Sarcomas are cancers that start in bones, muscles, connective tissues, blood vessels or fat, and can be found anywhere in the body. There are more than 50 different types of sarcoma, which fall into two main categories: bone cancers and soft tissue cancers.
- An estimated 13,130 new cases of soft tissue sarcomas and 3,600 new cases of bone cancers will be diagnosed in the U.S. in 2020, with around 7,070 deaths expected to result from the diagnosis.
- Sarcomas are rare in adults and make up approximately 1% of all adult cancer diagnoses.
- Sarcomas are relatively more common among children. Between 1,500 and 1,700 children are diagnosed with a bone or soft tissue sarcoma in the U.S. each year. This makes up about 15% of cancers in children under the age of 20.
- The overall relative five-year survival rate for people with soft tissue sarcoma is around 65% and for people with bone cancer, the overall relative five-year survival rate is 70%.
- When the sarcoma starts in an arm or leg, the five-year survival rates are slightly higher for each stage when compared with sarcoma that starts in other locations
Source: American Cancer Society’s Cancer Facts & Figures 2020; Sarcoma Alliance; and American Society of Clinical Oncology’s Cancer.Net
In addition to specific projects listed below, genomics research is helping us attack sarcoma – and all types of cancer. NFCR has distinguished itself from other organizations by emphasizing long-term, transformative research and working to move people toward cancer genomics.
Dr. Cesare Spadoni is leading a team to develop treatments for rhabdomyosarcoma, the most common pediatric soft tissue sarcoma. The aggressive subtype, alveolar rhabdomyosarcoma, results from the abnormal fusion of two genes and is difficult to treat. These young patients have a poor prognosis. Dr. Spadoni’s team is using the small molecule drug, volasertib, a highly potent inhibitor of the enzyme PLK1 – resulting in reduced activity and stability of the abnormal fused proteins. Significantly, the anti-cancer effects of volasertib are more pronounced when combined with chemotherapy agent, vincristine. Volasertib has already been studied as single agent in Phase I clinical trials in children with leukemia and refractory solid tumors. With support from the NFCR AIM-HI Translational Research Initiative, volasertib has advanced in pre-clinical research toward clinical trials. A Phase I clinical trial will begin in 2020 to bring this combined treatment to patients with relapsed or refractory rhabdomyosarcoma, with and without the gene fusion abnormalities.
Dr. Wei Zhang has devoted his entire career to the pursuit of precision oncology – specifically to the key molecular and genomic events that drive the development and progression of cancer. Over the last 20 years, Dr. Zhang has identified multiple novel cancer markers and oncogenic signaling molecules, including targets for the treatment of a soft tissue sarcoma that develops from tissues surrounding nerves called malignant peripheral nerve sheath tumor (MPNST). Results have provided evidence that the tyrosine kinase receptor pathway is a potential therapeutic target for patients with MPSNT.
Findings from these studies could give oncologists new diagnostic tools to improve disease management and patient survival.