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 fat, muscle, fibrous tissue, blood vessels, and other supporting tissue of the body; osteosarcomas develop in bone; and Ewing sarcomas (EWS) 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 2013, it is estimated that approximately 11,400 Americans will be diagnosed with soft tissue sarcomas and 3,000 with bone sarcomas; approximately 4,400 and 1,440 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 35 years. Soft tissue sarcomas are more common than bone sarcomas. Several subtypes of soft tissue and bone sarcoma exist, but the exact number of Americans with each sarcoma subtype is unknown. The 5-year relative survival rate for bone and soft tissue sarcoma is approximately 66 percent.
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.
Examples of NCI Activities Relevant to Sarcoma
- NCI supports the phase I trial Autologous T Cells and Cyclophosphamide in Treating Patients With Soft Tissue Sarcoma That Is Metastatic or Cannot Be Removed By Surgery, which is testing a combination of adoptive immunotherapy and chemotherapy to treat patients with advanced soft tissue sarcoma.
- 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.
- NCI funds Supportive and Palliative Care Clinical Trials that explore ways to manage the physical, psychological, and emotional side effects of cancer and cancer therapies, including those experienced by patients undergoing treatment for sarcoma.
- The NCI-supported 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 NCI-supported 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.
- Two sarcoma-specific Specialized Program of Research Excellence (SPORE) are developing therapies targeted to the molecular, genetic, epigenetic, and signaling pathway alterations for specific types of sarcoma.
Selected Advances in Sarcoma Research
- In a randomized clinical trial, adjuvant chemotherapy consisting of doxorubicin and ifosfamide provided no survival benefit for patients with resected soft tissue sarcoma. Published September 2012. [PubMed Abstract]
- A retrospective cohort study showed that patients diagnosed with EWS at age 40 and over are more likely to have extraskeletal tumors, metastatic disease, primary tumors at sites other than the arms and legs, and lower survival rates than younger EWS patients, suggesting a fundamental difference in tumor biology. Published September 2012. [PubMed Abstract]
- The results of preclinical studies suggest that a noninvasive positron emission tomography scan may help identify patients with liposarcoma who are likely to respond to gemcitabine treatment. Published December 2012. [PubMed Abstract]
- In a phase II trial, 84 percent of patients with advanced alveolar soft part sarcoma experienced a partial response or stable disease when treated with the experimental angiogenesis inhibitor cediranib. Published April 2013. [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 or to complete a search tutorial.
Trends in NCI Funding for Sarcoma Research
The National Cancer Institute's (NCI) investment2 in soft tissue and bone sarcoma research increased from $34.3 million in fiscal year (FY) 2008 to $41.0 million in FY 2011 before decreasing to $38.9 million in FY 2012. 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
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 Fact Sheet
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.
- 3 For more information regarding ARRA funding at NCI, see Recovery Act Funding at NCI.