Updated June 25, 2019
What is sarcoma?

Sarcoma is a group of cancers that often start in the soft tissue or bone. Soft tissue is composed of fat, blood vessels, nerves, muscles, deep skin tissues, and cartilage.

What are the types of sarcomas?

Soft tissue sarcoma and bone sarcoma are the main types, but within these main categories, there are approximately 100 different kinds of sarcoma that are based on the type of cell of origin. Soft tissue sarcoma comprises 1% of all adult cancers and is considered rare. The most common locations for soft tissue sarcomas include the arms, legs, and abdomen. Bone sarcoma is also considered rare, with fewer than 3,000 new cases diagnosed every year in the United States, but it is still the most common type of bone cancer.

How prevalent is sarcoma? Who is affected?

In the United States, it is estimated that there are 15,000 new cases of sarcoma diagnosed each year, with many young people affected. Sarcomas make up 20% of cancers in childhood versus 1% of cancers in adults, and approximately half of sarcoma cases occur before age 40. For more information, check out OHSU’s sarcoma information page.

What causes sarcoma?

Although we do not fully understand what triggers or influences sarcoma development, there are a few contributors that have been linked to sarcoma. There have been families with multiple cases of sarcoma, some of whom have been found to have rare hereditary conditions that cause an increased risk of sarcoma and potentially other cancers. Examples of hereditary conditions linked with sarcoma include Li-Fraumeni syndrome and  DICER1 syndrome. Long-term exposure to wood preservatives with chlorophenols, phenoxyacetic acid in herbicides, and vinyl chloride found in plastics can also elevate risk.

How do patients get diagnosed?

Surgical biopsies can be used to identify a tumor, including whether it is  benign or malignant. Tissue is removed and examined to make note of the grade (the degree of abnormality of the cancer cells), which can predict the probable growth rate and likelihood to metastasize (spread).

What is the treatment for sarcomas?

Treatment options differ depending on the type, size, grade, and spread of the tumor. Treatment may include surgery (most common), radiation therapy (before or after surgery or to shrink tumors), and/or chemotherapy (used with radiation, prior to or following surgery).
When should a hereditary reason for sarcoma be suspected?
Most sarcomas are sporadic, meaning they happen due to chance, environment, and/or other factors that we still do not fully understand. Researchers are currently studying whether it may be appropriate for all people younger than age 50 with sarcoma to undergo genetic testing for hereditary causes of sarcoma. Clues that currently point to genetics evaluation potentially being helpful include:

  • Rhabdyomosarcoma before 3 years old
  • Osteosarcoma before 10 years old
  • Multiple cases of sarcoma or sarcoma and other cancers on one side of the family

More resources:
Sarcoma Foundation of America
Sarcoma Alliance
“Identification, management, and evaluation of children with cancer-predisposition syndromes.”
“Germline Mutations in Cancer Predisposition Genes are Frequent in Sporadic Sarcomas”