Sarcomas are a diverse and relatively rare group of malignanttumors that develop in soft tissue and bone. Soft tissue sarcomas form in cartilage, fat, muscle, fibrous tissue, blood vessels, and other connective or supportive tissues of the body; osteosarcomas develop in bone; liposarcomas form in fat;rhabdomyosarcomas form in muscle; 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. In 2014, it is estimated that approximately 12,000 cases of soft tissue sarcoma and 3,000 cases of bone sarcomas will be diagnosed in the United States; approximately 4,700 and 1,500 people are expected to die from soft tissue and bone sarcomas, respectively.
Soft tissue1 and bone sarcoma incidence rates have increased over the past 35 years. Soft tissue sarcomas are more common than bone sarcomas. There are several subtypes of both soft tissue sarcoma and bone sarcoma, but the exact number of Americans with each sarcoma subtype is unknown. The 5-year relative survival rate for both bone and soft tissue sarcoma is approximately 65 percent.
Certain inherited disorders and past treatment with radiation therapy can increase the risk of soft tissue sarcoma and some bone sarcomas. Standard treatments for bone and soft tissue sarcoma include surgery, chemotherapy, and radiation therapy. In addition to those treatments, patients with bone sarcoma and uterine sarcoma may receive samarium 153 and hormone therapy, respectively. Children with soft tissue sarcoma may receive targeted therapy.
Source: Surveillance, Epidemiology, and End Results (SEER) Program and the National Center for Health Statistics. Additional statistics and charts are available at the SEER Web site.
NCI’s Molecular Profiling-Based Assignment of Cancer Therapy for Patients With Advanced Solid Tumors (M-PACT) trial is assessing whether choosing specific treatment options based on tumor gene variations is more effective than standard cancer treatments. M-PACT could identify patient subgroups that are likely to benefit from certain treatments and result in new treatments being developed quickly for some cancers.
Researchers with The Cancer Genome Atlas (TCGA) program are systematically identifying the major genomic changes involved in more than 20 cancers, including sarcoma, using state-of-the-art genomic technologies.
Two sarcoma-specific Specialized Programs of Research Excellence (SPOREs) are developing treatments targeted to the molecular, genetic, epigenetic, andsignaling pathway alterations for specific types of sarcoma and translating biological and technological advances into improvements in prevention, diagnostics, predictors of outcome, and advances in the treatment of sarcoma.
Selected Advances in Sarcoma Research
Source: NCI Funded Research Portfolio. Only projects with assigned common scientific outline area codes are included. A description of relevant research projects can be found on the NCI Funded Research Portfolio Web site.
Genomic analysis of three types of soft tissue sarcomas—myxoid liposarcomas, synovial sarcomas, and osteosarcomas—identified mutations that may drive cancer development or progression. Published November 2013. [PubMed Abstract]
Studies in cell lines and mouse xenograft models showed that addition of thetopoisomerase inhibitor SN-38 increases the sensitivity of Ewing sarcoma cells to the cytotoxic effects of the anti-tumor drug trabectedin, suggesting that this combination may have promise for treating patients with the disease. Published November 2013. [PubMed Abstract]
A comprehensive analysis of 147 rhabdomyosarcoma tumors and normal paired tissues identified multiple recurrently mutated genes. Published January 2014. [PubMed Abstract]
NCI’s investment2 in soft tissue and bone sarcoma research was $37.5 million in fiscal year (FY) 2013. In addition to the funding described in the graph, NCI supported $22.6 million in sarcoma research in FYs 2009 and 2010 using funding from the American Recovery and Reinvestment Act.
Source: NCI Office of Budget and Finance.
Additional Resources for Sarcoma
Soft Tissue Sarcoma Home Page Information about soft tissue sarcoma treatment, clinical trials, research, statistics, and other topics from the National Cancer Institute.
Bone Cancer Home Page Information about bone cancer treatment, clinical trials, research, and other topics from the National Cancer Institute.
Uterine Sarcoma Home Page Information about uterine sarcoma treatment and clinical trials from the National Cancer Institute.
Ewing Sarcoma Home Page Information about Ewing sarcoma family of tumors: treatment and clinical trials from the National Cancer Institute.
Bone Cancer A fact sheet about the diagnosis and treatment of cancers that develop in the bones.
1 Does not include Kaposi sarcoma, which is addressed in a separate Snapshot.
2 The estimated NCI investment is based on funding associated with a broad range of peer-reviewed scientific activities. For additional information on research planning and budgeting at the National Institutes of Health (NIH), see About NIH.
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