A Snapshot of Sarcoma

Incidence

Sarcomas are a diverse and relatively rare group of malignant tumors that develop in soft tissue and bone. Soft tissue sarcomas form in fat, muscles, nerves, joints, blood vessels, and deep skin tissues; osteosarcomas develop in bone; and Ewing sarcomas form in bone and soft tissue. Sarcomas can be difficult to distinguish from other malignancies when they are found within organs; thus, their incidence is probably underestimated. Because sarcomas are more common in adolescents and young adults than most other cancers, the number of years of life lost to sarcomas is substantial, despite their relatively low incidence. In 2012, it is estimated that approximately 11,280 Americans will be diagnosed with soft tissue sarcomas and 2,890 with bone sarcomas; approximately 3,900 and 1,410 people are expected to die from soft tissue and bone sarcomas, respectively.

Soft tissue¹ and bone sarcoma incidence rates have increased slightly over the past 30 years; however, soft tissue sarcoma is more deadly, possibly because the lack of specific symptoms at early disease stages may lead to delays in diagnosis. Several subtypes of soft tissue and bone sarcoma exist; the exact number of Americans with each sarcoma subtype is unknown.

Certain inherited disorders and past treatment with radiation therapy can increase the risk of soft tissue and bone sarcoma. No modifiable risk factors for sarcoma have been identified. Standard treatments for soft tissue sarcoma include surgery, chemotherapy, and radiation therapy. In addition to those treatments, patients with bone sarcoma and uterine sarcoma may receive samarium and hormone therapy, respectively.

Trends in NCI Funding for Sarcoma Research

The National Cancer Institute's (NCI) investment² in sarcoma research¹ increased from $37.1 million in fiscal year (FY) 2007 to $41.0 million in FY 2011. In addition to this funding, NCI supported $22.6 million in sarcoma research in FY 2009 and 2010 using funding from the American Recovery and Reinvestment Act (ARRA).³

Examples of NCI Activities Relevant to Sarcoma

• The Chemopreventive Agent Development Research Group provides scientific and administrative oversight for chemoprevention agent development from preclinical research to early phase I clinical trials. The program currently supports research on potential osteosarcoma preventive agents that contain natural plant antioxidants, which may prevent free-radical damage to DNA and other molecules.
• NCI's Strategic Partnering to Evaluate Cancer Signatures (SPECS) program supports large collaborative research groups that explore how information from molecular studies can be used to improve the care and outcomes of cancer patients. One SPECS project is refining and validating molecular signatures to provide a more accurate diagnosis of childhood sarcomas and to better predict their clinical behavior.
• The Therapeutically Applicable Research to Generate Effective Treatments (TARGET) initiative is identifying and validating treatment targets to develop new, more-effective treatments for children with pediatric cancers, including osteosarcoma.
• The NCI Experimental Therapeutics (NExT) program brings together researchers from academia, industry, and government to facilitate cancer drug discovery and development.
• The Phase II Study of Cediranib in Patients with Alveolar Soft Part Sarcoma is testing an experimental drug that blocks the growth of new blood vessels to a tumor for the treatment of alveolar soft part sarcoma, a rare type of soft tissue sarcoma that is most common in teenagers and young adults.
• The first sarcoma-specific Specialized Program of Research Excellence (SPORE) is developing therapies targeted to the molecular, genetic, epigenetic, and signaling pathway alterations for specific types of soft tissue sarcoma.

Selected Advances in Sarcoma Research

• Researchers identified several genetic variants associated with osteosarcoma. Published May 2011. [PubMed Abstract]
• Initial results from an NCI-funded clinical trial indicate that the investigational drug cediranib shrinks tumors in patients with alveolar soft part sarcoma. Reported June 2011.
• A new technique provides insights into the process that creates rearrangements in genomic DNA known as translocations, which may play a role in the development of some sarcomas. Reported October 2011.
• A study reveals how EWS-FLI, an abnormal protein caused by a translocation that is frequently present in Ewing sarcoma, causes changes in chromatin structure that may lead to cancer. Published November 2011. [PubMed Abstract]
• See this PubMed list of selected free full-text journal articles on NCI-supported research relevant to sarcoma. You can also search PubMed for additional scientific articles.