Undifferentiated pleomorphic sarcoma
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Undifferentiated pleomorphic sarcoma (UPS) is a rare form of cancer that primarily originates in the soft tissues of the body. These soft tissues encompass various types, such as blood vessels, fat, fibrous tissues (including tendons, ligaments, and cartilage), muscles, nerves, and skin. Although UPS typically affects soft tissues, it can also manifest in bones, behaving similarly to other more common bone–originating sarcomas like osteosarcoma. The most common locations for UPS are the arms and legs, while less frequently, it may occur in the retroperitoneum (the area behind the abdominal organs).
The name “undifferentiated pleomorphic sarcoma” derives from the appearance of cancer cells when observed under a microscope. “Undifferentiated” refers to the cells lacking resemblance to the body tissues in which they develop, while “pleomorphic” indicates that the cells exhibit multiple shapes and sizes.
Treatment for UPS depends on the cancer’s location, but it typically involves a combination of surgery, radiation, and drug therapies. It’s important to note that UPS was previously known as malignant fibrous histiocytoma.
Symptoms
The symptoms of undifferentiated pleomorphic sarcoma vary depending on the location of the cancer within the body. While it most commonly develops in the arms and legs, it can potentially occur anywhere.
Typical signs and symptoms may include:
- Development of a growing lump or an area of swelling.
- In case the lump grows large, it may lead to pain, tingling, and numbness.
- If the sarcoma appears in an arm or leg, there could be swelling in the hand or foot of the affected limb.
- When the cancer occurs in the abdomen, symptoms might include pain, loss of appetite, and constipation.
- Fever.
- Unintentional weight loss.
If you experience any persistent signs or symptoms that cause concern, it is important to schedule an appointment with a doctor.
Causes
The exact cause of undifferentiated pleomorphic sarcoma remains unclear. However, it is understood that the cancer originates from a cell that undergoes changes in its DNA. The DNA in a cell contains instructions that regulate its functions. When these changes occur, the affected cells start to multiply rapidly, leading to the formation of an abnormal mass of cells, referred to as a tumor. These cells have the ability to invade and damage nearby healthy tissues. Over time, the cancerous cells may also detach from the original tumor and spread to other areas of the body through a process called metastasis. Common sites of metastasis for this type of cancer include the lungs and bones.
Risk factors
Factors that may increase the risk of undifferentiated pleomorphic sarcoma include:
- Advanced age: This cancer is more frequently observed in individuals over the age of 50, although it can occur at any age.
- History of radiation therapy: In rare instances, undifferentiated pleomorphic sarcoma may develop in areas of the body that have previously undergone radiation treatment.
Diagnosis
As part of the diagnostic process, healthcare providers perform a comprehensive physical examination and delve into the patient’s medical history, gathering information about the onset and progression of symptoms. This involves a careful assessment of the affected area, considering its size, depth, and its relationship with surrounding tissues, while also looking for signs of swelling or nerve injury. To determine the cancer stage, the healthcare provider evaluates the tumor’s size, cell types involved, and the extent of its spread. This staging process helps guide treatment recommendations and provides valuable insights into the patient’s prognosis.
Other tests are necessary to confirm the diagnosis, such as:
- Imaging tests: Radiology tests, such as magnetic resonance imaging (MRI) for the legs and arms, or computed tomography (CT) scans for the abdominal areas and lungs, are often used to detect tumor size and involvement of blood vessels and nerves. Positron emission tomography (PET) scans are used in some cases. Imaging tests are requested to create images of the affected area and provide information about the condition.
- Biopsy: To make a definitive diagnosis, your doctor will collect a sample of the tumor tissue, either through a needle inserted through the skin or during a surgical procedure, depending on your specific case. This tissue sample is then sent to a specialized lab where skilled pathologists analyze it to identify the types of cells present and assess their aggressiveness. This process helps eliminate the possibility of other types of cancer and provides crucial guidance for your treatment plan.
Treatment
The optimal treatment approach for undifferentiated pleomorphic sarcoma is typically determined based on factors such as the size and location of the cancer. Common treatment options for this type of sarcoma include surgery, radiation therapy, chemotherapy, targeted therapy, and immunotherapy. The choice of treatment modalities will be carefully considered based on individual patient characteristics and the specific characteristics of the tumor.
- Surgery: The standard therapy for UPS is surgical excision of the entire tumor with a cuff of “normal tissue,” referred to as a margin, surrounding it. The goal is to remove the malignancy with as little harm as possible. Other therapies, such as radiation therapy and chemotherapy, may be suggested prior to surgery to reduce the tumor’s size, particularly those on the limb.
To assist patients, keep function in both legs and arms, healthcare experts undertake limb–saving surgery as often as possible. In rare cases, the damaged arm or leg may need to be amputated.
- Radiation therapy: In addition to surgery, healthcare experts frequently recommend radiation therapy. This potent combination gives the best chance of preventing the sarcoma from returning to the same location. Radiation may be required either before or after surgery. Radiation therapy kills cancer cells by delivering high–energy beams such as X–rays or protons.
Radiation therapy can be administered as follows:
- External beam radiation: Before surgery, this may be used to shrink a sarcoma and make it easier to remove. It may also be administered after surgery to destroy any remaining cancer cells. In this treatment, radiation is directed to certain locations in the body. The patient lies on a table and the machine producing the radiation moves around him or her.
- Intraoperative radiation therapy (IORT): IORT is often advised if the cancer is in a region where total removal during surgery is difficult. The radiation is focused on the area where the cancer used to be.
- Chemotherapy: Chemotherapy may be used in cases where the cancer is more advanced, has spread to many areas in the body, or has recur after first treatment.
Chemotherapy is sometimes administered before surgery to shrink the cancer so that it may be removed more easily during the operation. It can also be done in conjunction with radiation therapy. This treatment uses chemicals, taken as a pill, injected into a vein, or both, to kill cancer cells.
- Targeted drug therapy: Eligibility for this treatment is usually known during laboratory analysis of cancer cells. Some targeted therapy medications are only effective in patients whose cancer cells contain specific genetic abnormalities.
These cancer cells can be killed by specific medication treatments that prevent these abnormalities. It may be coupled with chemotherapy for treatment of undifferentiated pleomorphic sarcoma.
- Immunotherapy: Immunotherapy is often intended for patients with advanced cancer. Since cancer cells release proteins that blind immune system cells, the body’s disease–fighting immune system may fail to battle cancer. Immunotherapy works by disrupting that process. Immunotherapy is a cancer–fighting medicine that boosts the immune system.
