Undifferentiated pleomorphic sarcoma (UPS)

Undifferentiated pleomorphic sarcoma (UPS) is a rare type of cancer called sarcoma. It used to be known as malignant fibrous histiocytoma.

UPS most often develops in the soft tissues that connect, support and surround other body structures. Soft tissues include muscle, fat, blood vessels, nerves, tendons and the linings of joints. Rarely, UPS can start in bone. UPS of bone is considered a bone sarcoma and is treated differently from soft tissue UPS.

Undifferentiated pleomorphic sarcoma most often forms in a leg or an arm. Less often, it can happen in the area behind the organs in the belly 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 for UPS depends on where the cancer is located and whether it has spread. Treatment often involves surgery, radiation therapy and medicines.

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

Signs and symptoms may include:

• A growing lump or area of swelling.
• A feeling of pain or pressure.

UPS symptoms can be different if the cancer has spread to other parts of the body. When cancer spreads, it's called metastatic cancer. UPS that has spread most often spreads to the lungs. If the cancer has spread to the lungs, symptoms may include shortness of breath, a cough or chest pain.

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 gives instructions that help control when cells grow, multiply and die.

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 cancer cells to build up.

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, which is called metastasis.

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. UPS in children is rare.
• 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 (UPS) usually starts with a review of symptoms and a physical exam. Imaging tests and a biopsy may be used to diagnose this cancer.

Physical exam

A physical exam can help healthcare professionals decide what tests are needed. A healthcare professional may ask when symptoms started and whether they've changed over time.

Imaging tests

Imaging tests make pictures of the body. They can provide information that helps the healthcare team learn more about the size and location of the cancer. They also give information about nearby tissues. Imaging tests may include:

• X-ray.
• Magnetic resonance imaging (MRI).
• Computerized tomography (CT) scan.
• Positron emission tomography (PET) scan.

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 soft tissues.

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. Imaging tests can help show where to biopsy. Sometimes surgery may be needed to remove a tissue sample if a biopsy is hard to do because of the location.

In the lab, doctors trained in analyzing body tissues, called pathologists, look at the tissue sample to examine the types of cells. Because UPS is rare, pathologists who specialize in sarcoma can help confirm the diagnosis.

Treatment for undifferentiated pleomorphic sarcoma (UPS) that has not spread typically involves surgery to remove the cancer. Cancer that has not spread is called localized. 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 when possible. An area of healthy tissue also is removed to make sure all cancer cells are removed.

When the cancer affects an arm or a leg, surgery is done in a way that saves the limb whenever possible. Sometimes the arm or leg may need to be removed, although this is not common. 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 control the tumor. Or it might be done after surgery to kill cancer cells that might be left behind. The goal of radiation therapy before or after surgery is to lower the risk of the cancer coming back.

Chemotherapy

Chemotherapy treats cancer with strong medicines. Chemotherapy may be used for some people with UPS, including when the cancer has spread or come back. 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. The benefit of chemotherapy for localized UPS isn't clear.

Immunotherapy

Immunotherapy is a cancer treatment with medicine that helps the body's immune system kill cancer cells. Immunotherapy is being studied in localized and advanced UPS.

Targeted therapy

Targeted therapy is a cancer treatment that uses medicines to attack specific ways that cancer cells can grow or spread. By blocking these paths, targeted treatments can cause cancer cells to die.

Palliative care

Palliative care focuses on relieving symptoms and improving quality of life at any stage of cancer. Treatments such as surgery, radiation therapy or medicines may be used to relieve symptoms even when eliminating the cancer isn't possible. Palliative care can be provided alongside treatments intended to control the cancer.

Prognosis

The outlook for someone with undifferentiated pleomorphic sarcoma (UPS) depends on many things and is different from person to person. Prognosis depends on the features of the cancer and how well treatment works.

Other important factors include whether the cancer has spread to organs or bones and a person's overall health.

Because so many things affect the outlook, cancer statistics such as survival rates can't show exactly how long someone will live.

In general, survival rates for sarcoma are better when the cancer is localized, meaning it has not spread. UPS may be cured if it is localized and surgery can remove the entire tumor.

Your healthcare team knows the details of your cancer and treatment. Care team members can give you a personalized outlook and help you make sense of what it means for you. Your care team may share a range of survival outcomes to help you plan for the future. Some people want to know as much as possible about their prognosis, while others choose to focus on treatment and day-to-day life. It's up to you to decide how much information is helpful.

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 with symptoms or side effects 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 before the appointment, such as not eating or drinking for a certain amount of time. Make a list of:

• Your symptoms, including any pain from where the tumor is located.
• Key personal information, including medical and surgical history and family medical history.
• All medicines, vitamins or other supplements you take, including the doses.

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

For undifferentiated pleomorphic sarcoma, some questions to ask your healthcare professional include:

• 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?
• 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?

The stage describes how much cancer there is and how far it has spread. Soft tissue sarcomas range from stages 1 through 4. A lower stage generally means the cancer is more limited. A higher stage means it is more advanced or has spread to other parts of the body.

Healthcare professionals determine the cancer's stage using several pieces of information, commonly summarized as TNM grade. TNM stands for:

• Tumor: The size of the primary tumor.
• Node: Whether the cancer has spread to nearby lymph nodes.
• Metastasis: Whether the cancer has spread to distant parts of the body.

The grade describes what the cancer cells look like under the microscope. The staging system divides sarcomas into 3 grades, 1 to 3. Low-grade tumors tend to be slow-growing and slower to spread. They often have a better outlook, called prognosis, than higher-grade tumors.

Staging isn't identical for every sarcoma. The tumor's location and type matter, and bone sarcomas have their own staging considerations. So a person's sarcoma can't necessarily be assigned a stage based on tumor size alone.

Both tumor grade and stage can help your care team determine the most appropriate treatment.