvo (olaratumab), a targeted therapy approved by the FDA in 2016 for the initial treatment of metastatic sarcomas. I was suppose to start olaratumab but two days before a press release came out saying it did not do well in the phase III clinical trial. So I am getting doxorubicin alone, so far. I would like to know if there are any clinical trials that exist that I might want to volunteer for. Please feel free to post and or all of my comment regarding clinical trials and what is going on in sarcoma research.

Dear Larry, we’re sorry to hear about your diagnosis. MSK has a number of clinical trials for sarcoma. If you are interested in arranging a consultation to learn more, you can call 800-525-2225 or go to https://www.mskcc.org/experience/become-patient/appointment for more information.

You can browse the list of trials for sarcoma that are currently open here: https://www.mskcc.org/cancer-care/clinical-trials/search?keys=sarcoma&d…

If you are not in the New York City area or not able to come to MSK for another reason, you may want to go to www.clinicaltrials.gov to look for trials at other centers. Thank you for your comment and best wishes to you.

My daughter in law was diagnosed with GIST and is being operated on in a local hospital by a stomach surgeon and she has had every test and scan and all things look very positive but I do wish she was being treated in a facility more familiar with this rare type of cancer. All organs are clean from the scans and MRI and the mitotic rate is a low "2". If things don't go as well as we hope, is there a specialist at MSK that she should see? Since I am the "in law" I have to be careful not to overstep my boundaries but I certainly would suggest something if I knew what to suggest. Thank you

Dear Evelyn, we’re sorry to hear about your daughter-in-law’s diagnosis. MSK has a number of experts in treating GIST. If she is interested in coming to MSK for treatment or a second opinion, she can make an appointment online or call 800-525-2225. Thank you for your comment and best wishes to you and your family.

My husband has been diagnosed with pancreatic carcinosarcoma. He had three months of Gemcitabine/Abraxane neoadjuvant chemo. The Whipple procedure was done in early April, 2019.

The surgical pathology report indicates that the tumor is a high grade malignant neoplasm comprised of multiple components best classified as carcinosarcoma. The carcinoma component is comprised of pancreatic ductal adenocarcinoma (PDAC), moderately differentiated and undifferentiated carcinoma with osteoclast-like giant cells (OGC). The sarcoma component is composed of areas of osteosarcoma, angiosarcoma and undifferentiated sarcoma.

All margins were clean (negative) and 41 lymph nodes were removed, all negative for cancer. On sectioning the tumor revealed a possible necrosis of less than 5%. This of course means that the neoadjuvant chemotherapy was not effective in killing the cancer cells in the tumor.

We are aware that carcinosarcoma in the pancreas is extremely rare, thus there is not much clinical data documented or published treatment options. We met with the surgeon and a medical oncologist today to discuss possible treatment options. It is recommended that Folfirinox be used for adjuvant chemo, starting in early June, 2019, eight weeks post-op. Recommendation is for chemo once every two weeks for six months. My husband is also scheduled for genetic testing.

We have been told that the sarcoma components are not typical sarcoma and are instead “sarcoma like”. Still, we are wondering if we should get a second opinion, particularly from a large sarcoma center like MSK. Are there any oncologists at MSK that have treated carcinosarcomas? Is it possible to first have a remote consultation?

Any direction or help you can provide is most appreciated.

Thank you in advance for your prompt attention.

Dear Jeanne, we’re very sorry to hear about your husband’s diagnosis. Remote consultations at MSK are available only to international patients. If your husband would like to come to MSK in New York City, you can make an appointment online or call 800-525-2225 for more information. If you are not able to travel to New York, we recommend that you seek out a specialist at another National Cancer Institute-designated cancer center. Thank you for your comment and best wishes to both of you.

Are there any clinical trials on the horizon for Giant Cell Sarcoma Undifferentiated Soft Parts? Anti-sarcoma vaccines? CAR T-Cell Therapy? adjuvant therapy? Targeted Antibodies ? Adoptive immunotherapy ? What other names are given to Giant Cell Sarcoma Undifferentiated Soft Parts? I know different hospitals and different countries have different naming conventions.

Dear Larry, we do not have any trials for this cancer in particular, but you may quality for treatment with targeted drugs or immunotherapy based on the mutations in your tumor. If you’d like to arrange a consultation with an expert at MSK to learn more, you can make an appointment online or call 800-525-2225. Thank you for your comment and best wishes to you.

Good day. My dad has soft tissue sarcoma. The primary site was on the lower extremity. It was already removed and they did some test and said it was gone. During this time we did not know if it had metastasized to the lungs or it was a recurrence. After 6 months, we had a ct scan done and nodules more than 5 of them appeared. I think it was 5-8, and there was a mass also on the lungs. According to one doctor it is inoperable. Is there a chance that it can be operable again in the future? by chemo or radiation? The only part that the sarcoma metastasized is his lungs. It has spread to both lungs and involves mediastinum

Dear Ron, we’re sorry to hear that your father’s cancer has spread. If he would like to come to MSK for a consultation to learn about treatment options, you can make an appointment online or call 800-525-2225. Thank you for your comment and best wishes to both of you.

My son went to Mskcc for s bone marrow transplant for his leukemia. He survived that for a few years of hell then in sept 2013 he was diagnosed with Ewing’s sarcoma. We had it under control for a year. He relapsed in 2014 and sadly and unfortunately we lost him in December 2014. He was 21. I forever grateful to MSKcc for all their personal support and professionalism I miss my son dearly.

Dear Mary, we are so very sorry for the loss of your son, but glad to hear you were happy with his care at MSK. Thank you for sharing your story and best wishes to you.

My son, 21 at the time of Ewings sarcoma diagnosis in Dec. 2018, is being treated at Children's Hosp. In Pittsburgh. We were offered to participate in the clinical trial at MSK. Consulting with our oncologist, we decided the best option for my son was to use the protocol of MSK at home here in Pittsburgh. He has had chemo., surgery to remove his collarbone, radiation to the shoulder area & lungs & continuation of chemo. I am happy to say he has done fairly well throughout this process. We have about another 5 to 6 months of treatment left. I'm thankful for the care he is getting.

Dear Christine, we’re sorry to hear about your son’s diagnosis but glad to hear that he is doing well with his treatments. Thank you for sharing your story and best wishes to your son and your whole family.

I have been successfully treated by doctors at MSKCC for GIST since 2007, and most recently for prostate cancer. Drs. Tap, DeMatteo, De Fazion, Chen, Ehdaie, Kollmeier, Kang as well as the nurses, radiology technicians, MRI staff at both NYC and Commack. They have all been focused and provided top notch care. My life has been in their hands and so far -- I'm still here; and with an unsurpassed quality of life. My family and I are beyond grateful as they continue to care for me.

I have treated at MSKCC since 2007 and have received first class care by the medical providers as well as all staff. They are number ONE in my book.

My husband was diagnosed with Extraskeletal Myxoid Chodrosarcoma of the inguinal area, and we would love to contact Dr Tap and Dr Singer

We’re very sorry to hear about your husband’s diagnosis. If he would like to schedule a consultation with one of our sarcoma experts he can make an appointment online or call 800-525-2225. Thank you for your comment and best wishes to both of you.