Life & limb: A survival story

“I still remember the look on my mother’s face when I told her I had cancer,” says Tony. “Telling my family was the hardest thing I’ve ever had to do.”

Tony with Dr. Jay Wunder

In December 2005, Tony saw his doctor for pain in his left shoulder. He thought he had pulled a muscle. “I honestly thought I’d just done something at the gym,” he recalls.

To be thorough, his doctor ordered an X-ray, which revealed a growth in his left shoulder about the size of a small orange. Further testing showed that it was an osteosarcoma, a cancerous growth on his shoulder and upper arm bone.

“It was clear that it had been there, growing, for some time,” Tony says. “It didn’t make sense. I had just had a birthday a few days before. I was looking forward to the holidays. I was living a healthy, normal life.”

Knowing only that this was the kind of cancer that had led to Terry Fox’s amputation and, ultimately, death, Tony immediately searched for more information. He found little insight, but a terrifyingly low survival rate.

‘Rare as you seem to be’: Sarcoma

Sarcomas — tumours that occur in connective tissues, including fat, muscle, blood vessels, deep skin tissues, nerves, bone and cartilage — are not well known cancers, largely because they are very rare. Though they account for only about one per cent of all cancers in Canada, there are about 80 types of sarcomas and they are particularly dangerous because they can spread easily, often to the lungs.

“Sarcomas are really a family of diseases, each with a unique genetic makeup,” explains Dr. Jay Wunder, surgeon-in-chief at Mount Sinai Hospital and head of the Sarcoma Unit, as well as the Leadership Sinai Chair in Surgery, Rubinoff/Gross Chair in Orthopaedic Oncology and an associate member of the Lunenfeld-Tanenbaum Research Institute (LTRI).

Their rarity and diversity makes sarcomas especially challenging to treat.

As one of the few treatment centres in Canada, Mount Sinai Hospital’s Sarcoma Unit brings together a large, diverse group of patients. It is the largest sarcoma unit in Canada, and among the largest in the world, with a team made up of world-class experts. Most Canadian
orthopaedic surgeons specializing in the management of patients with sarcomas are trained by Dr. Wunder and his team, which has led to a dramatic improvement nationally in the standard of practice. The team also helps train other doctors to accurately diagnose sarcomas — a key factor in catching and treating these tumours early and effectively.

World-class: The Sinai Sarcoma Unit

Tony recognized quickly that he was fortunate to be treated by Dr. Wunder, an accomplished
surgeon who helped develop a new technique that allowed doctors to remove cancerous tissues without amputating an entire limb.

“Dr. Wunder put me at ease. He took the time to answer all my questions, even if it meant calling me late in the evening,” Tony remembers.

Such personalized care is a hallmark of Sinai’s Sarcoma Unit. Dr. Wunder and his team oversee
the highest patient volume and the most complex sarcoma cases in the country, and receive the largest number of sarcoma referrals from across Canada. The unit also hosts Canada’s
highest achieving surgical sarcoma team, curing up to a remarkable 75 per cent of patients.

These results represent years of work in refining our understanding and treatment of sarcomas.

“Twenty-five years ago, almost 100 per cent of osteosarcoma surgeries were amputations. Now that’s under five per cent,” says Dr. Wunder. “With advances in knowledge, improved radiological imaging, chemotherapy and new surgical techniques, we’re able to achieve much better patient outcomes today.”

The team’s multidisciplinary approach is central to its success. The entire Sarcoma Unit works
closely together, finding the best mix of surgery, radiation and chemotherapy to effectively treat
their patients, supported by specialists in every field from psychiatry to physiotherapy. 

“Everyone in the group is a field leader in their own right,” says Dr. Wunder. “We build on that by
working together. We really have an incredible team. It means that our patients get cutting-edge
care, founded on cutting-edge research. It’s an approach that most centres are trying to emulate
now — care that transcends barriers.”

Patients benefit from this collaborative approach, which offers a seamless care experience. And
that experience extends beyond the unit, taking advantage of Sinai’s other centres of excellence.

“We’re able to offer our patients access to a wide range of services because of the other specialties at the hospital,” says Dr. Rebecca Gladdy, a gastrointestinal surgeon at Mount Sinai and associate member of the LTRI who specializes in sarcomas. “When I have younger patients
who need to undergo radiation, for example, I can easily refer them to our oncofertility program, where we can preserve reproductive materials that will hopefully allow those patients to one day start families. All the different facets of care here help us fully support our patients.”

Saving lives and limbs: Sarcoma treatment

For some sarcomas, like Tony’s, surgery alone is enough.

“In a way Tony was lucky, because he had a localized osteosarcoma that we were able to completely remove,” recalls Dr. Wunder. “Because of the type of tumour, he didn’t need
chemotherapy. We were able to reconstruct his shoulder pretty well, so it’s stable and functional.”

But with so many different types of sarcomas, treatment plans vary widely and are individualized to ensure the best outcomes for patients. For example, most soft tissue sarcomas are treated with surgery combined with radiation, while bone sarcomas usually require chemotherapy in addition to surgical resection. Overall, physicians expect a high cure rate for most patients with localized tumours and without metastatic disease.

Sarcoma surgeries are typically complex and lengthy procedures. A surgery like Tony’s involves removing cancerous tissues, implanting titanium bone replacements and transplanting muscles from his back to his shoulder. It can take over eight hours. Dr. Gladdy and her colleagues often spend 15 to 20 hours in the operating room removing complex abdominal sarcomas, which are even more challenging to remove because of their location.

“It’s like running a marathon,” says Dr. Gladdy.

For some patients, that marathon is extended by chemotherapy and/or radiation treatments.  And for others with more advanced sarcomas, which often spread to other parts of the body, chemotherapy can be the only treatment option.

Unfortunately, for patients whose tumours have already metastasized the outlook is not nearly
as optimistic as it is for patients with localized sarcomas. Currently, chemotherapy alone is not a very effective treatment for metastatic sarcoma — only about 15 to 20 per cent of patients
respond well to it. New options are desperately needed, yet there have been relatively few
improvements in non-surgical treatments for advanced sarcomas in the last 20 years.

This is the challenge Dr. Albiruni Abdul Razak, who heads the unit’s medical oncology team, is tackling. Dr. Abdul Razak and his team are searching for new medicines that will target  sarcomas more effectively. His team is currently leading more than five early-phase clinical
trials within the program to evaluate the safety and effectiveness of potential new sarcoma
treatments, including drugs that target the blood supply of tumours, which could prevent them from growing, and new chemotherapy agents. These trials enable the Sarcoma Unit to offer patients innovative therapies five or more years earlier than other hospitals.

“There are some promising new treatments, and we’re involved in many of these trials from the
very beginning,” says Dr. Abdul Razak. “Now our patients have access to some really cutting-edge therapies, like immunotherapy, which activates the immune system to fight the tumours.”

Start at the very beginning: Sarcoma research

Treatments continue to improve as research provides new insights, and new options become
available. While the Sarcoma Unit’s cure rate of 75 per cent is one of the world’s highest, the
team is constantly looking for ways to better care for their patients, improve their odds of survival and preserve or improve their quality of life.

Tony and patients like him benefit from the team’s relentless drive for improvement, and the research behind it. Had Tony been diagnosed even a few years earlier, he might have lost his entire arm.

Sarcoma research at Mount Sinai takes place on several fronts, primarily better understanding
tumour growth and genetics, and developing more effective treatments. Research is embedded in the culture of the unit, with many of the clinicians also working as scientists. Dr. Gladdy, who spends about 80 per cent of her time on research, values this approach.

“I often find that treating patients raises questions we might not otherwise think of, which we can then explore in the lab — and hopefully, ultimately bring new knowledge back to the clinical setting to improve care,” she says. “It’s also incredibly rewarding to take discoveries and treatments from the lab and validate them through our patients.”

Dr. Gladdy’s current research focuses on understanding how sarcomas develop. Her lab grows tumours, tracking each genetic event in the process. The objective is to find a way to stop tumours from growing, and provide patients with more non-surgical treatment options. This approach is already leading down some exciting new paths, including a pre-clinical trial to see if inhibiting a specific gene found in children who develop sarcomas can prevent tumour growth.

At the same time, Dr. Wunder investigates the genetic causes of sarcomas together with Dr. Irene Andrulis, the Anne and Max Tanenbaum Chair in Molecular Medicine and a senior
investigator at the LTRI. Drs. Wunder and Andrulis have a long history of collaboration and are
looking to develop more specific treatments for different sarcoma types. This “personalized
medicine” approach allows their team to determine the most effective treatments using each tumour’s unique genetic information. This work relies heavily on the extensive Sarcoma Tumour Biorepository (the “Tumour Bank”) that they were instrumental in developing, which provides the necessary tissue samples.

“Almost all of our patients agree to let us bank their tumour tissue for research,” notes Dr. Wunder. “They want us to learn from them, so we can help others. It’s an incredible resource.”

The future

Today, ten years after his operation, Tony remains in excellent health, living a full and active life. Aside from limited mobility in his left shoulder and some impressive battle-scars, there’s no
visible sign of his treatment. But his experience as a sarcoma patient has never left him. 

“I’d been fundraising for health care for years, but it really changed my perspective,” says Tony. “I had a new appreciation for how many people are touched by illness — not just me, but everyone around me. And at Mount Sinai, we all got the care we needed.”

His care at Sinai also led to a new relationship with the hospital, as part of the fundraising
team that helps make this important, leading research and care possible. “I was looking for an opportunity to give back to Sinai,” recalls Tony. “I made a donation right away, but I wanted to do more. Seeing the dedication of the physicians and researchers first-hand is incredibly inspiring. I truly believe that the work they’re doing today will change everything for sarcoma patients. I’m so proud to be a part of it, supporting everyone who supported me.”


-Riannon John
Photo: John Packman

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Mount Sinai Hospital Foundation

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