Sarcoma 101

What is sarcoma?

Sarcoma is cancer in connective tissue or nonepithelial tissue. Connective tissue is composed of fat, blood vessels, nerves, bones, muscles, deep skin tissues, and cartilage.

What are the types of sarcomas?

There are two main types: soft tissue sarcoma and bone sarcoma. Within these main types, there are also approximately 100 different kinds of sarcoma that are based on the type of cell of origin. Soft tissue comprises 1% of all adult cancers and is considered rare. Soft tissue can metastasize (spread) and form secondary tumors. Bone sarcoma is considered very rare with only 2,890 new cases diagnosed every year in the United States. Bone sarcomas are usually not life-threatening but can compromise healthy bone tissue.

How prevalent is sarcoma? Who is affected?

In the United States, there are about 14,000 new cases of diagnosed every year. 15% of cancers diagnosed in people under the age of 20 are sarcomas. Over half of the people diagnosed with sarcoma are younger than 60 years old.

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. Long-term exposure to wood preservatives with chlorophenols, phenoxyacetic acid in herbicides, and vinyl chloride found in plastics can elevate risk. There have been families with multiple cases of sarcoma, some of which 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  DICER1syndrome.

How do patients get diagnosed?

Surgical biopsies can determine whether a tumor 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 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”