Description

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 are fibromatosis aggressive.

Some of desmoid tumors are slow growing and do not require immediate treatment. Others grow quickly and are treated with surgery, radiation therapy, chemotherapy, or other medications.

Desmoid tumors are not considered cancers, because they do not spread to other areas of the body. But it can be very aggressive, acting more like cancers and growing into nearby structures and organs. For this reason, people with desmoid tumors tend to be attended by doctors of the cancer.

Symptoms

Desmoid tumor symptoms differ depending on where the tumors occur. Desmoid tumors most often occur in the abdomen, arms, and legs. But they can form in any part of the body.

In general, the signs and symptoms include:

  • A mass or area of inflammation
  • The pain
  • The loss of function in the affected area
  • Cramps 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 is not clear what causes desmoid tumors.

Doctors know that these tumors form when the connective tissue of the cell develops changes in your DNA. A cell's DNA contains the instructions that tell a cell what to do. The changes tell the connective tissue cells that multiply quickly, 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 are:

  • Young to adulthood. Desmoid tumors tend to occur in young adults in their 20s and 30s. This tumor is rare in children and the elderly.
  • A genetic syndrome that causes many of the polyps in the colon. People with familial adenomatous polyposis (FAP) have a higher risk of desmoid tumors. FAP is caused by a mutation of the genes that are transmitted from parents to children. Causes numerous growths (polyps) in the colon.
  • Pregnancy. Rarely, a desmoid tumor can develop during or soon after pregnancy.
  • Of the injury. A small number of desmoid tumors develop in people who have recently had an injury or surgery.

Diagnosis

Tests and procedures used to diagnose desmoid tumors are:

  • 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 computed tomography and magnetic resonance imaging to create pictures of the area where the symptoms occur. The images may give your doctor clues about your diagnosis.
  • 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. For desmoid tumors, the sample can be collected with a needle or with surgery, depending on your particular situation. 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 guide treatment.

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. For desmoid tumors, the sample can be collected with a needle or with surgery, depending on your particular situation.

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 guide treatment.

Treatment

Treatments for desmoid tumors are:

  • The control of tumor growth. 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 up and may never require treatment. Some tumors may contract spontaneously 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 the nearby structures and may not be completely removed. In these cases, you can remove the tumor as possible.
  • The radiation therapy. Radiation therapy uses high-powered beams, such as X-rays and protons, to kill tumor cells. Radiation therapy may be recommended in place of the surgery if you are not healthy enough for surgery, or if the tumor is in a place that makes surgery risky. Radiation therapy is sometimes used after surgery if there is a risk that the tumor could return.
  • Chemotherapy and other medications.Chemotherapy uses strong drugs to kill tumor cells. Your doctor may recommend chemotherapy if your desmoid tumor is growing rapidly, and the surgery is not an option. Several other pharmacological treatments have shown promise in people with desmoid tumors, including anti-inflammatory drugs, hormonal therapies and targeted therapies.

Chemotherapy and other medications. Chemotherapy uses strong drugs to kill tumor cells. Your doctor may recommend chemotherapy if your desmoid tumor is growing rapidly, and the surgery is not an option.

Several other pharmacological treatments have shown promise in people with desmoid tumors, including anti-inflammatory drugs, hormonal therapies and targeted therapies.

Coping and support

With time, you will find what helps you to deal with the uncertainty and the anxiety 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 specific condition, including your test results, treatment options, and, if you like, your prognosis. As you learn more about desmoid tumors, may be more confident in making treatment decisions.
  • Keep friends and family close. Keep your close relationships strong will help you deal with your diagnosis. 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.
  • 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 a support group can also be useful.

Preparing for your appointment

Make an appointment with your doctor if you have any of the signs or symptoms that worry you. If your doctor suspects that you may have desmoid tumor, he or she may refer you to a doctor who specializes in the treatment of cancer (oncologist).

Here's some information to help you prepare for your appointment.

What you can do

When you make the appointment, ask if there is something that you need to do in advance, such as fasting before a specific test.

Collect the medical records that pertain to your condition and bring them to your appointment. If you are seeing a new doctor, ask your doctor previous to forward files and other information, such as glass slides containing 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 the medical history of the family
  • All medications, vitamins or supplements that you are taking, including dose
  • Questions to ask your doctor

Consider bringing a family member or friend to help you remember the information they give you.

For desmoid tumors, some basic questions to ask your doctor include:

  • What is likely causing my symptoms?
  • Other that the most likely cause, what are other possible causes of the symptoms?
  • What tests do I need?
  • What is 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?
  • There are restrictions that must be followed?
  • You should see a specialist?
  • Are there brochures or other printed material I can have? What sites do you recommend?

Do not hesitate to ask other questions.

What to expect from your doctor

Your doctor may ask you several questions, such as:

  • When did your symptoms begin?
  • The symptoms been continuous or occasional?
  • How severe are the 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 to make recommendations about your care?
Symptoms and treatment of desmoid tumors