July is Sarcoma Awareness Month – Here’s What You Need to Know

Sarcomas are a complex type of cancer that can trick the body into thinking the tumor is merely normal tissue. They develop in connective tissue, cartilage, bone, fat, and muscle. Sarcomas occur much more prevalently in children (about 15% of cancer cases) compared to adults (1%) in the U.S. 

The most common form of sarcoma in the U.S. is gastrointestinal stromal tumor (GIST), which affects approximately 4,000 to 6,000 people annually. Per the Fred Hutch Cancer Center, sarcoma can be diagnosed by: 

1. Imaging tests such as an MRI, CT scans, and PET scans to visually detect sarcoma. 

2. A biopsy whereby a medical practitioner extracts tissue to confirm the results of an imaging test. 

Immunotherapy’s Long-Standing Impact Against Sarcomas 

The study of immunotherapy treatment for sarcomas can be traced to William Coley, MD, who is regarded as the Father of Cancer Immunotherapy, and developed a bacteria-based immunotherapy approach called ‘Coley’s Toxins’ that was used in the treatment of sarcoma patients over 100 years ago.  

In 1953, Dr. Coley’s daughter and CRI co-founder Helen Coley Nauts, alongside George A. Fowler, MD, and Frances H. Bogatko, MD, published a series of monographs, several focusing on sarcomas, detailing outcomes for patients who were treated with Coley’s toxins. These treatments, and Nauts’ drive to keep her father’s legacy alive, were critical in developing the field of cancer immunotherapy research, and the progress of treatment against sarcomas. 

In fact, in 2017 CRI spoke with Donald Foley, who at the time was one of the last surviving patients treated at Memorial Hospital in New York City with the world’s first cancer immunotherapy treatment: Coley’s toxin. Foley’s doctor was none other than Bradley Coley, MD, the son of Dr. William Coley. 

“It was 1953, a long, long time ago, and it was bone cancer. They didn’t know as much as they know today… they told my parents that the diagnosis was not good… I had three months to live,” Foley recalled. “I had… the toxin treatment in the afternoon. Every day I would feel my shoulder, and I could notice a difference. I could feel the tumor shrinking.” 

Today, sarcomas still present challenges for immunologists. CRI Postdoctoral Fellow Md Torikul Islam, PhD at the University of Texas Southwestern Medical Center, says that while immunotherapy is an emerging treatment option for sarcomas, many patients with osteosarcoma, a type of bone cancer, become resistant. 

“To make immunotherapy more effective, understanding the mechanisms by which cancer cells evade immune cells is critical,” Dr. Islam says. “The patient care and survival rate haven’t improved in the last 30-40 years… but we are making strides in understanding its mechanisms and developing new treatments.” 

To Dr. Islam’s point, several FDA-approved cancer immunotherapy treatments have been produced in the past decade. Three are checkpoint inhibitors that target the PD/PD-L1 pathway for the treatment of various sarcomas, and one is a monoclonal antibody that targets the RANKL pathway for the treatment of patients with bone cancer. There are currently four cancer vaccines that are being evaluated in clinical trials for sarcoma treatment. 

CRI’s Impact on Sarcoma Immunotherapy Research 

Currently, CRI is funding several scientists who are diligently researching sarcoma cancer immunotherapy treatments. In recent years, CRI-funded scientists researched sarcoma-related projects such as immunotherapy that implemented genetically engineered CD8+ T cells designed to trigger tumor regression and T cell-dependent cancer immunoediting. Five years ago, CRI launched a partnership with the Chordoma Foundation to advance treatments and improve patient outcomes for chordoma, a rare form of sarcoma that is diagnosed in one in one million people annually worldwide. CRI-Chordoma Foundation CLIP Investigator Judith Bovee, MD, PhD, professor of pathology at Leiden University Medical Center in the Netherlands, conducts research to improve the diagnosis, prognosis, and treatment for sarcoma patients. 

“Sarcomas are classified based on their resemblance to normal tissue. For instance, bone tumors are classified according to their resemblance to normal bone or cartilage in various stages of development,” Dr. Bovee explains. “Sarcomas are rare, and some subtypes are even ultrarare. International collaboration and exchange of expertise is key, and the patient’s voice is important in helping to raise awareness with caregivers but also with regulators and funding agencies.” 

CRI is encouraged both by stories like Foley’s, where he outlived a dire prognosis by many decades, and with immunologists such as Drs. Islam and Bovee who strive to make sarcoma a cancer of the past. With their advocacy and research acumen, and your support, we can make a world immune to cancer.