Undifferentiated Pleomorphic Sarcoma

Undifferentiated pleomorphic sarcoma (UPS) is a rare cancer that starts in the body's soft tissues. These soft tissues are like the stretchy, supportive material that cushions and connects different parts of your body, like muscles, tendons, and fat.

Most often, UPS develops in the arms or legs. Sometimes, it can form in the area behind your organs in your abdomen (called the retroperitoneum).

The name "undifferentiated pleomorphic sarcoma" describes what the cancer cells look like under a microscope. "Undifferentiated" means the cancer cells don't look like the normal cells of the body tissue where they grew. "Pleomorphic" means the cancer cells come in many different shapes and sizes. This difference in shape and size makes it hard to identify them as normal cells.

Treating UPS involves a combination of strategies, often including surgery to remove the tumor, radiation therapy to target the cancer cells, and medicine (chemotherapy) to kill the cancer cells. The best treatment plan depends on where the cancer is located in the body.

Previously, this type of cancer was called malignant fibrous histiocytoma.

Undifferentiated pleomorphic sarcoma is a type of cancer that can develop almost anywhere in the body, but it's most common in the arms and legs. The symptoms of this cancer vary depending on its location.

One of the most common signs is a noticeable lump or swelling that grows over time. As the tumor gets bigger, it might cause pain, a prickly or numb feeling, or both.

If the cancer is in an arm or leg, the hand or foot on that same side might also swell.

If the sarcoma is in the belly (abdomen), you might experience pain, a reduced desire to eat, and trouble having bowel movements (constipation).

Other possible symptoms include a fever and unintentional weight loss.

It's important to see a doctor if you notice any of these persistent signs or symptoms that concern you. Don't ignore anything that feels unusual or bothersome. Early detection and treatment are key to managing this type of cancer.

If you have any ongoing health problems that concern you, schedule a visit with your doctor.

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Undifferentiated pleomorphic sarcoma is a type of cancer whose cause isn't fully understood.

We know that this cancer starts when a cell's DNA, which acts like a set of instructions for the cell, gets damaged. These changes tell the cell to grow and divide very quickly. This rapid growth creates a lump of abnormal cells called a tumor. These abnormal cells can push into and harm surrounding healthy tissues. Over time, these cancer cells can detach and travel to other parts of the body, like the lungs or bones, where they can form new tumors. This spread is called metastasis.

Undifferentiated pleomorphic sarcoma is a type of cancer. While some things might make a person more likely to get this cancer, many people who get it don't have any obvious reasons why.

One factor that can increase the risk is age. Most people diagnosed with this cancer are over 50, but it can affect people of any age.

Another possible risk factor is previous radiation therapy. In very rare cases, this cancer can develop in a part of the body that has had radiation treatment before.

It's important to remember that many people who develop undifferentiated pleomorphic sarcoma have no known risk factors at all. Also, many people with risk factors never get the cancer. So, while these factors might slightly increase the chance of getting the cancer, they don't guarantee it.

Finding out if you have undifferentiated pleomorphic sarcoma usually starts with a visit to your doctor. They'll ask about your symptoms, when they started, and how they've changed. They'll also examine the affected area, checking its size, how deep it is, if it's connected to nearby tissues, and looking for any swelling or signs of nerve problems.

Your doctor might order some imaging tests to get a better picture of what's happening. These tests can include X-rays, CT scans, MRI scans, and PET scans. These tests help create detailed images of the area to understand the cancer better.

To know for sure what's going on, your doctor needs to take a sample of the tumor tissue (a biopsy). This tissue sample is sent to a lab where specially trained doctors (pathologists) look at the cells under a microscope. They check the type of cells and if they seem likely to spread quickly. This helps the doctors figure out if it's undifferentiated pleomorphic sarcoma and rule out other types of cancer. The type of biopsy needed and exactly how it's done is carefully planned. This is crucial because the biopsy shouldn't interfere with any future surgery to remove the cancer. It's important to ask your doctor for a referral to a team of experts in soft tissue sarcomas before the biopsy. This specialized team has the experience to do the biopsy in a way that won't cause problems later.

At Mayo Clinic, our team of experts is here to provide the best possible care for your undifferentiated pleomorphic sarcoma concerns. We can help you every step of the way.

Undifferentiated Pleomorphic Sarcoma: Treatment Options and Support

Undifferentiated pleomorphic sarcoma is a type of cancer. It often requires surgery to remove the cancer cells. The goal of surgery is to remove as much cancer as possible, along with some healthy tissue surrounding it, to reduce the risk of the cancer returning. This is done with the least possible impact on the body.

Treatment Options

Several approaches may be used to treat undifferentiated pleomorphic sarcoma, depending on the size, location, and stage of the cancer.

• Surgery: Doctors aim to remove the entire tumor. If the cancer is in a limb, they often try to save the limb (limb-sparing surgery). However, amputation might be necessary in some cases. Sometimes, radiation therapy or chemotherapy is used before surgery to shrink the tumor, making it easier to remove without amputation.

• Radiation Therapy: This uses high-energy beams (like X-rays or protons) to kill cancer cells. There are two main types:

  • External Beam Radiation: A machine directs radiation beams to specific spots on the body. This can be used before surgery to shrink the tumor or after surgery to eliminate any remaining cancer cells.
  • Intraoperative Radiation Therapy (IORT): Radiation is used during surgery, targeting the area where the cancer was removed. This might be a better option if the cancer is in a location that makes complete removal during surgery difficult.

• Drug Treatments (Systemic Therapies): These treatments use medications to fight cancer throughout the body. Examples include:

  • Chemotherapy: Uses chemicals to kill cancer cells. It's often used if the cancer comes back after initial treatment or spreads. Sometimes, chemotherapy is used before surgery to shrink the tumor.
  • Targeted Therapy: Targets specific abnormalities within cancer cells, causing them to die. These drugs may be used in combination with chemotherapy for some cancers. A lab test may be needed to determine if targeted therapies will work for a particular person's cancer.
  • Immunotherapy: Uses the body's own immune system to fight cancer. This is often used for advanced cancers. The immune system might not recognize and attack the cancer because the cancer cells have proteins that help them hide. Immunotherapy helps the immune system to recognize and attack the cancer cells.

Important Considerations

• Treatment Planning: The best treatment plan depends on the individual's specific situation. Doctors will consider the size, location, and stage of the cancer when deciding on a course of action.
• Support: A cancer diagnosis can be overwhelming. It's important to seek support from friends, family, and if needed, counselors, support groups, or clergy. Learning as much as possible about the cancer and treatment options can help you feel more in control. Talking to a trusted friend or family member, counselor, medical social worker, or clergy member can help you express your feelings and concerns.

Alternative Treatments

While there aren't proven alternative treatments for undifferentiated pleomorphic sarcoma, some complementary therapies can help manage the side effects of cancer and treatment. These might include acupuncture, exercise, massage, meditation, and music therapy.

Coping with a Cancer Diagnosis

It's essential to:

• Learn about the cancer: Ask your doctor questions about the cancer, treatment options, and prognosis.
• Maintain relationships: Strong relationships with friends and family can provide practical and emotional support.
• Seek support: Find a trusted listener—a friend, family member, counselor, medical social worker, clergy member, or cancer support group—to talk to about your concerns and hopes.

This information is for general knowledge and does not constitute medical advice. Always consult with your doctor for personalized guidance and treatment plans.

A diagnosis of undifferentiated pleomorphic sarcoma can be very stressful. It's okay to feel overwhelmed. But there are ways to cope, and things you can do to feel more in control.

One important step is learning about the sarcoma. Talk to your doctor about the specific type of sarcoma you have, including the different treatment options available. Understanding your treatment choices and, if you wish, the likely course of the disease, can help you feel more prepared and confident.

Strong relationships with friends and family are crucial during this time. Having supportive people around will help you manage the practical and emotional aspects of your treatment. They can offer help with everyday tasks, like looking after your home while you're in the hospital, and provide a listening ear when you need it.

Talking to someone is another important way to cope. Whether it's a friend, family member, or a professional like a counselor, medical social worker, or clergy member, having someone to share your hopes and fears with can make a big difference. You can also find support groups for people with cancer. Your doctor can tell you about local support groups. You can also look for them in your phone book, at the library, or on the websites of organizations like the National Cancer Institute or the American Cancer Society.