A doctor converses with a woman about undifferentiated pleomorphic sarcoma Consultation

A Mayo Clinic doctor converses with a woman about a possible undifferentiated pleomorphic sarcoma diagnosis.

Initial evaluation for a suspected undifferentiated pleomorphic sarcoma usually starts with a history of when you first noticed the mass and how quickly it's been growing, as well as a physical examination. Your physical exam should focus on the current size and depth of the mass, whether it's connected to nearby normal tissues, and any signs of associated swelling or nerve damage.

Your doctors will want to check whether your mass is one of several much more common benign conditions, such as soft tissue tumors (for example, lipomas). Other malignant tumors should also be ruled out, including lymphoma, melanoma, carcinoma that has metastasized, or one of several other sarcomas.

Because benign soft tissue tumors are so much more common than undifferentiated pleomorphic sarcoma or other malignant soft tissue sarcomas, it can be challenging to assess which soft tissue masses call for further evaluation. Published criteria for urgent referral of a patient with a soft tissue lesion focus on three aspects:

  • A soft tissue mass, golf ball-sized or larger
  • A painful lump
  • A lump that's increasing in size

Also, deeper lying masses are more likely to be sarcomas, as are lumps that redevelop after being surgically removed. The more of these clinical features exist, the greater the chance of malignancy. Increasing size is the strongest individual indicator.

  • Undifferentiated pleomorphic sarcoma is best treated with a multidisciplinary approach that involves medical oncologists, pathologists, radiologists, surgeons, radiation therapists and others.
  • Early referral to such a multidisciplinary center — one that sees and treats a high number of such cases annually — is associated with better outcomes.
  • Even the initial biopsy should be done at the same treatment center, as well as any resection surgery.

In almost all instances your doctor will likely recommend you undergo diagnostic tests, including:

  • Imaging tests. Imaging tests, such as X-rays, computerized tomography (CT) scans, magnetic resonance imaging (MRI) and positron emission tomography (PET), may be used to evaluate the area of concern. The evaluation itself may include defining the source of a tissue mass, determining the extent of a primary tumor for surgical planning, and tests to indicate whether any malignant growth has metastasized to additional locations such as a lung.
  • Tissue sample removal and testing (biopsy). Your doctor may perform a biopsy procedure to remove a sample of the suspected sarcoma for testing in a lab. To remove the sample, your doctor may use a long, thin needle. Sometimes a biopsy sample is removed during surgery.

Again, if undifferentiated pleomorphic sarcoma is suspected, it's often best to seek care at a medical center that sees many people with this type of cancer. Experienced doctors will select the best biopsy technique to ensure proper surgical treatment and planning.

In the lab, a doctor trained in analyzing body tissues (pathologist) examines the tissue sample for signs of cancer. The pathologist also analyzes the sample to understand the type of cancer and to determine whether the cancer is aggressive.


Treatment options for undifferentiated pleomorphic sarcoma will depend on the size and location of your tumor.


Surgery is typically the primary treatment for UPS. The goal is to remove the cancer and a margin of healthy tissue around it with as minimal an impact as possible. When UPS affects the arms and legs, this means using limb-sparing techniques whenever feasible.

However, in some cases it may be necessary to amputate the affected arm or leg. Doctors work very hard to avoid amputation. For example, they may recommend radiation therapy to shrink the tumor before surgery, which can decrease the chances that an amputation will be needed.

If undifferentiated pleomorphic sarcoma spreads to another area of the body, it may be possible to surgically remove the other tumor, subject to its particular medical needs.

Radiation therapy

Radiation therapy involves treating cancer with high-powered beams of energy, such as X-rays or protons.

  • Your doctor may suggest using radiation therapy before an operation in order to shrink a tumor to make it easier to remove.
  • Radiation may also be used after surgery to kill any cancer cells that remain.
  • Radiation may also be employed during surgery just after a resection, a procedure called intraoperative radiation therapy (IORT).


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. While it's generally not used in early-stage UPS, chemotherapy may be used to treat tumors that have spread to other parts of the body or to treat more-advanced disease.

Chemotherapy may also be combined with radiation.

Targeted chemotherapy drugs used to treat undifferentiated pleomorphic sarcoma include:

  • Doxorubicin (Doxil) with olaratumab (Lartruvo)
  • Gemcitabine (Gemzar) with docetaxel (Taxotere)

These medications and others may be prescribed at the discretion of your doctor.

Clinical trials

Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this disease.

Alternative medicine

No alternative treatments have been found helpful in treating undifferentiated pleomorphic sarcoma. But some complementary and alternative treatments may relieve the symptoms you experience due to cancer or cancer treatment.

Alternative treatments that may help relieve symptoms include:

  • Acupuncture
  • Aromatherapy
  • Massage
  • Meditation
  • Relaxation exercises

Resources such as the American Cancer Society and the National Cancer Institute offer more information on complementary and alternative treatment options as well as further advice on symptom relief.

Coping and support

A diagnosis of cancer such as undifferentiated pleomorphic sarcoma can be overwhelming. With time you'll find ways to cope with the distress and uncertainty of cancer. Until then, you may find it helps to:

  • Learn enough about UPS to make decisions about your care. Ask your doctor about your sarcoma, including your treatment options and, if you like, your prognosis. As you learn more about UPS, you may become more confident in making treatment decisions.
  • Keep friends and family close. Keeping your close relationships strong will help you deal with your UPS diagnosis and care implications. Friends and family can provide the practical support you'll need, such as helping take care of your house if you're in the hospital. And they can serve as emotional support when you feel overwhelmed by cancer.
  • Find someone to talk with. Find a good listener who is willing to listen to you talk about your hopes and fears. This may be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful.

    Ask your doctor about support groups in your area. Or check your phone book, library or a cancer organization, such as the National Cancer Institute or the American Cancer Society.

Preparing for your appointment

If your family doctor suspects you have undifferentiated pleomorphic sarcoma, you'll likely be referred to a cancer doctor (oncologist) who specializes in sarcomas. Undifferentiated pleomorphic sarcoma is rare and often requires complex care. It's best treated by someone who has significant experience with it, which often means an academic or multispecialized cancer center.

Because appointments can be brief, and there's often a lot of ground to cover, it's a good idea to arrive well-prepared. Here's some information to help you get ready.

What you can do

  • Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
  • Make a list of all medications, vitamins or supplements that you're taking.
  • Ask a family member or friend to come with you. Sometimes it can be difficult to remember all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For undifferentiated pleomorphic sarcoma, some basic questions to ask your doctor include:

  • Do I have cancer?
  • Are there other possible causes for my symptoms?
  • What kinds of tests do I need to confirm the diagnosis? Do these tests require any special preparation?
  • What stage is the sarcoma?
  • What treatments are available for undifferentiated pleomorphic sarcoma, and which do you recommend?
  • Can the sarcoma be removed?
  • What types of side effects can I expect from treatment?
  • Are there any alternatives to the primary approach that you're suggesting?
  • I have other health conditions. How can I best manage these conditions together?
  • Are there any dietary or activity restrictions that I need to follow?
  • What's my prognosis?
  • Are there any brochures or other printed material that I can take with me? What websites do you recommend?
  • Should I get additional treatments such as radiation therapy either before or after an operation?
  • Is the surgeon you're recommending experienced in this specific type of cancer operation?

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may make time to cover other points you want to discuss. Your doctor may ask:

  • When did you first notice your signs and symptoms?
  • Are you experiencing pain?
  • Does anything seem to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions.

Undifferentiated pleomorphic sarcoma care at Mayo Clinic

Aug. 08, 2018
  1. Ryan CW, et al. Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma. https://www.uptodate.com/contents/search. Accessed Aug. 21, 2017.
  2. George S, et al. Systemic treatment of metastatic soft tissue sarcoma. https://www.uptodate.com/contents/search. Accessed Aug. 21, 2017.
  3. Mullen JT, et al. Clinical features, evaluation, and treatment of retroperitoneal soft tissue sarcoma. https://www.uptodate.com/contents/search. Accessed Aug. 21, 2017.
  4. Attia S (expert opinion). Mayo Clinic, Rochester, Minn. Sept. 12, 2017.
  5. DeLaney TF, et al. Overview of multimodality treatment for primary soft tissue sarcoma of the extremities and chest wall. https://www.uptodate.com/contents/search. Accessed Aug. 21, 2017.
  6. About soft tissue sarcoma. American Cancer Society. https://www.cancer.org. Accessed Aug. 31, 2017.
  7. Fletcher CDM, et al., eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon, France: IARC Press; 2002. http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb5/index.php. Accessed Aug. 31, 2017.
  8. Early detection, diagnosis, and staging. American Cancer Society. https://www.cancer.org. Accessed Aug. 31, 2017.
  9. Causes, risk factors, and prevention. American Cancer Society. https://www.cancer.org. Accessed Sept. 1, 2017.
  10. Treating soft tissue sarcoma. American Cancer Society. https://www.cancer.org. Accessed Sept. 1, 2017.
  11. Garner HW, et al. Preoperative radioactive seed localization of nonpalpable soft tissue masses: an established localization technique with a new application. Skeletal Radiology. 2017;46:209.
  12. Tap W, et al. Randomized phase 3, multicenter, open-label study comparing evofosfamide (Evo) in combination with doxorubicin (D) vs. D alone in patients (pts) with advanced soft tissue sarcoma (STS): Study TH-CR-406/SARC021. Annals of Oncology. 2016;27(suppl 6):13950.
  13. Attia S, et al. A phase Ib dose-escalation study of TRC105 (anti-endoglin antibody) in combination with pazopanib in patients with advanced soft tissue sarcoma (STS). Journal of Clinical Oncology. 2015;33(suppl 15):10514.
  14. Burgess MA, et al. SARC 028: A phase II study of the anti-PD1 antibody pembrolizumab (P) in patients (Pts) with advanced sarcomas. Journal of Clinical Oncology. 2015;33(suppl 15):10578.
  15. Riggin EA. Allscripts EPSi. Mayo Clinic, Rochester, Minn. June 6, 2017.
  16. Goldblum JR, et al. Clinical evaluation and treatment of soft tissue tumors. In: Enzinger and Weiss's Soft Tissue Tumors. 6th ed. Philadelphia, Pa.: Saunders Elsevier; 2014. https://www.clinicalkey.com. Accessed Sept. 1, 2017.
  17. Dangoor A. et al. UK guidelines for the management of soft tissue sarcomas. Clinical Sarcoma Research. 2016;6:20.
  18. Soft tissue sarcoma. Fort Washington, Pa.: National Comprehensive Cancer Network. http://www.nccn.org/professionals/physician_gls/f_guidelines.asp. Accessed March Sept. 7, 2017.
  19. Derbel O, et al. Survival impact of centralization and clinical guidelines for soft tissue sarcoma (A prospective and exhaustive population based cohort). PLOS One. 2017;12:e0158406.http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0158406. Accessed Sept. 7, 2017.
  20. Townsend CM Jr, et al. Soft tissue sarcoma. In: Sabiston Textbook of Surgery: The Biological Basis of Modern Surgical Practice. 20th ed. Philadelphia, Pa.: Elsevier; 2017. https://www.clinicalkey.com. Accessed Sept. 8, 2017.
  21. Goldman L, et al., eds. Malignant tumors of bone, sarcomas, and other soft tissue neoplasms. In: Goldman-Cecil Medicine. 25th ed. Philadelphia, Pa.: Saunders Elsevier; 2016. https://www.clinicalkey.com. Accessed Sept. 8, 2017.
  22. Adult soft tissue sarcoma treatment (PDQ) — Health professional version. National Cancer Institute. https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq. Accessed Sept. 8, 2017.
  23. Taking time: Support for people with cancer. National Cancer Institute. http://cancer.gov/cancertopics/takingtime. Accessed Sept. 8, 2017.
  24. Niederhuber JE, et al., eds. Sarcomas of soft tissue. In: Abeloff's Clinical Oncology. 5th ed. Philadelphia, Pa.: Churchill Livingstone Elsevier; 2014. https://www.clinicalkey.com. Accessed Sept. 15, 2017.