Undifferentiated pleomorphic sarcoma
Overview
Undifferentiated pleomorphic sarcoma (UPS) is
a rare type of cancer that begins mostly in the soft tissues of the body. Soft
tissues connect, support and surround other body structures.
UPS 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. The cancer is called pleomorphic (plee-o-MOR-fik) because the cells
grow in multiple shapes and sizes.
Treatment for UPS depends on the
location of the cancer, but often involves surgery, radiation and drug treatments.
UPS used to be called malignant fibrous
histiocytoma.
Symptoms
Undifferentiated pleomorphic sarcoma symptoms
depend on where the cancer occurs. It most often happens in the arms and legs,
but it can happen anywhere in the body.
Signs and symptoms may include:
·
Growing lump or area
of swelling
·
If it grows very
large, there may be 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 pain, loss of appetite and constipation
·
Fever
·
Weight loss
When to see a doctor
Make an appointment with a doctor if you
develop any persistent signs or symptoms that worry you.
Causes
It's not clear what causes undifferentiated
pleomorphic sarcoma.
Doctors know this cancer begins when a cell
develops changes in its DNA. A cell's DNA contains the instructions that tell a
cell what to do. The changes tell the cell to multiply rapidly, creating a mass
of abnormal cells (tumor). The cells can invade and destroy nearby healthy
tissue. In time, the cancer cells can break away 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:
·
Older
age. This cancer
tends to occur in adults older than 50, though 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, and many people who have risk
factors never develop the cancer.
Diagnosis
Diagnosis for undifferentiated pleomorphic
sarcoma usually starts with a review of your symptoms and a physical
examination. This cancer is often diagnosed after other types 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 if they've changed over
time. He or she will examine the area to better understand the size and depth
of the growth, whether it's connected to nearby tissues, and whether there are
any signs of swelling or nerve damage.
·
Imaging
tests. Your doctor may
recommend imaging tests to create pictures of the affected area and understand
more about your condition. Imaging tests may include X-rays, CT, MRI and
positron emission tomography (PET) scans.
·
Removing
a sample of tissue for testing (biopsy). To make a definitive diagnosis, your doctor collects a
sample of the tumor tissue and sends it to a lab for testing. Depending on your
particular situation, the tissue sample may be collected with a needle inserted
through your skin or during an operation.
In
the lab, doctors trained in analyzing body tissues (pathologists) examine the
sample to determine the types of cells involved and whether the cells are
likely to be aggressive. This information helps rule out other types of cancer
and guides your treatment.
Determining the type of biopsy needed and the
specifics of how it should be performed requires careful planning by the
medical team. Doctors need to perform the biopsy in a way that won't interfere
with future surgery to remove the cancer. For this reason, ask your doctor for
a referral to a team of experts with extensive experience in treating 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. Which treatments are best for you will
depend on the size and location of your cancer.
Surgery
When possible, doctors try to remove the
sarcoma completely with surgery. The goal is to remove the cancer and a margin
of healthy tissue around it with as minimal an impact as possible.
When the cancer affects the arms and legs,
surgeons prefer to use limb-sparing operations. However, in some cases it may
be necessary to amputate the affected arm or leg. Other treatments, such as
radiation therapy and chemotherapy, might be recommended before surgery to
shrink a cancer so that it's easier to remove 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. Radiation therapy 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 the radiation to precise points on your body.
Radiation
may be used before surgery to shrink a sarcoma and make it easier to remove. It
may also be used after surgery to kill any cancer cells that remain.
·
Intraoperative
radiation therapy (IORT). IORT is
used during surgery just after the cancer is removed. The radiation is directed
to the area around where the cancer used to be. IORT might be
recommended if the cancer is located in an area that makes it difficult to
remove the cancer completely during surgery.
Chemotherapy
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.
Chemotherapy is most often used to treat
undifferentiated pleomorphic sarcoma that comes back after initial treatment or
that spreads to other areas of the body.
Sometimes chemotherapy is used before surgery
to shrink the cancer so that it's easier to remove during an operation.
Chemotherapy may 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 undifferentiated pleomorphic sarcoma,
targeted therapy drugs may be combined with chemotherapy.
Some targeted therapies only work in people
whose cancer cells have certain genetic mutations. Your cancer cells may be
tested in a laboratory to see if these drugs might help you.
Immunotherapy
Immunotherapy uses your immune system to fight
cancer. Your body's disease-fighting immune system may not attack your cancer
because the cancer cells produce proteins that help them hide. Immunotherapy
works by interfering with that process.
Immunotherapy treatments are generally
reserved for people with advanced cancer.
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
·
Exercise
·
Massage
·
Meditation
·
Music therapy
·
Relaxation exercises
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 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, 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 diagnosis and care
implications. Friends and family can provide the practical support you'll need,
such as helping take care of your home 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 information to discuss, 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.
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