Desmoid
tumors
Overview
Desmoid tumors are noncancerous growths that
occur in the connective tissue. Desmoid tumors most often occur in the abdomen,
arms and legs.
Another term for desmoid tumors is aggressive
fibromatosis.
Some desmoid tumors are slow growing and don't
require immediate treatment. Others grow quickly and are treated with surgery,
radiation therapy, chemotherapy or other drugs.
Desmoid tumors aren't considered cancers
because they don't spread to other areas of the body. But they can be very
aggressive, acting more like cancers and growing into nearby structures and
organs. For this reason, people with desmoid tumors are often cared for by
cancer doctors.
Symptoms
Desmoid tumor symptoms differ based on where
the tumors occur. Desmoid tumors most often happen in the abdomen, arms and
legs. But they can form anywhere in the body.
In general, signs and symptoms include:
·
A mass or area of
swelling
·
Pain
·
Loss of function in
the affected area
·
Cramping and nausea,
when desmoid tumors occur in the abdomen
When to see a doctor
Make an appointment with your doctor if you
have any persistent signs or symptoms that worry you.
Causes
It's not clear what causes desmoid tumors.
Doctors know these tumors form when a
connective tissue cell develops changes in its DNA. A cell's DNA contains the
instructions that tell a cell what to do. The changes tell the connective
tissue cell to multiply rapidly, creating a mass of cells (tumor) that can
invade and destroy healthy body tissue.
Risk factors
Factors that may increase the risk of desmoid
tumors include:
·
Young
adult age. Desmoid tumors
tend to occur in younger adults in their 20s and 30s. This tumor is rare in
children and older people.
·
A
genetic syndrome that causes many colon polyps. People with familial adenomatous
polyposis (FAP) have an increased risk of desmoid tumors. FAP is
caused by a gene mutation that can be passed down from parents to children. It
causes numerous growths (polyps) in the colon.
·
Pregnancy. Rarely, a desmoid tumor may develop
during or soon after pregnancy.
·
Injury. A small number of desmoid tumors develop
in people who've recently had an injury or surgery.
Diagnosis
Tests and procedures used to diagnose desmoid
tumors include:
·
Physical
exam. Your doctor will
examine your body to better understand your signs and symptoms.
·
Imaging
tests. Your doctor may
recommend imaging tests, such as CT and MRI, to create pictures of the area
where your symptoms are occurring. The images may give your doctor clues about
your diagnosis.
·
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. For desmoid
tumors, the sample can be collected with a needle or with surgery, depending on
your particular situation.
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 guide
your treatment.
Treatment
Treatments for desmoid tumors include:
·
Monitoring
the growth of the tumor. If
your desmoid tumor causes no signs or symptoms, your doctor may recommend
monitoring the tumor to see if it grows. You may undergo imaging tests every
few months. Some tumors never grow and may never require treatment. Some tumors
may shrink on their own without any treatment.
·
Surgery. If your desmoid tumor causes signs and
symptoms, your doctor may recommend an operation to remove the entire tumor and
a small margin of healthy tissue that surrounds it. But sometimes the tumor
grows to involve nearby structures and can't be completely removed. In these
cases, surgeons may remove as much of the tumor as possible.
·
Radiation
therapy. Radiation
therapy uses high-powered beams, such as X-rays and protons, to kill tumor
cells. Radiation therapy may be recommended instead of surgery if you're not
healthy enough for surgery or if the tumor is located in a place that makes
surgery risky. Radiation therapy is sometimes used after surgery if there's a
risk that the tumor might return.
·
Chemotherapy
and other medications. Chemotherapy
uses strong drugs to kill tumor cells. Your doctor may recommend chemotherapy
if your desmoid tumor is growing quickly and surgery isn't an option.
Several other drug treatments have shown promise in people with
desmoid tumors, including anti-inflammatory drugs, hormone therapies and
targeted therapies.
Coping and support
With time, you'll find what helps you cope
with the uncertainty and distress of being diagnosed with a rare tumor. Until
then, you may find that it helps to:
·
Learn
enough about desmoid tumors to make decisions about your care. Ask your doctor about your condition,
including your test results, treatment options and, if you like, your prognosis.
As you learn more about desmoid tumors, 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. 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.
·
Find
someone to talk with. Find a good
listener who is willing to hear 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 support group also may be helpful.
Preparing for your
appointment
Make an appointment with your doctor if you
have any signs or symptoms that worry you. If your doctor suspects that you
might have desmoid tumor, he or she may refer you to a doctor who specializes
in treating cancer (oncologist).
Here's some information to help you get ready
for your appointment.
What you can do
When you make the appointment, ask if there's
anything you need to do in advance, such as fasting before having a specific
test.
Gather the medical records that pertain to
your condition and bring them to your appointment. If you're seeing a new
doctor, ask your previous doctor to forward files and other information, such
as glass slides that contain tissue samples, to your new doctor.
Make a list of:
·
Your
symptoms, including any
that seem unrelated to the reason for your appointment
·
Key
personal information, including major
stresses, recent life changes and family medical history
·
All
medications, vitamins or other supplements you take, including the doses
·
Questions
to ask your doctor
Consider bringing a family member or friend to
help you remember the information you're given.
For desmoid tumors, some basic questions to
ask your doctor include:
·
What's likely causing
my symptoms?
·
Other than the most
likely cause, what are other possible causes for my symptoms?
·
What tests do I need?
·
What's the best course
of action?
·
What are the
alternatives to the primary approach you're suggesting?
·
What will happen if I
don't have surgery or other medical treatments for my condition?
·
I have other health
conditions. How can I best manage them together?
·
Are there restrictions
I need to follow?
·
Should I see a
specialist?
·
Are there brochures or
other printed material I can have? What websites do you recommend?
Don't hesitate to ask other questions.
What to expect from
your doctor
Your doctor is likely to ask you several questions,
such as:
·
When did your symptoms
begin?
·
Have your symptoms
been continuous or occasional?
·
How severe are your
symptoms?
·
What, if anything,
seems to improve your symptoms?
·
What, if anything,
appears to worsen your symptoms?
·
What else should I
know about you that will help me make the right recommendations about your
care?
0 Comments