Overview
Undifferentiated pleomorphic sarcoma (UPS) is a rare type of cancer that typically begins in the soft tissues of the body, such as the arms or legs. The cancer cells appear abnormal under the microscope, as they are undifferentiated and pleomorphic (having multiple shapes and sizes). UPS treatment often involves surgery, radiation, and drug therapy, and the cancer was previously known as malignant fibrous histiocytoma. Symptoms may include a growing lump or swelling, pain, tingling, numbness, fever, weight loss, and loss of appetite, among others, depending on the location of the tumor. The exact cause of UPS is not clear, but it is believed to result from changes in a cell's DNA that cause it to multiply rapidly, leading to the formation of a tumor that can invade and destroy healthy tissue. Risk factors for UPS include older age and previous radiation therapy. If you develop persistent symptoms that worry you, it is important to consult with a doctor.
Understanding the disease
Symptoms
The symptoms of undifferentiated pleomorphic sarcoma (UPS) depend on the location of the cancer. Common symptoms include:
- Growing lump or area of swelling
- Pain, tingling, and numbness (if the cancer grows very large)
- Swelling in the hand or foot of an affected limb (if it occurs in the arms or legs)
- Pain, loss of appetite, and constipation (if it occurs in the abdomen)
- Fever
- Weight loss If you experience any persistent signs or symptoms that worry you, it's important to make an appointment with a doctor.
Risk Factors
Risk factors for developing undifferentiated pleomorphic sarcoma may include:
- Older age: This cancer tends to occur in adults older than 50, though it can occur at any age.
- Previous radiation therapy: Rarely, undifferentiated pleomorphic sarcoma can develop in an area of the body that was previously treated with radiation therapy. Please note that many people who have risk factors never develop the cancer, and some people who develop the cancer have no known risk factors.
Development
Undifferentiated pleomorphic sarcoma (UPS) develops when a cell in the soft tissues of the body undergoes genetic changes in its DNA. These changes instruct the cell to multiply rapidly, resulting in a mass of abnormal cells, or tumor. The cancer cells can invade and destroy nearby healthy tissue, and over time, they may spread (metastasize) to other parts of the body. The type of soft tissue sarcoma is determined by the type of cell with DNA changes. In the case of UPS, the cancer cells are pleomorphic, meaning they grow in multiple shapes and sizes. UPS usually occurs in the arms or legs, but can also develop in the area behind the abdominal organs.
Assessment and Diagnosis
Triage
You should see a doctor if you develop any persistent signs or symptoms that worry you, including the presence of a growing lump or area of swelling, pain, tingling, and numbness, swelling in the hand or foot of an affected limb, pain, loss of appetite, and constipation.
Diagnosis
The diagnosis of undifferentiated pleomorphic sarcoma usually begins with a review of symptoms and a physical examination. Imaging tests such as X-rays, CT scans, MRI scans, and positron emission tomography (PET) scans may be recommended to create pictures of the affected area and understand more about the condition. A biopsy, which involves removing a sample of cells for testing, is also typically performed to confirm the diagnosis. It's important to seek care at a medical center with experience in treating sarcomas, as the biopsy procedure should be done in a way that won't cause problems with future surgery.
Management and Treatment
Home Remedies
It is important to consult with a healthcare professional for proper diagnosis and treatment of undifferentiated pleomorphic sarcoma.
Treatment
Treatment for undifferentiated pleomorphic sarcoma (UPS) usually involves a combination of surgery, radiation, and drug therapies. These treatments depend on the location and extent of the cancer. Surgery is often the primary treatment, followed by radiation therapy and/or chemotherapy to remove any remaining cancer cells. In some cases, targeted therapies or immunotherapy may also be used. The exact treatment plan will be determined by a medical team specializing in sarcomas, taking into account the patient's age, overall health, and specific cancer characteristics.
Preparing for medical consultation
To prepare for an appointment for undifferentiated pleomorphic sarcoma, you should:
- Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for the appointment.
- Make a list of all medications, vitamins, or supplements that you're taking.
- Bring a family member or friend with you to help remember the information discussed during the appointment.
- Prepare a list of questions to ask your doctor, prioritizing them based on importance.
- Request any necessary medical records or imaging tests to be sent to the doctor before the appointment.
- Research undifferentiated pleomorphic sarcoma and understand its diagnosis, treatment options, and prognosis.
- Consider seeking support from a counselor, medical social worker, clergy member, or cancer support group.
- Inquire about support groups in your area or check resources such as the National Cancer Institute or the American Cancer Society.
- If possible, seek a referral to a cancer doctor (oncologist) who specializes in sarcomas, particularly one with experience in treating undifferentiated pleomorphic sarcoma. Remember to be open and honest with your doctor about your concerns and any questions you may have. It's essential to work together to understand your condition and create a personalized treatment plan.