Sarcoma

Overview

Sarcoma refers to a broad group of cancers that start in the bones and soft tissues. Soft tissues connect, support and surround other body structures. Soft tissues include muscle, fat, blood vessels, nerves, tendons and the lining of the joints. Cancer that starts in the soft tissue is called soft tissue sarcoma.

There are many types of sarcomas. They can happen in many parts of the body. Treatment for sarcoma varies depending on sarcoma type, where it is and other factors.

Symptoms

Signs and symptoms of sarcoma include:

• A lump that can be felt through the skin that may or may not be painful.
• Bone pain.
• A bone that breaks with no clear cause, such as with a minor injury or no injury.
• Belly pain.
• Weight loss.

When to see a doctor

Make an appointment with a doctor or other healthcare professional for symptoms that worry you.

Causes

It's not clear what causes sarcoma. This cancer starts as a growth of cells in the bones and soft tissues.

Sarcoma happens when cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA tells the cells to grow and multiply at a set rate. The DNA also tells the cells to die at a set time.

In cancer cells, the DNA changes give other instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.

The cancer cells might form a mass called a tumor. The tumor can grow to invade and destroy healthy body tissue. In time, cancer cells can break away and spread to other parts of the body. When cancer spreads, it's called metastatic cancer.

Risk factors

Factors that can increase the risk of sarcoma include:

• Inherited syndromes. Some syndromes that raise the risk of cancer can pass from parents to children. For instance, syndromes that increase the risk of sarcoma include Li-Fraumeni syndrome, familial retinoblastoma and neurofibromatosis type 1.
• Radiation therapy for cancer. Radiation treatment for cancer raises the risk of getting a sarcoma later.
• Lymphedema. Lymphedema is swelling that happens when something blocks or damages the lymphatic system. Lymphedema raises the risk of a type of sarcoma called angiosarcoma.
• Exposure to viruses. The virus called human herpes virus 8 can raise the risk of a type of sarcoma called Kaposi sarcoma in people with weakened immune systems.

Diagnosis

Sarcoma diagnosis often begins with a physical exam. This cancer happens in the bones and soft tissues. The tests you have may depend on the part of your body that's affected. Other tests may include imaging tests and a procedure to remove a sample of cells for testing.

Physical exam

Your healthcare professional may ask about your symptoms and your health history. A physical exam can help your healthcare professional understand your symptoms and look for other clues that help with your diagnosis.

Imaging tests

Imaging tests make pictures of the inside of the body. The pictures can help your healthcare team understand the size of your sarcoma and where it is.

The imaging tests you have depend on your condition. Some tests, such as X-rays, are better for seeing bones. Other tests, such as MRIs, are better for seeing connective tissues. Other imaging tests may include ultrasound, CT, bone scans and positron emission tomography scans, also called PET scans.

Biopsy

A biopsy is a procedure to remove a sample of tissue for testing in a lab. How a healthcare professional collects the biopsy sample depends on where the affected tissue is.

A biopsy for sarcoma may involve placing a needle through the skin to get the sample. Or it may involve surgery. Sometimes a surgeon does a biopsy during surgery to remove the cancer.

Treatment

Treatment for sarcoma often involves surgery to remove the cancer. Other treatments for sarcoma may include radiation therapy, ablation therapy and treatment with medicines. Medicines that help treat sarcoma include chemotherapy, targeted therapy and immunotherapy.

Which treatments are best for you will depend on the type of sarcoma you have. Your treatment options also might depend on where the cancer is in your body, how fast it's growing and whether it has spread to other parts of your body.

Surgery

The goal of surgery for sarcoma is to remove all the cancer cells. Sometimes surgeons need to remove a limb, such as an arm or a leg, to get all the cancer. This is called amputation. But surgeons try to save the limb when they can.

Sometimes surgeons can't remove all the cancer without hurting nearby nerves or organs. Then surgeons work to remove as much of the sarcoma as they can.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources.

For sarcoma, you might have radiation therapy before, during or after surgery to remove the cancer.

Chemotherapy

Chemotherapy treats cancer with strong medicines. Some types of sarcoma are more likely to respond to chemotherapy treatment than are others. Healthcare professionals might use chemotherapy before or after surgery. It also can help treat sarcoma that grows large or spreads to other parts of the body.

Targeted therapy

Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die.

Your healthcare professional may have your sarcoma cells tested to see if they are likely to respond to targeted therapy medicines. Targeted therapy may treat certain types of sarcoma. It might be used if the cancer comes back or spreads.

Immunotherapy

Immunotherapy for cancer is a treatment with medicine that helps the body's immune system kill cancer cells. The immune system fights off diseases by attacking germs and other cells that shouldn't be in the body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells find and kill the cancer cells.

Tests can show if a sarcoma is likely to respond to immunotherapy. This treatment might help control a sarcoma that has spread to other parts of the body.

Ablation therapy

Ablation is a procedure that applies treatment directly to the cancer cells to harm them. Some types of ablation apply energy to the cancer cells that causes them to heat up. Procedures that do this include radiofrequency ablation and microwave ablation.

Using extreme cold to harm the cancer cells is called cryoablation. Sometimes ablation involves using alcohol to harm the cancer cells.

Ablation might be used to treat sarcoma when surgery isn't an option. It also might be used when the cancer spreads to other parts of the body.

Coping and support

With time, you'll find what helps you cope with your sarcoma diagnosis. Until then, you may find that it helps to:

Learn enough about sarcoma to make decisions about your care

Ask your healthcare team about your cancer. Ask about your test results and the treatments you might have.

If you like, ask about the likely outcome of your condition, called prognosis, with and without treatment. As you learn more about cancer, you may feel better about making treatment decisions.

Keep friends and family close

Strong relationships can help you deal with your cancer. Friends and family can give you the help you need. For instance, they can take care of your home if you're in the hospital. They can serve as emotional support when you feel like your cancer is too much to handle.

Find someone to talk with

Find a good listener who is willing to hear you talk about your condition. This may be a friend or family member. A counselor, medical social worker, clergy member or cancer support group also may be helpful.

Ask your healthcare team about support groups in your area. In the United States, look to the National Cancer Institute and the American Cancer Society for more information.

Preparing for an appointment

Start by making an appointment with a doctor or other healthcare professional if you have symptoms that worry you.

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a certain test. Make a list of:

• Your symptoms, including symptoms that don't seem linked to the reason for your appointment, and when they began.
• Key personal information, including major stresses, recent life changes and family medical history.
• All medications, vitamins or other supplements you take, including the doses.
• Questions to ask your healthcare team.

Bring a family member or friend with you, if possible, to help you remember the information you're given.

For sarcoma, some basic questions to ask include:

• What's likely causing my symptoms?
• Other than the most likely cause, what are other possible causes for my symptoms?
• What tests do I need?
• What's the best course of action?
• I have other health conditions. How can I manage them together?
• Should I see a specialist?
• Are there brochures or other printed material I can have? What websites do you suggest?

Be sure to ask all the questions you have.

What to expect from your doctor

Your healthcare team is likely to ask you questions, such as:

• Do you have your symptoms all the time or do they come and go?
• How bad are your symptoms?
• What, if anything, seems to make your symptoms better?
• What, if anything, appears to make your symptoms worse?