When Terry Fox was diagnosed with osteogenic sarcoma in 1977, his options for treatment were limited. The first line of defence at the time was to amputate the affected limb (in Terry’s case his right leg) and harsh chemotherapies.
These life-altering treatments helped keep Terry’s cancer at bay for a few years (enough time for him to launch his Marathon of Hope and change cancer research and care in Canada forever), but his cancer would eventually return, taking hold of his lungs and leading to his premature death in 1981.
Since then, research has helped improve survival and quality of life for many patients diagnosed with sarcomas, but there’s still a lot to be done.
“The sarcoma that Terry had was incurable in over 80 per cent of cases before chemotherapy. Now the reverse is true: 70 to 80 per cent survive and in most cases with their limb intact,” says Dr. Livia Garzia, assistant professor at McGill University and principal investigator at the Research Institute of the McGill University Health Centre. “But sadly, Terry’s case is still pretty typical when it comes to metastatic sarcomas, and this is a huge gap that needs to be addressed through research.”
What are sarcomas anyway?
The term ‘sarcoma’ refers to a heterogenous group of cancers that have a common origin in connective tissue, such as muscle, bones, cartilage and fat. These cancers are quite rare, with 1,400 people being diagnosed with soft-tissue sarcomas and bone cancers in Canada in 2022.
While people of all ages can be diagnosed with sarcomas, they are more common among children and young adults.
Along with limited options to effectively treat metastatic cancers, one of the main gaps with sarcoma research today is that its various subtypes are poorly understood, according to Dr. Garzia and her colleague, Dr. Michael Monument, assistant professor at the University of Calgary and an adult and pediatric surgeon who completes the aggressive surgeries needed to remove sarcomas.
Both researchers agree that while advances in molecular profiling and genomic sequencing technologies have enabled the molecular characterization of sarcomas, including ultra-rare subtypes, there is more work to be done to acquire an in-depth knowledge of their biology.
For rare diseases like sarcoma, collaborative efforts across Canada are needed to accelerate scientific advances,” says Dr. Monument. “This should include a standardized national sarcoma clinical database, a Canada-wide sequencing effort to molecularly characterize all existing and newly diagnosed sarcomas and strategic infrastructure that enables more clinical trial opportunities for Canadian sarcoma patients.”
How research funded through the Terry Fox Research Institute and the Marathon of Hope Cancer Centres network is bringing new hope to patients with sarcoma
With her Terry Fox New Investigator Award, Dr. Garzia studied how and why certain sarcomas metastasize in the first place, with the aim of understanding what tumours are more at risk of becoming metastatic, why they resist therapy and identifying new treatments for children and young adults with these cancers.
In addition to creating model systems to understand, test and manipulate the metastatic process, Garzia’s team discovered that some bone sarcomas have errors in their epigenetics, or the way genes are turned on and off – an aspect of bone sarcoma’s biology was previously unrecognized.
When Dr. Monument isn't in the operating room, he spends time studying what makes sarcomas resistant to immunotherapies and testing novel strategies to overcome this.
As co-leader of the MOHCCN’s Prairie Consortium sarcoma cohort, he’s also using state of the art sequencing technologies and computer programming to profile the molecular blueprint of all new sarcoma diagnosed in southern Alberta. By tumour banking these sarcoma tissue samples for multi-omic sequencing initiatives (DNA, RNA, protein), his team hopes to create the most comprehensively annotated sarcoma clinical and sequencing dataset and develop computational strategies to support precision oncology reports for high-risk sarcomas.
“I take great pride and privilege in my role to cure patients of their sarcoma,” he says. “I dream of a future in Canadian sarcoma care where we have access to new drugs and immune-based therapies capable of decreasing tumour burden prior to surgery, preventing sarcoma relapse after surgery and treating metastatic tumours in patients with unresectable, advanced disease.”
Uniting Canadian researchers to improve the future of sarcoma research and care
Much like his Marathon of Hope that united Canadians, Terry’s dream of ending cancer through research will be achieved together, one step at a time.
“As we look across our great country, there is a growing density of talented clinicians, scientists and trainees who have embraced the mission of sarcoma research and care delivery. If we can unify and connect these talents and forge national sarcoma initiatives, I believe Canada can become a world leader in sarcoma research,” says Dr. Monument.
“About the patients who we are still failing to cure, we know we will need to attack their tumours with drugs that take in to account the specific features of each of them. A one-size-fits-all approach won’t work,” says Dr. Garzia.
Both Drs. Monument and Garzia are dedicated to their goals of ending the suffering caused by cancer, and as Dr. Garzia explains, the reason is simple. “I get up every day so that less patients will have to hear: Sorry, there is nothing we can do.”