Undifferentiated Pleomorphic Sarcoma

What Is an Undifferentiated Pleomorphic Sarcoma?

Undifferentiated Pleomorphic Sarcoma (also known as Malignant Fibrous Histiocytoma) is a rare type of cancer that begins in the soft tissues of the body.

It 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.

Who Do Undifferentiated Pleomorphic Sarcomas Affect?

• Older age. This cancer is more common in persons over the age of 50, although 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.

What Causes an Undifferentiated Pleomorphic Sarcoma?

The cause of undifferentiated pleomorphic sarcoma is unknown.

Doctors understand that cancer starts when a cell's DNA changes. The DNA of a cell includes the instructions that tell it what to do. The changes cause the cell to grow rapidly, resulting in a swarm of abnormal cells (tumour).

The cells have the ability to penetrate and destroy healthy tissue nearby. Cancer cells can break away and spread to other regions of the body, such as the lungs and bones, over time.

Symptoms of Undifferentiated Pleomorphic Sarcoma

The symptoms of undifferentiated pleomorphic sarcoma vary depending on where the tumour is found. It most commonly affects the arms and legs, but it can affect any part of the body.

The following are possible signs and symptoms:

• A lump or area of swelling
• If it grows very large, there may be a 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 a pain, loss of appetite and constipation
• Weight loss

How is Undifferentiated Pleomorphic Sarcoma Diagnosed

A review of your symptoms and a physical examination are usually the first steps in diagnosing Undifferentiated Pleomorphic Sarcoma. The following tests and procedures may be performed:

Physical Examination

Your doctor will inquire about the start of your symptoms and whether they have changed over time. He or she will check the location to determine the size and depth of the growth, whether it is related to nearby tissues, and whether any swelling or nerve damage is present.

Imaging Tests

Imaging tests may be recommended by your doctor to create images of the affected area and learn more about your condition. CT scans, MRI scans, and PET scans are all examples of imaging examinations.

Performing a biopsy 

Your doctor takes a sample of tumour tissue and sends it to a lab for testing in order to get a clear diagnosis. The tissue sample may be taken with a needle put through your skin or during an operation, depending on your situation.

Surgical Treatment for Undifferentiated Pleomorphic Sarcoma

Surgery

Doctors try to surgically remove the sarcoma completely whenever possible. The goal is to remove the tumour as well as a margin of healthy tissue around it with the least amount of damage possible.

Surgeons prefer to use limb-sparing procedures when cancer affects the arms and legs. However, in some circumstances, the affected arm or leg may need to be removed. Before surgery, various therapies such as radiation therapy and chemotherapy may be used to enable surgical removal of the tumour 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. The use of radiotherapy for these tumours is common. Radiotherapy (either before or after surgery) is useful in the management of sarcomas. It has been shown to reduce the chance of the tumour re-occurring locally after surgical resection.

Chemotherapy

Chemotherapy is a medicinal treatment that kills cancer cells by using chemicals. It can be taken as a pill, injected into a vein (intravenously), or both.

Chemotherapy is sometimes given before surgery to shrink cancer and make it easier to remove during surgery. Chemotherapy is also used after surgery to improve cancer survival.

Chemotherapy and radiation can be used together.

Targeted Drug Therapy

Targeted chemotherapy treatments are designed to target specific abnormalities found in cancer cells. Targeted medication treatments can kill cancer cells by blocking these abnormalities.

Some targeted medicines are only effective in patients with cancer cells that contain specific genetic abnormalities. These genetic abnormalities can be identified by sending a sample of the tumour for genetic testing.

Immunotherapy

Immunotherapy is a cancer treatment that makes use of your immune system. Because cancer cells produce proteins that help them hide, your body's disease-fighting immune system may not attack your cancer. Immunotherapy works by interfering with the immune system's natural processes.

Immunotherapy is often reserved for patients with advanced cancer.