What is sarcoma?

Sarcoma is a rare type of cancer that arises in the musculoskeletal system. Unlike carcinoma — the most common type of cancer, which arises in organs such as breasts, prostates and the gastrointestinal tract — sarcoma is generally less understood, and the rarity of sarcoma can make it more difficult to recognize and diagnose. Here's what you should know about sarcoma.

What are the different types of sarcoma?

Cancer is named for the place where it starts, even if it metastasizes (spreads) to other parts of the body. For example, lung cancer is still lung cancer even if spreads to nearby lymph nodes or the adrenal glands.

Sarcoma is a general term for a group of about 70 different types of cancers that start in mesenchymal cells, which are cells that develop into connective or lymphatic tissue and blood vessels.

“Sarcoma is a cancer that develops from skeletal muscle, bone, soft tissue, tendons, ligaments, joints, and sometimes blood vessels,” says Dr. Suhel Kotwal, an orthopedic surgeon with HCA Midwest Health who specializes in complex orthopedic care including sarcomas, bone and soft tissue tumors in the pelvis and limbs and reconstruction in extremities after cancer. “They can also develop anywhere in the body but are most common in the arms, legs, chest and abdomen.”

Sarcomas are grouped into two main types: bone sarcoma and soft tissue sarcoma. There are about 65 different subtypes of soft tissue sarcoma and three main subtypes of bone sarcoma: chondrosarcoma, osteosarcoma and ewing sarcoma.

What are the signs and symptoms of sarcoma?

“They appear as lumps around the center of the body or on limbs. It can be a painless lump for a while and can get easily neglected as we tend to ignore these problems on a daily basis,” Dr. Kotwal says. Soft tissue sarcomas are usually painless, even when they grow larger.

"It’s important to know that these lumps or bumps are not to be ignored,” he says. “If the patient feels a painless lump they will generally to go to their primary care doctor and may then need to seek out expert care from a physician specializing in sarcomas," he says. If you notice a growing mass on your body, that could be a sign of soft tissue sarcoma.

What causes sarcoma, and what are the risk factors?

There isn't a clear cause of sarcoma; some sarcomas occur when an individual cell takes on multiple mutations and develops a cancerous behavior. Other sarcomas occur when chromosomes get misaligned or mismatched during cell division. These are known as translocation-based sarcomas.

There is no specific known risk factor for sarcoma, Dr. Kotwal says. However, some factors that can increase risk include:

• Family cancer syndromes
• Lymph system damage
• Exposure to certain chemicals
• Radiation treatment for other cancers

Men and women develop sarcoma equally, although it represents only about 1% of cancers in adults. However, sarcoma accounts for 15% of childhood cancers.

What are the treatment options for sarcoma?

Soft tissue sarcomas are typically treated with a combination of radiation therapy, surgery and sometimes, chemotherapy. For bone sarcoma, the main treatment is surgery and possibly chemotherapy, depending on the subtype.

What precautions can people take to reduce their risk?

Anyone can develop sarcoma, which means it is important to advocate for yourself in the healthcare system. Some patients report having their masses or long-standing pain dismissed without getting an imaging test to determine the cause.

Dr. Kotwal says factors that increase the risk of sarcoma include inherited conditions, radiation therapy for cancer, chronic swelling or lymphedema, exposure to industrial chemicals and herbicides, and exposure to certain viruses for people with weakened immune systems.

Depending on your insurance, your conversation may start with your primary care provider before you are referred to an orthopedic surgeon or oncologist.