Undifferentiated pleomorphic sarcoma

Undifferentiated pleomorphic sarcoma is a rare type of cancer. It starts mostly in the body's soft tissues. Soft tissues connect, support and surround other body structures. Soft tissues include muscle, fat, blood vessels, nerves, tendons and linings of the joints.

Undifferentiated pleomorphic sarcoma typically forms in a leg or an arm. Less often it can happen in the area behind the organs in the belly. That area is called the retroperitoneum.

This cancer gets its name from the way the cancer cells look under a microscope. Undifferentiated means the cells don't look like the healthy cells around them. Pleomorphic (plee-o-MOR-fik) means the cells grow in many shapes and sizes.

Treatment depends on where the cancer is located. Treatment often involves surgery, radiation therapy and medicines.

Symptoms of undifferentiated pleomorphic sarcoma depend on where the cancer is located. It most commonly forms in a leg or an arm. But it can happen anywhere in the body.

Signs and symptoms may include:

• A painless slow-growing lump or area of swelling.
• A feeling of pain or pressure.
• Loss of appetite.
• Weight loss.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you have ongoing symptoms that worry you.

The exact cause of undifferentiated pleomorphic sarcoma isn't known.

Cancer happens when cells get 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 different 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.

Factors that may raise the risk of undifferentiated pleomorphic sarcoma include:

• Older age. This cancer is most common in people 50 to 70 years old. But it can happen at any age.
• Previous radiation therapy. Rarely, this cancer can form in an area of the body that was previously treated with radiation therapy.

Most people who develop undifferentiated pleomorphic sarcoma have no known risk factors. Many people who have risk factors never develop this cancer.

Diagnosis of undifferentiated pleomorphic sarcoma usually starts with a review of symptoms and a physical exam. Imaging tests and a biopsy also may be used to diagnose this form of cancer.

Physical exam

A healthcare professional may ask when symptoms started and if they've changed over time. A physical exam of the area where symptoms are happening can help the healthcare team understand:

• The size and depth of the growth.
• If the growth may be connected to nearby tissues.
• Whether there are signs of swelling or nerve damage.

Imaging tests

Imaging tests make pictures of the body. They can provide information that helps the healthcare team learn more about your condition. Imaging tests may include X-rays, CT, MRI and positron emission tomography (PET) scans.

Biopsy

A biopsy is a procedure to remove a sample of tissue for testing in a lab. The sample may be collected using a needle to draw out some of the tissue. Or sometimes surgery may be needed to remove a tissue sample.

In the lab, doctors trained in analyzing body tissues, called pathologists, look at the tissue sample to examine the types of cells and see whether the cells are likely to be aggressive. This information helps rule out other types of cancer and guide treatment.

Treatment for undifferentiated pleomorphic sarcoma typically involves surgery to remove the cancer. Radiation therapy often is used along with surgery. Other treatments may include medicines, such as chemotherapy, targeted therapy and immunotherapy. Treatment choices are based on the size and location of the cancer.

Surgery

The goal of surgery is to remove the sarcoma completely, along with an area of healthy tissue around it, when possible. Sometimes radiation therapy is done before surgery to shrink the cancer. This may lower the risk of the cancer coming back after surgery.

When the cancer affects an arm or a leg, surgery to keep the limb is used whenever possible. Although it's not common, sometimes the arm or leg may need to be removed. That procedure is called amputation.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. The energy beams can come from X-rays, protons or other sources. Radiation therapy might be done before surgery to help shrink the sarcoma. Or it can be done after surgery to kill cancer cells that might be left behind.

Chemotherapy

Chemotherapy treats cancer with strong medicines. Chemotherapy may be used to treat undifferentiated pleomorphic sarcoma that comes back after surgery or radiation therapy. It also might be used when cancer spreads to other parts of the body. Sometimes chemotherapy is used before surgery to shrink the cancer so that it's easier to remove. Or chemotherapy may be used after surgery to lower the risk that the sarcoma will come back.

Targeted therapy

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

Some targeted therapies only work in people whose cancer cells have certain genetic changes. Because of that, the cancer cells may be tested in a lab to see if targeted therapy might help.

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.

No alternative treatments have been found helpful in treating undifferentiated pleomorphic sarcoma. But complementary and alternative treatments might help ease some symptoms or side effects of cancer treatment.

Examples of treatments that might help include acupuncture, exercise and massage. Activities called mindfulness-based interventions, such as yoga, relaxation and music therapy, also may be useful. If you're interested in trying alternative treatments, talk with your healthcare professional first.

With time, you may find what helps you cope with worries or other feelings that may come with learning you have undifferentiated pleomorphic sarcoma. Until then, here are some ideas to help you cope with a cancer diagnosis.

Learn enough about your diagnosis to make decisions about your care

Talk with your healthcare team about undifferentiated pleomorphic sarcoma. Ask about your test results and treatment choices. As you learn more, you may become more confident in making treatment decisions.

Find a good listener

Finding someone who is willing to listen to you talk about your hopes and fears can be helpful as you manage your diagnosis. This could be a friend or family member. A counselor, medical social worker or clergy member also may offer helpful guidance and care.

Connect with others

Other people who have had sarcoma may be able to offer you unique support and insight because they understand what you're experiencing. Ask your healthcare team about support groups in your area or contact the American Cancer Society. Find support online through Mayo Clinic Connect, which is a community where you can connect with others for support, practical information and answers to everyday questions.

Make an appointment with a healthcare professional if you have any symptoms that worry you. If your healthcare professional suspects that you might have undifferentiated pleomorphic sarcoma, you'll likely be referred to a doctor who treats cancer, called an oncologist.

Undifferentiated pleomorphic sarcoma is rare, and it often requires complex care. This type of cancer is best handled by a team of experts who have a lot of experience treating it.

Because there's often a great deal of information to talk about, it's a good idea to be prepared for your appointment. Here's some information to help you get ready.

What you can do

When you make the appointment, ask if there's anything you need to do beforehand, such as not eating or drinking for a certain amount of time. Make a list of:

• Your symptoms, including any that seem unrelated to the reason for your appointment.
• Key personal information, including major stresses, recent life changes and family medical history.
• All medicines, vitamins or other supplements you take, including the doses.
• Questions to ask your healthcare professional.

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

For undifferentiated pleomorphic sarcoma, some basic questions to ask your healthcare professional 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 are my treatment options?
• What are the potential side effects of each treatment option?
• I have other health conditions. How can I best manage them together?
• Should I see a specialist?
• Are there brochures or other printed materials I can take with me? What websites do you recommend?

Be sure to ask any other questions you may have.

What to expect from your doctor

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

• When did your symptoms start?
• Are your symptoms always present, or do they come and go?
• How severe are your symptoms?
• What, if anything, makes your symptoms better?
• What, if anything, makes your symptoms worse?