Cancer Types | Sarcoma - National Foundation for Cancer Research

Sarcoma

Sarcoma

Sarcomas are cancers that start in bones, muscles, connective tissues, blood vessels or fat, and can be found anywhere in the body. There are more than 50 different types of sarcoma, which fall into two main categories: bone cancers and soft tissue cancers.

Key Facts

  • An estimated 13,130 new cases of soft tissue sarcomas and 3,600 new cases of bone cancers will be diagnosed in the U.S. in 2020, with around 7,070 deaths expected to result from the diagnosis.
  • Sarcomas are rare in adults and make up approximately 1% of all adult cancer diagnoses.
  • Sarcomas are relatively more common among children. Between 1,500 and 1,700 children are diagnosed with a bone or soft tissue sarcoma in the U.S. each year. This makes up about 15% of cancers in children under the age of 20.
  • The overall relative five-year survival rate for people with soft tissue sarcoma is around 65% and for people with bone cancer, the overall relative five-year survival rate is 70%.
  • When the sarcoma starts in an arm or leg, the five-year survival rates are slightly higher for each stage when compared with sarcoma that starts in other locations
Source: American Cancer Society’s Cancer Facts & Figures 2020; Sarcoma Alliance; and American Society of Clinical Oncology’s Cancer.Net

Sarcoma Research

In addition to specific projects listed below, genomics research is helping us attack sarcoma – and all types of cancer. NFCR has distinguished itself from other organizations by emphasizing long-term, transformative research and working to move people toward cancer genomics.

Yellow Sarcoma Ribbon
16730
new cases of sarcoma expected in 2020
7070
deaths expected in 2020
65
% 5-year survival rate for soft tissue sarcoma
Cesare Spadoni, Ph.D.
Cesare Spadoni, Ph.D.

Dr. Cesare Spadoni is leading a team to develop treatments for rhabdomyosarcoma, the most common pediatric soft tissue sarcoma. The aggressive subtype, alveolar rhabdomyosarcoma, results from the abnormal fusion of two genes and is difficult to treat. These young patients have a poor prognosis. Dr. Spadoni’s team is using the small molecule drug, volasertib, a highly potent inhibitor of the enzyme PLK1 – resulting in reduced activity and stability of the abnormal fused proteins. Significantly, the anti-cancer effects of volasertib are more pronounced when combined with chemotherapy agent, vincristine. Volasertib has already been studied as single agent in Phase I clinical trials in children with leukemia and refractory solid tumors. With support from the NFCR AIM-HI Translational Research Initiative, volasertib has advanced in pre-clinical research toward clinical trials. A Phase I clinical trial will begin in 2020 to bring this combined treatment to patients with relapsed or refractory rhabdomyosarcoma, with and without the gene fusion abnormalities.

Wei Zhang, Ph.D.
Wei Zhang, Ph.D.

Dr. Wei Zhang has devoted his entire career to the pursuit of precision oncology – specifically to the key molecular and genomic events that drive the development and progression of cancer. Over the last 20 years, Dr. Zhang has identified multiple novel cancer markers and oncogenic signaling molecules, including targets for the treatment of a soft tissue sarcoma that develops from tissues surrounding nerves called malignant peripheral nerve sheath tumor (MPNST). Results have provided evidence that the tyrosine kinase receptor pathway is a potential therapeutic target for patients with MPSNT.

Findings from these studies could give oncologists new diagnostic tools to improve disease management and patient survival.

Related Content

Sarcoma: The Young Person’s Cancer?

Sarcoma Origin Identified

Cancer is anything but a straightforward disease; one tumor type can be entirely different from another not only in location, but causal agent and origin. There is differentiation even within the same type of cancer. Take sarcoma, which is in fact an umbrella term for nearly 100 cancers originating in the skeleton or the soft parts of the body. Some affect the young, others the elderly. But now a study out of Sweden is bringing the starting points of some sarcomas into focus by identifying for the first time an interaction between specific proteins. “We now know which mechanisms to shine the spotlight on,” says Pierre Åman, Professor of Tumor Biology at Sahlgrenska Academy, University of Gothenburg, the corresponding author behind an article published in the journal EMBO Reports. Osteosarcomas develop in bone; liposarcomas form in fat; rhabdomyosarcomas form in muscle; Ewing sarcomas form in bone and soft tissue, and Kaposi’s sarcoma is practically synonymous with HIV/AIDS infection. However, among cancers, sarcoma is relatively uncommon; in the United States, around 12,750 people — 7,240 men and boys and 5,510 women and girls  — will be diagnosed with a soft-tissue sarcoma (STS) this year. The overall five-year survival rate for people with STS is around 65 percent; if the cancer is caught early and has not left its point of origin, the survival rate can be as high as 81 percent; if the sarcoma has spread to another area of the body, the 5-year survival rate falls precipitously to 16 percent. However, rates vary based on the specific type and the stage, or extent, of the sarcoma, showing how even just one type of cancer can have wildly different subsets. In the Swedish study, the 15 or more different forms of sarcoma studied were found to be caused by mutations in the FET family of proteins, involved in the regulation of lifespan and stress resistance, acting partially through the insulin/IGF-signaling pathway. The new results show that most of the tumor-altered FET proteins bind to another protein complex, SWI/SNF, which regulates gene activity, cell maturity and growth. This interaction results in SWI/SNF misregulation and, accordingly, disruptions in the genetic programming within a cell. The misregulation is a mechanism common to all the 15 or more forms of tumor caused by mutations in the FET genes. This now gives oncologists a solid target upon which to aim their efforts, and could clear the way for some type of screening technology. Importantly, the FET family is already a focus of scientific inquiry: they are of medical interest because chromosomal rearrangements of their genes promote not only various sarcomas, but because point mutations in FUS or TAF15 (both FET) can cause neurodegenerative diseases such as amyotrophic lateral sclerosis and frontotemporal lobar dementia. Because of that, “There are already an abundance of new drug candidates that affect this protein complex and are being tested on other diseases,” Åman says. “With the discoveries we’ve now made, we can test the same candidates on these forms of sarcoma as well.” July is Sarcoma Awareness Month, and studies such as Åman’s continue to shed light on the cancer’s ultimate […]

Rhabdomyosarcoma: The Muscle Cancer That Isn’t

In a proof-positive that cancer research still has miles to cover, a pediatric cancer long associated with aberrant muscle cells has now been found to arise from cells that would normally develop into cells lining blood vessels. Oncologists at St. Jude Children’s Research Hospital researching rhabdomyosarcoma, the most prevalent soft tissue cancer in children, made the discovery earlier this year, publishing their results in the Cancer Cell science journal. Most importantly, this newfound origin is hoped to lead to new insight for a cancer that has seen very little in the way of scientific progress regarding cures. “We are still using the same chemotherapy that was in use 46 years ago, with the same outcomes,” said Mark Hatley, M.D., Ph.D., of St. Jude’s Department of Oncology. “A better understanding of the machinery of rhabdomyosarcoma could enable entirely new treatment approaches.” Hatley led a team that spanned the highest levels of academia: Andrew McMahon, then at Harvard University, had genetically engineered a mouse to have a biological switch that enabled researchers to selectively turn on a key piece of cellular machinery called the Hedgehog pathway. Abnormal activation of this pathway was known to trigger cancers. Jonathan Graff at the University of Texas Southwestern Medical Center used this model to study the role of the hedgehog pathway in fat cell development, however the animals developed head and neck tumors. Hatley, along with Rene Galindo, Eric Olson and in collaboration with Graff, determined these tumors were rhabdomyosarcoma. But a fundamental question remained. “While these tumors appear to be muscle cells under the microscope, and clinicians had thought that they arose from muscle progenitor cells, that didn’t explain why the tumors can occur in tissues that don’t have skeletal muscle, like bladder, prostate and liver,” Hatley continued. “These tumors were not driven by muscle cells at all, so we decided to zero in on the biological machinery to find the cell of origin in these mouse tumors.” Upon closer inspection, it was found that the cancer was originating in the blood vessels between muscle cells, specifically, cells that would mature into the lining the inner surface of blood vessels. Also found was that the cancer grew at a particularly quick rate in not after birth, but very much before. “When we studied the mice at the embryonic stage, we saw the cells between the muscle fibers expanded explosively and formed tumors early in development,” Hatley said. A significant finding was that the tumor cells had characteristic biological machinery that drives development of head and neck muscles, which may help explain why rhabdomyosarcomas tend to occur in the head and neck. More over, Hatley’s team found evidence that a cancer-activating pathway, called KRAS, which can trigger rhabdomyosarcoma, also leads to the development of another kind of cancer called angiosarcoma. “This finding told us that the tumors in our model depended on activation of the Hedgehog pathway,” Hatley said. “But it also suggested there are likely multiple cells of origin for rhabdomyosarcoma. The different location of such rhabdomyosarcomas may depend, for example, on the cell of origin.” Now that cancer scientists and researchers have a clear […]