A Snapshot of Sarcoma
Incidence and Mortality
Sarcomas are a diverse and relatively rare group of malignant tumors 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.
Examples of NCI Activities Relevant to Sarcoma
- The phase II clinical trial Cabozantinib for Adults With Advanced Soft Tissue Sarcoma is assessing the effectiveness of cabozantinib as a treatment for patients with soft tissue sarcoma that did not respond to prior treatment.
- The clinical trial Phase II Study of Cediranib (AZD2171) 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.
- 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.
- NCI’s Division of Cancer Epidemiology and Genetics is conducting population and multidisciplinary research to discover the genetic and environmental causes of cancer, including several studies on osteosarcoma.
- 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, and signaling 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
- 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 the topoisomerase 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]
- The multi-kinase inhibitor pazopanib and the histone deacetylase inhibitors valproate and vorinostat interacted in an additive to greater-than-additive fashion to cause the death of sarcoma cells in cell culture and to reduce tumor volume in sarcoma-bearing mice. Published February 2014. [PubMed Abstract]
Trends in NCI Funding for Sarcoma Research
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.
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.
- Adult Soft Tissue Sarcoma Treatment (PDQ®)
Expert-reviewed information summary about the treatment of soft tissue sarcoma.
- Ewing Sarcoma Treatment (PDQ®)
Expert-reviewed information summary about the treatment of the Ewing sarcoma family of tumors.
- Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment (PDQ®)
Expert-reviewed information summary about the treatment of osteosarcoma.
- Uterine Sarcoma Treatment (PDQ®)
Expert-reviewed information summary about the treatment of uterine sarcoma.
- Clinical Trials for Soft Tissue Sarcoma
- Clinical Trials for Ewing Sarcoma
- Clinical Trials for Osteosarcoma
- Clinical Trials for Uterine Sarcoma
- 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.