Undifferentiated Pleomorphic Sarcoma
Undifferentiated pleomorphic sarcoma (UPS) is a rare type of cancer that begins mostly in the soft tissues of the body. Soft tissues connect, support and surround other body structures.
UPS usually occurs in the arms or legs. Less often it can happen in the area behind the abdominal organs (retroperitoneum).
The name undifferentiated pleomorphic sarcoma comes from the way the cancer cells appear under the microscope. Undifferentiated means the cells don’t look like the body tissues in which they develop. The cancer is called pleomorphic (plee-o-MOR-fik) because the cells grow in multiple shapes and sizes.
Treatment for UPS depends on the location of the cancer, but often involves surgery, radiation and drug treatments.
UPS used to be called malignant fibrous histiocytoma.
Undifferentiated pleomorphic sarcoma symptoms depend on where the cancer occurs. It most often happens in the arms and legs, but it can happen anywhere in the body.
Signs and symptoms may include:
- Growing lump or area of swelling.
- If it grows very large, there may be pain, tingling and numbness.
- If it occurs in an arm or leg, there may be swelling in the hand or foot of an affected limb.
- If it occurs in the abdomen, there may be pain, loss of appetite and constipation.
- Fever.
- Weight loss.
When to see a doctor
Make an appointment with a doctor if you develop any persistent signs or symptoms that worry you.
- Inflamed skin
- Scratch marks
- Bumps, spots or blisters
- Dry, cracked skin
- Leathery or scaly patches
Sometimes itchiness lasts a long time and can be intense. As you rub or scratch the area, it gets itchier. And the more it itches, the more you scratch. Breaking this itch-scratch cycle can be difficult.
When to see a doctor
See your health care provider or a skin disease specialist (dermatologist) if the itching:
- Lasts more than two weeks and doesn’t improve with self-care measures
- Is severe and distracts you from your daily routines or prevents you from sleeping
- Comes on suddenly and can’t be easily explained
- Affects your whole body
- Comes with other symptoms, such as weight loss, fever or night sweats
If the condition persists for three months despite treatment, see a dermatologist to be evaluated for skin disease. You may also need to see a doctor who specializes in internal medicine (internist) to check for other diseases.
It’s not clear what causes undifferentiated pleomorphic sarcoma.
Doctors know this cancer begins when a cell develops changes in its DNA. A cell’s DNA contains the instructions that tell a cell what to do. The changes tell the cell to multiply rapidly, creating a mass of abnormal cells (tumor). The cells can invade and destroy nearby healthy tissue. In time, the cancer cells can break away and spread (metastasize) to other parts of the body, such as the lungs and bones.
Factors that may increase the risk of undifferentiated pleomorphic sarcoma include:
- Older age. This cancer tends to occur in adults older than 50, though it can occur at any age.
- Previous radiation therapy. Rarely, this cancer can develop 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, and many people who have risk factors never develop the cancer.
Diagnosis for undifferentiated pleomorphic sarcoma usually starts with a review of your symptoms and a physical examination. This cancer is often diagnosed after other types of cancer have been ruled out.
Tests and procedures may include:
- Physical exam. Your doctor will ask you questions about when your symptoms began and if they’ve changed over time. He or she will examine the area to better understand the size and depth of the growth, whether it’s connected to nearby tissues, and whether there are any signs of swelling or nerve damage.
- Imaging tests. Your doctor may recommend imaging tests to create pictures of the affected area and understand more about your condition. Imaging tests may include X-rays, CT, MRI and positron emission tomography (PET) scans.
Removing a sample of tissue for testing (biopsy). To make a definitive diagnosis, your doctor collects a sample of the tumor tissue and sends it to a lab for testing. Depending on your particular situation, the tissue sample may be collected with a needle inserted through your skin or during an operation.
In the lab, doctors trained in analyzing body tissues (pathologists) examine the sample to determine the types of cells involved and whether the cells are likely to be aggressive. This information helps rule out other types of cancer and guides your treatment.
Determining the type of biopsy needed and the specifics of how it should be performed requires careful planning by the medical team. Doctors need to perform the biopsy in a way that won’t interfere with future surgery to remove the cancer. For this reason, ask your doctor for a referral to a team of experts with extensive experience in treating soft tissue sarcomas before the biopsy.
Treatment for undifferentiated pleomorphic sarcoma usually involves surgery to remove the cancer cells. Other options include radiation therapy and drug treatments (systemic therapies), such as chemotherapy, targeted therapy and immunotherapy. Which treatments are best for you will depend on the size and location of your cancer.
Surgery
When possible, doctors try to remove the sarcoma completely with surgery. The goal is to remove the cancer and a margin of healthy tissue around it with as minimal an impact as possible.
When the cancer affects the arms and legs, surgeons prefer to use limb-sparing operations. However, in some cases it may be necessary to amputate the affected arm or leg. Other treatments, such as radiation therapy and chemotherapy, might be recommended before surgery to shrink a cancer so that it’s easier to remove without amputating the affected limb.
Radiation therapy
Radiation therapy uses high-powered beams of energy, such as X-rays or protons, to kill cancer cells. Radiation therapy can be given as:
External beam radiation. This type of radiation comes from a machine that moves around you as you lie on a table. The machine directs the radiation to precise points on your body.
Radiation may be used before surgery to shrink a sarcoma and make it easier to remove. It may also be used after surgery to kill any cancer cells that remain.
- Intraoperative radiation therapy (IORT). IORT is used during surgery just after the cancer is removed. The radiation is directed to the area around where the cancer used to be. IORT might be recommended if the cancer is located in an area that makes it difficult to remove the cancer completely during surgery.
Chemotherapy
Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. It can be administered by pill or through a vein (intravenously), or both.
Chemotherapy is most often used to treat undifferentiated pleomorphic sarcoma that comes back after initial treatment or that spreads to other areas of the body.
Sometimes chemotherapy is used before surgery to shrink the cancer so that it’s easier to remove during an operation.
Chemotherapy may also be combined with radiation.
Targeted drug therapy
Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die.
For undifferentiated pleomorphic sarcoma, targeted therapy drugs may be combined with chemotherapy.
Some targeted therapies only work in people whose cancer cells have certain genetic mutations. Your cancer cells may be tested in a laboratory to see if these drugs might help you.
Immunotherapy
Immunotherapy uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that help them hide. Immunotherapy works by interfering with that process.
Immunotherapy treatments are generally reserved for people with advanced cancer.
References
- Goldblum JR, et al. Undifferentiated pleomorphic sarcoma. In: Enzinger and Weiss’s Soft Tissue Tumors. 7th ed. Elsevier; 2020. https://www.clinicalkey.com. Accessed Jan. 23, 2020.
- Azar FM, et al. Soft-tissue tumors. In: Campbell’s Operative Orthopaedics. 13th ed. Elsevier; 2017. https://www.clinicalkey.com. Accessed Jan. 23, 2020.
- Soft tissue sarcoma. National Comprehensive Cancer Network. https://www.nccn.org/professionals/physician_gls/default.aspx. Accessed Jan. 23, 2020.
- Ryan CW, et al. Clinical presentation, histopathology, diagnostic evaluation and staging of soft tissue sarcoma. https://www.uptodate.com/contents/search. Accessed Jan. 24, 2020.
- Widemann BC, et al. Biology and management of undifferentiated pleomorphic sarcoma, myxofibrosarcoma and malignant peripheral nerve sheath tumors: State of the art and perspectives. Journal of Clinical Oncology. 2017; doi:10.1200/JCO.2017.75.3467.
- Roeder F, et al. Intraoperative radiation therapy in soft-tissue sarcoma. Radiation Oncology. 2017; doi:10.1186/s13014-016-0751-2.
- Deng GE, et al. Evidence-based clinical practice guidelines for integrative oncology: Complementary therapies and botanicals. Journal of the Society for Integrative Oncology. 2009; doi:10.2310/7200.2009.0019.
- NCI dictionary of cancer terms. National Cancer Institute. https://www.cancer.gov/publications/dictionaries/cancer-terms. Accessed Jan. 24, 2020.
- Warner KJ. Allscripts EPSi. Mayo Clinic. July 18, 2019.
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