Shedding Light on Sarcoma
July is Sarcoma Awareness Month.
Sarcomas are a rare group of cancers in which malignant cells form in the bones or soft tissues of the body.
Soft tissue sarcomas form in cartilage, fat, muscle, blood vessels, tendons, nerves, and around joints.
Osteosarcomas develop in bone; liposarcomas form in fat;
rhabdomyosarcomas form in muscle; and
Ewing sarcomas form in bone and soft tissue.
Bone and joint cancer is most frequently diagnosed among teenagers, while soft tissue cancers typically affect those 55 years or older.
In 2017, over 12,300 cases of soft tissue sarcoma and 3,200 cases of bone sarcomas will be diagnosed in the United States, according to the National Cancer Institute’s Surveillance, Epidemiology, and End Results Program (SEER). Approximately 5,000 and 1,550 people are expected to die from soft tissue and bone sarcomas, respectively. The five-year survival rate for soft-tissue sarcomas is 64 percent, while the rate is 67 percent for bone sarcomas.
Because sarcomas are difficult to distinguish from other cancers when they are found within organs, their incidence is probably underestimated, according to the National Cancer Institute.
Having certain inherited disorders can increase the risk for soft-tissue sarcomas, including retinoblastoma, tuberous sclerosis, Werner syndrome, and nevoid basal cell carcinoma syndrome. Other risk factors for soft tissue sarcoma include past treatment with radiation therapy for certain cancers; exposure to certain chemicals, such as thorium dioxide, vinyl chloride, or arsenic; and long-term lymphedema in the arms or legs.
Past treatment with radiation can increase the risk of osteosarcoma and other types of bone cancers. Other risk factors for osteosarcoma include treatment with anticancer drugs called alkylating agents, having a certain change in the retinoblastoma gene, and having certain conditions including Paget disease, Diamond-Blackfan anemia, and Werner syndrome.
What is the AACR Doing in This Area?
Advances in Sarcomas: From Basic Science to Clinical Translation
In May 2017, the AACR hosted a special conference, Advances in Sarcomas: From Basic Science to Clinical Translation, which covered cutting-edge topics on the basic biology of sarcomas and soft-tissue tumors and how these may be translated to the clinic. Recognized experts in the field spoke on the topics of genomics, epigenetics, novel sarcoma models, immunotherapy, sarcoma biology, and targeted therapeutics.
Since 2013 the AACR has partnered with the
QuadW Foundation to offer the QuadW Foundation-AACR Fellowship for Clinical/Translational Sarcoma Research. The 2017 grantee was Michael J. Wagner, MD, of the University of Texas MD Anderson Cancer Center, for his project, "Overcoming primary resistance to VEGF/VEGFR targeted agents in angiosarcoma."
A 2017 AACR-Aflac, Inc. Career Development Award for Pediatric Cancer Research was also awarded to Amit J. Sabnis, MD, of the University of California, San Francisco, for his sarcoma-focused study, "PAX3-FOXO1 requires activation of mTOR by GATOR2 in rhabdomyosarcoma."
Studies in AACR Publications
According to a study from Frédéric Amant, MD, PhD, of the Leuven Cancer Institute in Belgium and the Netherlands Cancer Institute, the protein P-S6S240 may serve as an indicator of poor prognosis for patients with a hard-to-treat type of uterine sarcoma called leiomyosarcoma, and preclinical data suggest that patients whose tumors have this protein may respond to PI3K/mTOR inhibitors. This study was published in Clinical Cancer Research, a journal of the American Association for Cancer Research.
The AACR's mission is to
prevent and cure all forms of cancer.