[8 MIN READ]

In this article:

• Sarcoma is a rare cancer that develops in the bones and soft tissues. There are many types of sarcoma, and it affects both children and adults.

• Signs of sarcoma can include pain, difficulty moving, swelling, unexplained weight loss and hard or soft lumps under the skin that grow bigger. 

• Surgery, chemotherapy and radiation are traditional treatments for sarcoma, but new options such as immunotherapy, cell therapy and targeted therapy are showing promise.  

Understanding sarcoma: types, signs and treatment advances

Sarcoma is a rare type of cancer that develops in the bones and soft tissues or connective tissues. This includes fat, muscle, blood vessels, cartilage, deep skin tissues and nerves. It shows up as a malignant (cancerous) tumor.

According to the American Society of Clinical Oncology (ASCO), sarcoma makes up about 1% of all adult cancer diagnoses. It accounts for about 15% of childhood cancer diagnoses.

Sarcoma isn’t widely understood. Let’s take a closer look in this detailed guide.

What is sarcoma?

Sarcoma tumors can appear anywhere in the body, but most often begin in the arms, legs, head, neck and back.

“There are more than 70 types of sarcomas,” says Jianguo Huang, Ph.D., assistant member of the Preclinical Cancer Therapy Laboratory at the Earle A. Chiles Research Institute, which is part of the Providence Cancer Institute. “Although the cause of most sarcomas is not fully understood, we know that genetic and environmental factors play a role.”

Possible risk factors for sarcoma include a genetic mutation or a family history. Exposure to radiation and certain chemicals or toxins also may cause sarcoma.

Types of sarcoma

There are two categories of sarcoma based on where they originate: bone sarcoma (osteosarcoma) and soft tissue sarcoma.

Bone sarcoma

Bone sarcoma can occur in any bone but is most commonly found in the long bones of the arms and legs. It tends to spread (metastasize) to other parts of the body.   

Osteosarcoma is also called osteogenic sarcoma. It is the most common type of bone sarcoma and can appear during periods of rapid bone growth. Because of this, it mostly affects adolescents and young adults.

Chondrosarcoma starts in the cartilage. Ewing sarcoma can show up in the bone or soft tissues. Typically, it appears in the pelvis, legs or arms. It also primarily affects children, adolescents and young adults. It is linked with a genetic abnormality. 

Soft tissue sarcoma

Soft tissue sarcomas can show up in various parts of your body, and there are many subtypes. These include:

• Angiosarcoma: affects the blood or lymph nodes
• Kaposi’s sarcoma: mainly affects the skin
• Leiomyosarcoma: occurs in the smooth muscles of the walls of organs, such as the abdomen
• Liposarcoma: begins in fat cells
• Rhabdomyosarcoma: forms in the skeletal muscles

Most soft tissue sarcomas (excluding rhabdomyosarcoma) occur in adults.

Signs of sarcoma

Symptoms of sarcoma vary among different types. Among the typical signs:

• Abdominal pain: or increasing girth
• Difficulty moving: limited range of motion or a limp
• Fatigue: persistent over time
• Lump: can grow, and may or may not hurt
• Pain: commonly in the arms, legs or back
• Swelling: near the site of the lump or pain
• Weight loss: without any known cause

“Lumps typically appear as rounded masses under the skin, and they can be soft or firm,” Dr. Huang explains. “They can grow noticeably bigger in just weeks and make one arm or leg appear larger than the other. You can have one lump or several.”

A lump may not cause pain until it grows and presses on nerves or muscles. Lumps tend to appear in the arms, legs, chest, back, abdomen or retroperitoneum (area behind the abdomen).

Signs of sarcoma are similar to those of many other conditions. You should see your doctor if you have symptoms.

Diagnosing sarcoma

To diagnose sarcoma, your doctor will review your health history and do a physical exam. You may also have other tests, such as:

• Biopsy: Your doctor removes tissue from your lump and sends it to a lab. Pathologists look at it under a microscope.
• Bone scan: Your doctor injects dye into your body to get an internal look at your bones.
• Other scans: Doctors may use tests such as a CT scan, MRI, PET scan, ultrasound and X-rays to check your bones and soft tissues for tumors.

If you have sarcoma, you will work with a group of healthcare professionals to treat it.

“At Providence, our expert multidisciplinary team walks with our patients during every step of the process,” says John Creasy, M.D., FACS, a surgical oncologist at the Providence Cancer Institute Franz Liver, Pancreas and Surgical Oncology Clinic. “They develop a personalized care plan and answer questions about the disease, cancer treatment options and outcomes.”

Sarcoma treatment

Your treatment depends on the type of sarcoma, its size and location and whether it has spread. You may have one treatment or a combination.

Traditional treatments include:

• Chemotherapy: drugs kill or slow the growth of cancer cells
• Radiation therapy: X-rays destroy cancer cells or radiation sources are implanted inside the body
• Surgery: surgeon physically removes the tumor

Recent advancements 

New and developing sarcoma treatments also are available, including:

• Cell therapy: genetically modifying immune cells to identify and attack cancer cells
• Immunotherapy: using the body’s immune system to recognize and attack cancer cells
• Targeted therapy: administering drugs that target molecules involved in cancer growth and progression
• Thermal ablation: inserting a needle into the tumor to heat or freeze it

Dr. Huang is helping advance cell therapy. “This can be so powerful because you don’t have the side effects of chemotherapy and radiation,” he says.

Research and clinical trials

Research to develop new treatments for sarcoma is ongoing.

“I want to fill in the gap in sarcoma research,” he explains. “Our team is studying the biology of sarcomas to better understand how to eliminate sarcoma cells.”

Conducting clinical trials can be limited by the complexity of sarcoma, Dr. Huang adds. “Because sarcomas are rare and genetically complex, developing clinical trials poses a unique challenge that must be addressed in preclinical studies.” 

However, Providence Cancer Institute offers various clinical trials for sarcoma. Talk with your doctor for more information.

Prognosis and managing sarcoma

The prognosis for sarcoma varies widely. It depends on the type and stage of the cancer, and if it has spread. It’s also based on your overall health and response to treatment. Earlier treatment tends to produce the best outcomes. 

Many people live for longer than five years after diagnosis. Dr. Huang points to specific outcomes for average five-year survival rates: 

• All sarcoma types: 65% survival rate
• Bone cancer: localized is 77%; if spread to nearby tissues, 65%
• Soft tissue sarcoma: localized is 81%; if it has spread to nearby tissues, 58%

Long-term management strategies

If you have sarcoma, you will work with your health care team to manage it over time. This can include:

• Regular follow-up care: You will have ongoing physicals and tests to monitor for any signs of progression or recurrence.
• Rehabilitation and support: Physical and occupational therapy help you regain strength and mobility. Psychosocial support and palliative care can help you live with sarcoma.

“Our oncology nurse navigators work closely with patients to provide support and care coordination,” Dr. Creasy says. “Patients can also access the National Comprehensive Cancer Network (NCCN), national sarcoma societies and local support groups.”

Providence offers experienced, compassionate care and leading-edge treatments for sarcoma. Ongoing research will provide even more treatment options and hope.

Contributing caregivers

John Creasy, M.D., FACS, is a liver and pancreas surgeon and surgical oncologist at Providence Cancer Institute in Portland, Oregon.   

Jianguo Huang, Ph.D., is an assistant member of the Preclinical Cancer Therapy Laboratory at the Earle A. Chlles Research Institute at the Providence Cancer Institute in Portland, Oregon. 

Find a doctor

Providence takes a highly personalized approach to sarcoma care. Our multidisciplinary cancer care teams include medical oncologists, orthopedic surgeons and radiation oncologists.

If you are looking for a primary care provider, you can search for one who’s right for you in our provider directory. 

Download the Providence app

It’s all in the app: easily stay connected with Providence and your health. With the Providence app, you can schedule appointments, have virtual visits from the comfort of your own home, get health recommendations personalized for you, access your health records and so much more. Learn more and download the app.

Related resources

Is it sarcoma or just a lump? 

3 types of cancers you’ve probably never heard of

Preclinical studies in soft tissue sarcomas may be tomorrow’s targeted therapies

This information is not intended as a substitute for professional medical care. Always follow your health care professional’s instructions.