Sarcomas are rare cancers that develop in the bones and soft tissues, including fat, muscles, blood vessels, nerves, deep skin tissues and fibrous tissues. According to the National Cancer Institute, about 12,000 cases of soft tissue sarcomas and 3,000 cases of bone sarcomas are diagnosed in the U.S. each year. Bone sarcomas are more common among children while soft tissue sarcomas are more common in adults.

Types of Sarcoma

Sarcomas are categorized as soft tissue or bone sarcomas, depending on where they develop in the body.

Soft Tissue Sarcomas

Soft tissue sarcomas originate in the soft tissues of the body and are most commonly found in the arms, legs, chest or abdomen. Soft tissue tumors can occur in children and adults. 

Bone Sarcomas

Bone sarcomas are primary bone tumors, which means that they develop in the bones. They are most commonly diagnosed in children. In addition to osteosarcoma, the most common form of primary bone cancer, there are several other types of bone tumors. 

Sarcoma Risk Factors

Most sarcomas do not have a known cause, although there are several factors that could increase a person’s risk of developing a sarcoma. The most common sarcoma risk factors include the following:

• History of radiation therapy. Patients who have received radiation therapy for previous cancers may have a higher risk of developing a sarcoma.
• Genetic disorders. Patients with a family history of inherited disorders, such as Von Recklinghausen’s disease (neurofibromatosis), Gardner syndrome, Werner syndrome, tuberous sclerosis, nevoid basal cell carcinoma syndrome, Li-Fraumeni syndrome or retinoblastoma, have a higher risk of developing a sarcoma.
• Chemical exposure. Exposure to vinyl chloride monomer (a substance used to make some types of plastics), dioxin or arsenic may increase the risk of sarcoma. However, most sarcomas are not known to be associated with specific environmental hazards.
• Long-term swelling. Having lymphedema, or swelling, in the arms or legs for a long time could increase your risk of developing a sarcoma.

Sarcoma Symptoms

Early signs of soft tissue sarcoma can include a painless lump or swelling. Some sarcomas may not cause any symptoms until they grow and press on neighboring nerves, organs or muscles. Their growth may cause pain, a feeling of fullness or breathing problems.

The most common symptoms of bone sarcomas include the following:

• Pain and/or swelling in an arm or leg, the trunk, the pelvis or the back; swelling may or may not feel warm to the touch
• A limited range of motion in a joint
• A fever of unknown origin
• A bone that breaks from no apparent cause

These symptoms could be signs of many other medical conditions. Always check with your doctor for a proper diagnosis.

Sarcoma Treatment

Sarcoma is treated with a combination of chemotherapy, radiation therapy and surgery. Reconstruction of the surgical area typically takes place at the same time the tumor is removed.

Your treatment plan and recovery will depend on a variety of factors, including the following:

• type of sarcoma
• tumor’s location, grade and size
• your age
• whether the cancer is new or recurrent.

For primary tumors, radiation is used in conjunction with surgery (either before or after) to reduce the risk of tumor recurrence.

For patients with metastatic disease (cancer that has spread to other areas of the body), stereotactic radiosurgery offers a noninvasive alternative to surgery. Patients with isolated metastasis might be treated with radiation in conjunction with chemotherapy as well as stereotactic radiosurgery. Radiation is an essential part of treating most high-grade tumors.