Description

Undifferentiated pleomorphic Sarcoma (UPS) is a rare type of cancer that originates mainly in the soft tissues of the body. The soft tissues that connect, support and surround other body structures.

UPS usually occurs in the arms or legs. Less frequently it may occur in the area behind the abdominal organs (retroperitoneum).

The name of undifferentiated pleomorphic sarcoma is the way in which the cancer cells appear under the microscope. Undifferentiated means that the cells do not resemble the tissue of the body in which they develop. The cancer called pleomorphic (plee-o-MOR-fik) because the cells grow in various shapes and sizes.

Treatment for UPS depends on the location of the cancer, but it often involves surgery, radiation and drug treatments.

UPS is called malignant fibrous histiocytoma.

Symptoms

Undifferentiated pleomorphic Sarcoma symptoms depend on where the cancer occurs. This happens most often in the arms and legs, but can happen anywhere in the body.

Signs and symptoms may include:

  • Growing lump or area of inflammation.
  • If it is very large, there may be pain, tingling, and numbness.
  • If it occurs in an arm or a leg, there may be swelling of the hand or foot of the affected limb.
  • If it occurs in the abdomen, there may be pain, loss of appetite and constipation.
  • Fever.
  • The loss of weight.

When to see a doctor

Make an appointment with your doctor if you develop any persistent signs or symptoms that worry you.

Causes

It is not clear what causes the undifferentiated pleomorphic sarcoma.

The doctors know this type of cancer begins when a cell develops changes in your DNA. A cell's DNA contains the instructions that tell a cell what to do. The changes that tell the cell to multiply rapidly, creating a mass of abnormal cells (tumour). The cells can invade and destroy surrounding healthy tissue. At the time, cancer cells can break off and spread (metastasize) to other parts of the body, such as the lungs and bones.

Risk factors

Factors that may increase the risk of undifferentiated pleomorphic sarcoma include:

  • Advanced age. This type of cancer tends to occur in adults over the age of 50 years, although it can occur at any age.
  • Previous radiation therapy. Rarely, this type of cancer can develop in one area of the body that was previously treated with radiation therapy.

The majority of people who develop undifferentiated pleomorphic sarcoma do not have any known risk factors, and many people who have risk factors never develop cancer.

Diagnosis

The diagnosis of undifferentiated pleomorphic sarcoma usually begins with a review of the symptoms and a physical examination. This type of cancer is often diagnosed after other kinds 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 whether they have changed over time. He or she will examine the area to better understand the size and depth of the growth, as it connects to the tissues nearby, and if there are signs of inflammation or nerve damage.
  • Imaging tests. Your doctor may recommend imaging tests to create images of the affected area, and to understand more about your condition. Imaging tests may include x-rays, computed tomography , magnetic resonance imaging, and positron emission tomography (PET).
  • The removal of a sample of tissue for testing (biopsy).To make a definite diagnosis, the doctor will take a sample of tumor tissue and sends it to a laboratory for analysis. Depending on your particular situation, the tissue sample may be collected using a needle that is inserted through the skin or during an operation. In the laboratory, doctors trained in the analysis of the tissues of the body (pathologists) to examine the sample to determine the type of cells affected, and whether the cells are likely to be aggressive. This information helps to rule out other types of cancer, and treatment guidelines.

The removal of a sample of tissue for testing (biopsy). To make a definite diagnosis, the doctor will take a sample of tumor tissue and sends it to a laboratory for analysis. Depending on your particular situation, the tissue sample may be collected using a needle that is inserted through the skin or during an operation.

In the laboratory, doctors trained in the analysis of the tissues of the body (pathologists) to examine the sample to determine the type of cells affected, and whether the cells are likely to be aggressive. This information helps to rule out other types of cancer, and treatment guidelines.

Determine the type of biopsy that is needed, and the details of how it should be carried out requires a lot of careful planning by the team doctor. Physicians need to perform the biopsy in a way that does not interfere with the future of surgery to remove the cancer. For this reason, ask your doctor for a referral to a team of experts with extensive experience in the treatment of soft tissue sarcomas before the biopsy.

Treatment

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. What treatments are best for you will depend on the size and location of the cancer.

Surgery

When possible, doctors try to remove the tumor completely with surgery. The goal is to remove the cancer and a margin of healthy tissue around it with the minimum possible impact.

When the cancer affects the arms and legs, surgeons prefer to use the limb-sparing operations. However, in some cases it may be necessary to amputate the affected arm or leg. Other treatments, such as chemotherapy and radiation therapy, may be recommended before surgery to reduce the size of a cancer, so that it is easier to remove without amputation of the affected limb.

Radiation therapy

Radiation therapy uses high-powered beams of energy, such as X-rays or protons, to kill cancer cells. Radiotherapy 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 radiation to precise points on your body. Radiation may be used before surgery to reduce the size of a tumor and make it easier to remove. It can also be used after surgery to destroy any cancer cells that remain.
  • Intraoperative radiotherapy (IORT). IORT is used during the surgery only after the cancer is removed. The radiation is directed to the area where the cancer used to be. IORT may be recommended if the cancer is located in an area that makes it difficult to completely remove the cancer during surgery.

External-beam radiation. This type of radiation comes from a machine that moves around you as you lie on a table. The machine directs radiation to precise points on your body.

Radiation may be used before surgery to reduce the size of a tumor and make it easier to remove. It can also be used after surgery to destroy any cancer cells that remain.

Chemotherapy

Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. It can be administered in pill form, or through a vein (intravenously), or both.

Chemotherapy is most often used to treat undifferentiated pleomorphic sarcoma that comes back after initial treatment, or spreads to other areas of the body.

Sometimes chemotherapy is used before surgery to reduce the size of the cancer, so that it is easier to remove during an operation.

Chemotherapy can 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 the undifferentiated pleomorphic sarcoma, targeted therapy drugs may be combined with chemotherapy.

Some targeted therapies only work in the people that the cancer cells have certain gene mutations. Cancer cells can be analyzed in a laboratory to see if these medications can help.

Immunotherapy

Immunotherapy uses your immune system to fight cancer. From your body to fight off diseases immune system will not attack the cancer because the cancer cells produce proteins that help to hide. Immunotherapy works by interfering with that process.

Immunotherapy treatments are generally reserved for people with advanced cancer.

Alternative medicine

There are No alternative treatments have been found to be useful in the treatment of undifferentiated pleomorphic sarcoma. But some complementary and alternative treatments can relieve the symptoms you experience due to cancer or treatment for cancer.

Alternative treatments that can help relieve the symptoms include:

  • Acupuncture.
  • Exercise.
  • The massage.
  • Meditation.
  • Music therapy.
  • The relaxation exercises.

Coping and support

A diagnosis of cancer, such as undifferentiated pleomorphic sarcoma can be overwhelming. With the time you're going to find ways to cope with the distress and uncertainty of cancer. Until then, you can find help for:

  • Learn enough about sarcoma 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 undifferentiated pleomorphic sarcoma, may be more confident in making treatment decisions.
  • Keep friends and family close. Keep your close relationships strong will help you deal with the diagnosis and care of its implications. Friends and family can provide the practical support you need, such as help to take care of your house if you're in the hospital. And that you can serve as emotional support when you feel overwhelmed by the cancer.
  • Find someone to talk to.Find a good listener who is willing to listen to you talk about your hopes and fears. This can be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group may also be useful. Ask your doctor about support groups in your area. Or look in your telephone directory, or the library of a cancer of the organization, such as the National Cancer Institute or the American Cancer Society.

Find someone to talk to. Find a good listener who is willing to listen to you talk about your hopes and fears. This can be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group may also be useful.

Ask your doctor about support groups in your area. Or look in your telephone directory, or the library of a cancer of the organization, such as the National Cancer Institute or the American Cancer Society.

Preparing for your appointment

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

Because appointments can be brief, and there is often a lot of information to discuss, it is a good idea to get well prepared. Here's some information to help you prepare.

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 you are 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 questions to ask their doctor.

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

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

What to expect from your doctor

Your doctor may ask you a series of questions. Be prepared to respond to them can make the time to cover other points you want to discuss. Your doctor may ask:

  • When did you first notice the signs and symptoms?
  • Are you experiencing pain?
  • Nothing seems to improve the symptoms?
  • What, if anything, appears to worsen your symptoms?

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

Symptoms and treatment of Undifferentiated pleomorphic sarcoma