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Desmoid Tumor - Introduction

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Desmoid Tumors. Use the menu to see other pages. Think of that menu as a roadmap for this entire guide.

What are tumors?

A tumor often begins when the DNA of healthy cells is damaged, causing the cells to change and grow out of control, forming a mass. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body, called metastasis, if it is not found early and treated. A benign tumor means the tumor can grow but will not spread to distant places in the body.

About desmoid tumors

Desmoid tumors are a type of soft-tissue tumor that come from fibrous tissue. They are related to connective tissue cancers called sarcomas, but desmoid tumors are not cancer because they do not spread to other parts of the body. Because fibrous tissue is found throughout the body, desmoid tumors can occur in many locations throughout the body.

Many desmoid tumors grow slowly and do not cause problems. But some desmoid tumors grow quickly and can interfere with the body's normal activities. Most desmoids are not life threatening. However, in rare cases, they can be life threatening when they are in very close contact with nearby vital tissues and organs, such as the kidneys, intestines, lungs, major blood vessels, or nerves. This type of tumor can be hard to remove and control, and they often recur, or come back, after surgery. Sometimes, desmoid tumors may shrink without any treatment and become inactive, more like a scar. If this happens, it is called spontaneous regression, and no further treatment is needed. (Learn more about treatment options later in this guide.)

Desmoid tumors begin in cells called fibroblasts, which are the most common type of cells in connective tissue. Fibroblasts also play an important role in wound healing. Desmoid tumors are sometimes referred to as aggressive fibromatosis, desmoid fibromatosis, or deep fibromatosis. They can occur in adults or children.

Although desmoid tumors can develop in many locations, they typically are found in an arm, a leg, or the abdomen. Doctors describe the tumors as:

  • Abdominal wall desmoid tumors. These tumors form within the abdominal wall, which surrounds the organs in the abdomen.

  • Intra-abdominal desmoid tumors. These tumors form in the mesentery, which is the tissue that provides blood vessels and padding to the intestines in the abdomen. They often cause no symptoms unless they compress internal organs or can be felt as a mass.

  • Extra-abdominal desmoid tumors. These tumors form in areas of the body other than the abdomen or abdominal wall. They typically are found in the shoulder, upper arms, chest wall, and upper legs. Sometimes they can be found in the head or neck. Rarely, they can be found in the urologic system, such as in the bladder or scrotum.

A person usually develops a single desmoid tumor, although it is possible to develop more than 1 at a time. When a tumor is found and the doctor believes it could be a desmoid tumor, it is very important to confirm the diagnosis by a biopsy so that appropriate next steps may be followed. Because desmoid tumors are very rare, it is best if an expert pathologist who is familiar with the disease examines the tissue sample. A pathologist is a doctor who uses a microscope to look at a specimen of the tumor to make the diagnosis.

The pathologist will look under the microscope to see the size and shape (called morphology) of the tumor cells and do special stains (called immunohistochemistry) to confirm or revise the diagnosis of desmoid tumor. Sometimes they need more information, and additional genetic tests are performed. If a desmoid tumor is diagnosed, it is important that you are cared for by a health care team that specializes in sarcoma.

Looking for More of an Introduction?

If you would like more of an introduction, explore these related items about sarcomas. Please note that these links will take you to other sections on Cancer.Net:

The next section in this guide is Statistics. It helps explain the number of people who are diagnosed with a desmoid tumor and general survival rates. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Statistics

Approved by the Cancer.Net Editorial Board, 02/2023

ON THIS PAGE: You will find information about the estimated number of people who will be diagnosed with a desmoid tumor each year. You will also read general information on surviving the disease. Remember, survival rates depend on several factors, and no 2 people with a tumor are the same. Use the menu to see other pages.

Every person is different, with different factors influencing their risk of being diagnosed with this tumor and the chance of recovery after a diagnosis. It is important to talk with your doctor about any questions you have around the general statistics provided below and what they may mean for you individually. The original sources for these statistics are provided at the bottom of this page.

How many people are diagnosed with a desmoid tumor?

Desmoid tumors are rare. Each year, approximately 900 to 1,500 people in the United States will be diagnosed with a desmoid tumor. This type of tumor mostly affects people between the ages of 15 and 60 years, but it can occur at any age. Desmoid tumors are more common in women than in men.

In children, desmoid tumors most often occur between the ages of 15 and 16 years. Boys and girls are equally as likely to develop this type of tumor.

What is the survival rate for a desmoid tumor?

There are different types of statistics that can help doctors evaluate a person’s chance of recovery from a desmoid tumor. These are called survival statistics.

It is important to remember that statistics on the survival rates for people with a desmoid tumor are only an estimate. They cannot tell an individual person if the tumor will or will not shorten their life. Instead, these statistics describe trends in groups of people previously diagnosed with the same disease, including specific stages of the disease.

Desmoid tumors rarely cause death. However, they can be painful and grow into nearby tissue, organs, and structures, reducing a person’s quality of life. Because desmoid tumors are so rare, it is hard to determine accurate survival rates, but 1 report found that 99% of patients were alive 5 years after their diagnosis.

The survival rates for desmoid tumor vary based on several factors. These include the size of tumor, a person’s age and general health, and how well the treatment plan works. Other factors that can affect outcomes include where the tumor is located and how quickly the tumor grows. Intra-abdominal desmoid tumors are the most likely to cause potentially life-threatening complications, such as intestinal obstruction. Desmoid tumors in the head and neck region can also be life threatening.

Desmoid tumors often recur, or come back, at or near the original tumor site after surgery. Reported recurrence rates after surgery vary widely, from approximately 20% to as high as 80%. Learn more in this guide's Follow-up care section.

Talk with your doctor if you have any questions about this information. Learn more about understanding statistics.

Statistics adapted from the websites of the National Cancer Institute, National Institutes of Health, National Organization for Rare Disorders, St. Jude Children’s Research Hospital, and The Desmoid Tumor Research Foundation. Additional sources were Meazza C, et al.: Aggressive Fibromatosis in Children and Adolescents: The Italian Experience. Cancer. 2010 Jan 1;116(1):233–240, doi: 10.1002/cncr.24679, and Koskenvuo L, et al.: Comparison of Sporadic and FAP-associated Desmoid-Type Fibromatoses. J Surg Oncol. 2017 Nov;116(6):716–721, doi: 10.1002/jso.24699. (All sources accessed February 2023.)

The next section in this guide is Risk Factors. It describes the factors that may increase the chance of developing a desmoid tumor. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Risk Factors

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will find out more about the factors that increase the chance of developing a desmoid tumor. Use the menu to see other pages.

What are the risk factors for a desmoid tumor?

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause a tumor. Some people with several risk factors never develop a tumor, while others with no known risk factors do. Knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

There are no known causes for desmoid tumors. However, the following risk factors may increase a person’s chance of developing a desmoid tumor:

  • Sex. Desmoid tumors are slightly more common in women than in men.

  • Pregnancy: Desmoid tumors may form around or during the time of pregnancy. Pregnancy-associated desmoid tumors usually develop in the wall of the abdomen. The tumors may be related to the stretching of the abdominal wall during pregnancy, high levels of estrogen that occur during pregnancy, or both.

  • Familial adenomatous polyposis. People with a rare, inherited disease called familial adenomatous polyposis (FAP) have a high risk of desmoid tumors and colorectal cancer. This is sometimes called Gardner syndrome. The desmoid tumors that develop in people with FAP are often intra-abdominal tumors. Approximately 10% to 20% of people with FAP develop a desmoid tumor. Desmoid tumors are the second most common cause of death in people who have FAP. They cause about 9% to 11% of deaths in people with FAP. Certain people diagnosed with a desmoid tumor, particularly in the intra-abdominal area, may need to be screened for FAP.

  • Trauma to the body. Some research shows that serious or repeated trauma to the body, such as surgery or a severe injury, may increase the risk of developing a desmoid tumor at the site of injury, such as in a scar.

The next section in this guide is Symptoms and Signs. It explains what changes or medical problems a desmoid tumor can cause. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will find out more about the changes and medical problems that can be a sign of a desmoid tumor. Use the menu to see other pages.

What are the symptoms and signs of a desmoid tumor?

A desmoid tumor can develop in almost any soft tissue of the body. The types of symptoms that people have from a desmoid tumor depend on where it starts and its size. People with a desmoid tumor may experience one or more of the following symptoms or signs. Symptoms are changes that you can feel in your body. Signs are changes in something measured, like taking your blood pressure or doing a lab test. Together, symptoms and signs can help describe a medical problem. Sometimes, people with a desmoid tumor do not have any of the symptoms and signs described below. Or, the cause of a symptom may be a medical condition that is not a desmoid tumor.

The first sign of a desmoid tumor may be a firm, painless lump or swelling in the arm, leg, chest, neck, or abdomen. Sometimes, the lump is painful. Most lumps are not a desmoid tumor. However, it is important to talk with your doctor about any lumps that are larger than 2 inches (5 centimeters), grow larger, or are painful, regardless of their location.

Because a desmoid tumor develops in flexible, elastic tissues or deep spaces in the body, the tumor can often push normal tissue out of its way as it grows. Therefore, the tumor may sometimes grow quite large before it causes symptoms.

Symptoms may include:

  • Soreness or pain caused by the tumor pressing on nearby nerves, muscles, or blood vessels

  • Tingling or a feeling of “pins and needles,” when the tumor presses on local nerves or blood vessels

  • Limping or other difficulties moving the legs or feet

  • Difficulty moving the arms or hands

  • Pain

Intra-abdominal desmoid tumors may also cause some of the symptoms above, as well as those related to a blocked bowel, including:

  • Bloating

  • Constipation

  • Abdominal pain

  • Rupture of intestines

If you are concerned about any changes you experience, please talk with your doctor. Your doctor will try to understand what is causing your symptom(s). They may do an exam and order tests to understand the cause of the problem, which is called a diagnosis.

If a desmoid tumor is diagnosed, relieving symptoms remains an important part of your medical treatment. Managing symptoms may also be called "palliative and supportive care,” which is not the same as hospice care given at the end of life. It is often started soon after diagnosis and continued throughout treatment. This type of care focuses on managing symptoms and supporting people who face serious illnesses, such as cancer. You can receive palliative and supportive care at any time during treatment. Learn more in this guide’s section on Coping With Treatment.

Be sure to talk with your health care team about the symptoms you experience, including any new symptoms or a change in symptoms.

The next section in this guide is Diagnosis. It explains what tests may be needed to learn more about the cause of the symptoms. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Diagnosis

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will find a list of common tests, procedures, and scans that doctors use to find the cause of a medical problem. Use the menu to see other pages.

Doctors use many tests to find, or diagnose, a desmoid tumor. They also do tests to see the tumor’s size and learn if the tumor is growing. For example, imaging tests can show pictures of the inside of the body to see if the tumor has invaded nearby tissue or structures. Doctors may also do tests to learn which treatments may work best.

For most types of tumors, a biopsy is the only sure way for the doctor to know if an area of the body has a tumor. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Although biopsies have a small chance of not giving a definite answer, they are very important to allow your doctor make a clear diagnosis and develop a clear, comprehensive treatment plan.

How a desmoid tumor is diagnosed

There are different tests used for diagnosing a desmoid tumor. Not all tests described here will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • The suspected location of the tumor

  • Your signs and symptoms

  • Your age and general health

  • The results of earlier medical tests

Desmoid tumors are rare, and there are no standard screening tests for this type of tumor. A doctor should examine any unusual or new lumps or bumps that are growing or are larger than 2 inches. Desmoid tumors can have no symptoms and grow in the body for many months or years before they are diagnosed. A tumor also may be found unexpectedly when people have imaging procedures done for reasons unrelated to the tumor. If a desmoid tumor is suspected, it is very important to talk with a doctor who has experience with this type of tumor.

A diagnosis of a desmoid tumor is made by a combination of clinical examination by a doctor and imaging tests. It is confirmed by the results of a biopsy. In addition to a physical examination, some of the tests described below may be used to diagnose a desmoid tumor.

Imaging tests

Imaging tests show pictures of the inside of the body. Radiologists will use the way the tumor looks on imaging tests to help determine whether it may be a desmoid tumor; however, a biopsy is almost always needed as well. A radiologist is a medical doctor who uses imaging tests to help diagnose disease. If a desmoid tumor is suspected, it is important to have a radiologist experienced in reviewing the imaging scans of desmoid tumors. If possible, the same type of imaging test and the same radiologist should review all subsequent scans so that tumor size measurements are consistent.

  • Magnetic resonance imaging (MRI). An MRI produces detailed images of the inside of the body using magnetic fields, not x-rays. MRI can be used to measure the tumor’s size. A special dye, called contrast, is given before the scan to create a clearer picture. This dye is injected into a patient’s vein. An MRI is the recommended imaging test for abdominal wall and extra-abdominal desmoid tumors. It is also used to look for a recurrence of the tumor after surgery.

  • Computed tomography (CT or CAT) scan. A CT scan takes pictures of the inside of the body using x-rays taken from different angles. A computer combines these pictures into a detailed, 3-dimensional image that shows any abnormalities or tumors. A CT scan can be used to measure the tumor’s size. Sometimes, a special dye called contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein and/or be a pill or liquid the patient must drink. CT scans with contrast are often recommended for diagnosing intra-abdominal tumors.

  • Ultrasound. An ultrasound creates a picture using sound waves. It may be used to look at lumps under the skin or other organs in the body.

Biopsy

Imaging tests may suggest the diagnosis of a desmoid tumor, but a biopsy is needed to confirm the diagnosis. It is very important for a patient to see a desmoid tumor specialist before any surgery or biopsy is done.

  • Biopsy. A biopsy is the only way to make a definite diagnosis, even if other tests can suggest that cancer is present. During biopsy, a small amount of tissue is removed for examination under a microscope. A pathologist analyzes the sample(s). A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

    Because desmoid tumors are uncommon and can be misdiagnosed, an expert sarcoma pathologist should review the tissue sample to properly diagnose the tumor. Properly diagnosing a desmoid tumor requires special tests on the tumor tissue, and it is best if a specialist familiar with this type of tumor does this.

    There are different types of biopsies. The type that is done depends on the location of the tumor. For a needle biopsy, a doctor removes a small sample of tissue from the tumor with a needle-like instrument—usually a core needle biopsy. Fine needle aspirations are not recommended because they do not reliably find desmoid tumors. The biopsy may be performed with the help of ultrasound, CT scan, or MRI (see “Imaging tests,” above) to precisely guide the needle into the tumor. In an incisional biopsy, a surgeon cuts into the tumor and removes a sample of tissue. In an excisional biopsy, the surgeon removes the entire tumor. Because desmoid tumors frequently recur after surgery (see Types of Treatments), it is important to talk with your doctor about the potential benefits and risks of this type of biopsy with your doctor.

    The type of biopsy and how it is done are important in diagnosing and treating desmoid tumors, so patients should be seen in a sarcoma specialty center even before the biopsy is performed.

  • Tissue testing of the tumor. Your doctor or the pathologist looking at the tumor may recommend running laboratory tests on a tumor sample to identify specific genes, proteins, and other factors unique to the tumor. This may also be called molecular testing of the tumor.

    Beta-catenin     (CTNNB1) gene mutations occur in 85% of desmoid tumors that are sporadic. This means the mutation is acquired during a patient’s lifetime and is not inherited. Mutations in the adenomatous polyposis coli (APC) gene cause 10% to 15% of sporadic desmoid tumors. In addition, inherited mutations in the APC gene can cause desmoid tumors associated with familial adenomatous polyposis (FAP; see Risk Factors). Results of laboratory tests that screen for CTNNB1 and/or APC mutations can help confirm a desmoid tumor diagnosis and may help determine what the treatment should be (see Types of Treatment).

After diagnostic tests are done, your doctor will review the results with you. If the diagnosis is a desmoid tumor, these results also help the doctor describe it. This is called staging.

If you are diagnosed with a desmoid tumor, it is important to learn if any other members of your family may have had desmoid tumors, colorectal cancer, or multiple colon polyps in the past. Approximately 10% to 20% of people with FAP, a genetic syndrome, develop a desmoid tumor (see Risk Factors). When planning treatment, doctors may recommend that certain patients have a colonoscopy to look for colon polyps or talk with a genetic counselor to learn whether their tumor is associated with FAP. If so, the syndrome may affect other family members as well, and specific genetic tests may be recommended for them. Learn more about genetic testing.

The next section in this guide is Stages. It explains the system doctors use to describe the extent of the disease. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Stages

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will learn about how doctors describe a tumor’s growth or spread. This is called the stage. Use the menu to see other pages.

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting other parts of the body.

There are no official staging systems for desmoid tumors. Instead, the health care team develops a treatment plan based on the individual's specific situation. This may include the size of the tumor, if the tumor is causing symptoms or complications, and other factors.

Information about the tumor will help the doctor recommend a specific treatment plan. The next section in this guide is Types of Treatment. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Types of Treatment

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will learn about the different types of treatments doctors use for people with a desmoid tumor. Use the menu to see other pages.

This section explains the types of treatments, also known as therapies, that are the standard of care for desmoid tumors. “Standard of care” means the best treatments known. Information in this section is based on medical standards of care for a desmoid tumor in the United States. Treatment options can vary from one place to another.

When making treatment plan decisions, you are encouraged to discuss with your doctor whether clinical trials offer additional options to consider. A clinical trial is a research study that tests a new approach to treatment. Doctors learn through clinical trials whether a new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Clinical trials are an option for all stages of tumors. Your doctor can help you consider all your treatment options. Learn more about clinical trials in the About Clinical Trials and Latest Research sections of this guide.

How a desmoid tumor is treated

For a desmoid tumor, different types of doctors who specialize in tumors, called oncologists, often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team. Tumor care teams include other health care professionals, such as physician assistants, nurse practitioners, oncology nurses, social workers, pharmacists, counselors, dietitians, physical therapists, occupational therapists, and others. Because desmoid tumors are rare, it is important for your treatment plan to be developed by a team that specializes in sarcomas. Learn more about the clinicians who provide cancer care.

Treatment options and recommendations depend on several factors, including the:

  • Growth rate of the desmoid tumor

  • Whether the desmoid is causing symptoms

  • Possible side effects of treatment

  • Patient’s age, preferences, and overall health

Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. These types of conversations are called “shared decision-making.” Shared decision-making is when you and your doctors work together to choose treatments that fit the goals of your care. Shared decision-making is important for desmoid tumors because there are different treatment options. Learn more about making treatment decisions.

The common types of treatments used for a desmoid tumor are described below. Your care plan may also include treatment for symptoms and side effects, an important part of tumor care.

READ MORE BELOW:

Active surveillance

Active surveillance may be an option for people with desmoid tumors that are not causing any symptoms or entangling with vital organs. This approach is sometimes called “watchful waiting.” While some desmoid tumors can grow quickly, others grow slowly, stay the same size, or shrink without active treatment. During active surveillance, the patient is monitored closely with periodic examinations and tests (see Diagnosis) to watch for changes in the tumor, along with appropriate treatments to manage symptoms. Active treatment may begin if the tumor progresses, causes symptoms, or is getting close to vital structures (see Symptoms and Signs).

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Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. Surgery used to be the standard treatment for desmoid tumors. Today, many doctors are considering more conservative, non-surgical, treatment options first. That is because a desmoid tumor frequently comes back at or near the original tumor site after surgery. Recurrence rates have been reported to be as high as 70% by some researchers. In addition, desmoid tumors that recur after surgery are often more aggressive. However, surgery may sometimes still be a successful treatment option for some patients.

Before surgery, it is important to have a biopsy and appropriate imaging to confirm the diagnosis of a desmoid tumor (see Diagnosis). Surgical oncologists and orthopedic oncologists are doctors who specialize in treating desmoid tumors and other sarcomas using surgery.

The surgeon's goal is to remove the entire tumor and enough normal tissue surrounding it to obtain a clean margin around the tumor. A clean margin is when there are no tumor cells visible at the borders of the surgical specimen. This is currently the best method available to ensure that there are no tumor cells left in the area from which the tumor was removed. But because desmoid tumors can intertwine with nearby tissue and structures, it may be difficult or even impossible for the surgeon to identify where the tumor starts and stops. Radiation therapy (see below) may be used after surgery to destroy any remaining tumor cells, if the patient did not receive radiation therapy before surgery. This type of treatment after surgery is called “adjuvant therapy.”

Before surgery, talk with your health care team about the risk of the tumor returning. Certain types of desmoid tumors are more likely to recur after surgery than others. You should also discuss possible side effects from the specific surgery you will have, including the recovery period. Learn more about the basics of cancer surgery.

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Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy tumor cells. A doctor who specializes in giving radiation therapy to treat a tumor is called a radiation oncologist.

Many sarcoma experts do not recommend radiation therapy for desmoid tumors. This is because, in rare cases, radiation therapy can cause cancerous tumors or new and more aggressive sarcomas. In the unlikely event that this happens, a cancer may develop 7 to 20 years after radiation therapy. It is important to talk with your health care team about this risk and other potential side effects of radiation therapy before you have this treatment (see “Physical, emotional, social, and financial effects of a tumor”). Since desmoid tumors are rare, if radiation therapy is being considered it is very important to talk with a radiation oncologist who has experience treating them.

Learn more about radiation therapy.

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Cryosurgery or cryoablation

Cryosurgery, also called cryotherapy or cryoablation, is a localized treatment that uses extremely cold substances to freeze and kill tumor cells. It may be used if surgery is not an option. The therapy appears to be more effective in controlling smaller extra-abdominal tumors than larger extra-abdominal tumors that have invaded vital structures. Typically, several cycles of cryosurgery are done. One recent study found that cryosurgery stopped tumor progression in almost 90% of patients. In other patients, the tumor was completely destroyed.

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Radiofrequency thermal ablation

Radiofrequency thermal ablation (RFA) uses heat to destroy tumor cells. Like cryosurgery (see above), it is a minimally invasive treatment option that may be useful for treating desmoid tumors that cannot be removed with surgery.

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Therapies using medication

The treatment plan may include medications to destroy tumor cells. Medication may be given through the bloodstream to reach tumor cells throughout the body. When a drug is given this way, it is called systemic therapy. Medication may also be given locally, which is when the medication is applied directly to the tumor or kept in a single part of the body.

This treatment is generally prescribed by a medical oncologist, a doctor who specializes in treating tumors with medication.

Medications are often given through an intravenous (IV) tube placed into a vein using a needle or as a pill or capsule that is swallowed (orally). If you are given oral medications to take at home, be sure to ask your health care team about how to safely store and handle them.

The types of medications used for desmoid tumors include:

  • Nonsteroidal anti-inflammatory drugs

  • Hormone therapy

  • Targeted therapy

  • Chemotherapy

Each of these types of therapies is discussed below in more detail. A person may receive 1 type of medication at a time or a combination of medications given at the same time. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy. Some of these may be used as first-line treatment, if surgery is not recommended.

The medications used to treat tumors are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications.

It is also important to let your doctor know if you are taking any other prescription or over-the-counter medications or supplements. Herbs, supplements, and other drugs can interact with medications used to treat tumors, causing unwanted side effects or reduced effectiveness. Learn more about your prescriptions by using searchable drug databases.

Nonsteroidal anti-inflammatory drugs

Nonsteroidal anti-inflammatory drugs (NSAIDs) can sometimes slowly shrink a desmoid tumor, as well as relieve associated pain and swelling. However, data from recent clinical trials have shown that it is less clear whether NSAIDs may actively shrink tumors. These drugs may include celecoxib (Celebrex), ibuprofen (multiple brand names), indomethacin (Tivorbex), naproxen (multiple brand names), or sulindac (available as a generic drug). NSAIDs may be used in combination with other treatments, such as hormone therapy.

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Hormone therapy

Research shows that the hormone estrogen appears to contribute to desmoid tumor growth. Hormone therapy may be used to control tumor growth. Tamoxifen (available as a generic drug) is the most commonly used hormone therapy for desmoid tumors. Tamoxifen blocks a cell’s ability to use estrogen, which may stop or slow the growth of tumor cells that depend on it. However, recent clinical trial data has shown that patients receiving a placebo also had spontaneous regression of desmoid tumors, which makes it less clear that tamoxifen is able to actively shrink tumors. The side effects of tamoxifen may include hot flashes, vaginal discharge, sexual side effects, mood changes, and a higher risk of developing uterine cancer, blood clots, and stroke. Hormone therapy may be used in combination with NSAIDs or other treatments. Patients diagnosed with a desmoid tumor should stop estrogen replacement therapy if they receive hormone therapy for a desmoid tumor. Learn more about the basics of hormone therapy.

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Targeted therapy (updated 11/2023)

Targeted therapy is a treatment that targets the tumor’s specific genes, proteins, or the tissue environment that contributes to its growth and survival. This type of treatment blocks the growth and spread of tumor cells and limits damage to healthy cells.

For progressing desmoid tumors that cannot be treated with surgery, a medication called nirogacestat (Ogsiveo) may be recommended. This is the first drug approved by the U.S. Food and Drug Administration (FDA) specifically for treating desmoid tumors. Nirogacestat works by blocking gamma secretase. Gamma secretase activates a protein called Notch, and researchers suspect that the Notch protein helps desmoid tumors grow. The most common side effects of nirogacestat include diarrhea, rash, nausea, fatigue, mouth sores, headache, abdominal pain, cough, hair loss, infections of the respiratory tract, and shortness of breath. Women who took this medication also experienced fertility problems linked to the function of the ovaries. For the majority of these women, the fertility problems went away after treatment was stopped.

Tyrosine kinase inhibitors (TKIs) are drugs that target and block specific proteins called kinases that contribute to the tumor’s growth and survival. Some TKIs may be useful in treating a progressing or recurrent desmoid tumor by shrinking it or halting its growth.

A global phase III clinical trial studied using a TKI called sorafenib (Nexavar) to treat desmoid tumors. Sorafenib is currently used to treat other cancers, including liver, thyroid, and kidney cancers. In this clinical trial, patients were randomly assigned to 2 groups, either given sorafenib or a placebo tablet. Of those who took sorafenib in the clinical trial, 33% of patients had their desmoid shrink and 87% had their disease stopped for more than 1 year. It is important to note that approximately 20% of patients who received placebo also had their desmoids shrink. In this study, 18% of patients who took sorafenib had to stop taking the drug because of side effects. Common side effects of sorafenib include high blood pressure, fatigue, diarrhea, decrease in appetite, nausea, vomiting, rash, dry or itchy skin, and mouth ulcers. This drug is taken as a pill by mouth.

Another commonly used TKI in desmoid tumor treatment is imatinib (Gleevec). This drug is also taken as a pill by mouth. It is approved by the FDA for a type of soft-tissue sarcoma called gastrointestinal stromal tumor (GIST), as well as other conditions. The most common side effects of imatinib are fluid accumulation, rash, nausea, diarrhea, fatigue, and minor muscle aches. Serious but relatively rare side effects include bleeding and inflammation of the liver. These side effects, and side effects of other TKIs, can often be successfully managed by your doctor.

In addition, pazopanib (Votrient) is a multitargeted TKI that is sometimes used to treat desmoid tumors. In a recent phase II clinical trial, the tumors of patients with advanced desmoid tumors who received pazopanib as a pill by mouth showed more shrinkage compared to a combined chemotherapy treatment of methotrexate and vinblastine (see “Chemotherapy”). The drug received FDA approval to treat advanced soft-tissue sarcomas in 2012. The most common side effects of pazopanib are diarrhea, fatigue, loss of coloration in the hair and skin, and high blood pressure.

Learn more about the basics of targeted treatments.

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Chemotherapy

Chemotherapy is medication that kills tumor cells and keeps them from growing, dividing, and making more cells. Tumor cells grow and divide faster than normal cells. However, normal cells also grow and divide, so the side effects of chemotherapy are due to the treatment’s effects on normal cells that are growing and dividing.

A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or a combination of different drugs given at the same time. Chemotherapy for a desmoid tumor can usually be given as an outpatient treatment, which means you don’t need to stay overnight at the hospital.

Different drugs are used to treat desmoid tumors. Some types of chemotherapy that might be used alone or in combination for desmoid tumor include:

  • Liposomal doxorubicin (Doxil)

  • Methotrexate (Trexall)

  • Vinblastine (Velban)

  • Doxorubicin (available as a generic drug), used alone or combined with dacarbazine

  • Dacarbazine (available as a generic drug), only used in combination with doxorubicin

  • Vinorelbine (available as a generic drug)

Chemotherapy may be used to shrink an aggressive, organ-threatening desmoid tumor or one that is causing symptoms. The decision to use chemotherapy should be made after discussion with an experienced sarcoma expert.

The side effects of chemotherapy depend on the individual drug and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away after treatment is finished. In rare cases, there are long-term problems that affect the heart or kidneys or cause cancer.

Learn more about the basics of chemotherapy.

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Physical, emotional, social, and financial effects of a tumor

A desmoid tumor and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative and supportive care. It is an important part of your care that is included along with treatments intended to slow, stop, or eliminate the tumor.

Palliative and supportive care focuses on improving how you feel during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of tumor may receive this type of care. And it often works best when it is started right after a tumor diagnosis. People who receive palliative and supportive care along with treatment for the tumor often have less severe symptoms, better quality of life, and report that they are more satisfied with treatment.

Supportive treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. You may also receive palliative treatments, such as chemotherapy, surgery, or radiation therapy, to improve symptoms.

Before treatment begins, talk with your doctor about the goals of each treatment in the recommended treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative and supportive care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.

Cancer care is often expensive, and navigating health insurance can be difficult. Ask your doctor or another member of your health care team about talking with a financial navigator or counselor who may be able to help with your financial concerns.

During treatment, your health care team may ask you to answer questions about your symptoms and side effects and to describe each problem. Be sure to tell the health care team if you are experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.

Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative and supportive care in a separate section of this website.

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Remission and the chance of recurrence

A remission is when tumor cells cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED. Sometimes desmoid tumors will shrink on their own and will not require further treatment. If this happens, it is called spontaneous regression.

Often, desmoid tumors will shrink and/or become inactive but a mass is still present. This mass is like a scar and does not require treatment. However, some patients may continue to have pain or other symptoms due to the location of the scar.

A remission may be temporary or permanent. This uncertainty causes many people to worry that the tumor will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the tumor returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the tumor does return. Learn more about coping with the fear of recurrence.

If the tumor returns or becomes active again after the original treatment, it is called a recurrent tumor. Desmoid tumors do not metastasize to other parts of the body, but they can recur locally. This means they come back in the same place they started.

If a recurrence happens, a new cycle of testing will begin to learn as much as possible about it. After this testing is done, you and your doctor will talk about the treatment options. Often the treatment plan will include the treatments described above, such as surgery, chemotherapy, targeted therapy, and radiation therapy, but they may be used in a different combination or given at a different pace. Your doctor may suggest clinical trials that are studying new ways to treat a recurrent desmoid tumor. Whichever treatment plan you choose, palliative and supportive care will be important for relieving symptoms and side effects.

People with a recurrent desmoid tumor sometimes experience emotions such as disbelief or fear. You are encouraged to talk with your health care team about these feelings and ask about support services to help you cope. Learn more about dealing with cancer recurrence.

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If treatment does not work

Recovery from a desmoid tumor is not always possible. If the tumor cannot be removed, it can often still be controlled for a very long time. It is important to understand that patients can live with a tumor in their body as long as it does not affect the function of a major organ. Therefore, the goal of treatment is to control the tumor and preserve organ function.

If the tumor can no longer be controlled, it is called end stage or terminal. This is very rare in patients with a desmoid tumor. But if it does happen, this diagnosis is stressful and can be difficult to discuss. However, it is important to have open and honest conversations with your health care team to express your feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help. Making sure a person is physically comfortable, free from pain, and emotionally supported is extremely important.

Planning for your future care and putting your wishes in writing is important, especially at this stage of disease. Then, your health care team and loved ones will know what you want, even if you are unable to make these decisions. Learn more about putting your health care wishes in writing.

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The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for people with a tumor. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - About Clinical Trials

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are studied to see how well they work. Use the menu to see other pages.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for people with a desmoid tumor. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. Every drug that is now approved by the U.S. Food and Drug Administration (FDA) was tested in clinical trials.

Clinical trials are used for all types of desmoid tumors. Many focus on new treatments to learn if a new treatment is safe, effective, and possibly better than the existing treatments. These types of studies evaluate new drugs, different combinations of treatments, new approaches to radiation therapy or surgery, and new methods of treatment.

People who participate in clinical trials can be some of the first to get a treatment before it is available to the public. However, there are some risks with a clinical trial, including possible side effects and the chance that the new treatment may not work. People are encouraged to talk with their health care team about the pros and cons of joining a specific study.

Some clinical trials study new ways to relieve symptoms and side effects during treatment. Others study ways to manage the late effects that may happen a long time after treatment. Talk with your doctor about clinical trials for symptoms and side effects.

Deciding to join a clinical trial

People decide to participate in clinical trials for many reasons. For some, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Others volunteer for clinical trials because they know that these studies are a way to contribute to the progress in treating desmoid tumors. Even if they do not benefit directly from the clinical trial, their participation may benefit future people with a desmoid tumor.

Insurance coverage and the costs of clinical trials differ by location and by study. In some programs, some of the expenses from participating in the clinical trial are reimbursed. In others, they are not. It is important to talk with the research team and your insurance company first to learn if and how your treatment in a clinical trial will be covered. Learn more about health insurance coverage of clinical trials.

Sometimes people have concerns that, in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” Study participants will always be told when a placebo is used in a study. Find out more about placebos in clinical trials.

Patient safety and informed consent

To join a clinical trial, people must participate in a process known as informed consent. During informed consent, the doctor should:

  • Describe all of the treatment options so that the person understands how the new treatment differs from the standard treatment.

  • List all of the risks of the new treatment, which may or may not be different from the risks of standard treatment.

  • Explain what will be required of each person in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

  • Describe the purposes of the clinical trial and what researchers are trying to learn.

Clinical trials also have certain rules called “eligibility criteria” that help structure the research and keep patients safe. You and the research team will carefully review these criteria together. You will need to meet all of the eligibility criteria in order to participate in a clinical trial. Learn more about eligibility criteria in clinical trials.

People who participate in a clinical trial may stop participating at any time for personal or medical reasons. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that people participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if they choose to leave the clinical trial before it ends.

Finding a clinical trial

Research through clinical trials is ongoing for all types of tumors. For specific topics being studied for desmoid tumor, learn more in the Latest Research section.

Cancer.Net offers more information about clinical trials in other areas of the website, including a complete section on clinical trials.

There are many resources and services to help you search for clinical trials for desmoid tumors, including the following services. Please note that these links will take you to separate, independent websites:

  • ClinicalTrials.gov. This U.S. government database lists publicly and privately supported clinical trials.

  • World Health Organization (WHO) International Clinical Trials Registry Platform. The WHO coordinates health matters within the United Nations. This search portal gathers clinical trial information from many countries’ registries.

Read more about the basics of clinical trials matching services.

The next section in this guide is Latest Research. It explains areas of scientific research for desmoid tumors. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Latest Research

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will read about the scientific research being done to learn more about desmoid tumors and how to treat them. Use the menu to see other pages.

Doctors are working to learn more about desmoid tumors, ways to prevent them, how to best treat them, and how to provide the best care to people diagnosed with them. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the best diagnostic and treatment options for you.

  • Targeted therapy. As explained in Types of Treatment, certain targeted therapies, such as sorafenib and pazopanib, are being used to treat desmoid tumors. This is an active area of research for desmoid tumors. One promising targeted therapy starting to be researched for the treatment of adults with aggressive desmoid tumors is a small molecule, gamma-secretase inhibitor called nirogacestat. Drugs called “small molecule drugs” can block the process that helps tumor cells multiply and spread.

    Another targeted therapy beginning to be researched is tegavivint. This drug may bind to a protein called TBL1. TBL1 binds to beta-catenin, which the CTNNB1 gene provides instructions for making. The goal is to inhibit excess beta-catenin caused by a CTNNB1 gene mutation.

  • New hormone therapies. Because estrogen may play a role in desmoid tumors, 1 study is exploring the use of endoxifen (Z-Endoxifen HCl) for treatment in tumors that have not responded to standard hormone treatments. This experimental drug has been shown to slow or stop the growth of other estrogen-linked cancers.

  • High-frequency ultrasound. This emerging, noninvasive therapy uses focused ultrasound beams to target and destroy a desmoid tumor without harming nearby tissue. It could offer an alternative to surgery in some patients. Although high-frequency ultrasound is being used to treat desmoid tumors in a few medical centers, clinical trial research on its effectiveness and long-term effects is just beginning.

  • Palliative and supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current desmoid tumor treatments to improve comfort and quality of life for patients.

Looking for More About the Latest Research?

If you would like more information about the latest areas of research in desmoid tumors, explore these related items that take you outside of this guide:

The next section in this guide is Coping with Treatment. It offers some guidance on how to cope with the physical, emotional, social, and financial changes that a tumor and its treatment can bring. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Coping with Treatment

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will learn more about coping with the physical, emotional, social, and financial effects of a tumor and its treatment. Use the menu to see other pages.

Every treatment can cause side effects or changes to your body and how you feel. For many reasons, people do not experience the same side effects even when they are given the same treatment for the same type of tumor. This can make it hard to predict how you will feel during treatment.

READ MORE BELOW:

As you prepare to start treatment, it is normal for some people to fear treatment-related side effects. It may help to know that your health care team will work to prevent and relieve side effects. This part of treatment is called palliative and supportive care. It is an important part of your treatment plan, regardless of your age or the stage of disease.

Coping with physical side effects

Common physical side effects from each treatment option for desmoid tumor are described in the Types of Treatment section. Learn more about side effects of a tumor and its treatment, along with ways to prevent or control them. Changes to your physical health depend on several factors, including the tumor’s stage, the length and dose of treatment, and your general health.

Talk with your health care team regularly about how you are feeling. It is important to let them know about any new side effects or changes in existing side effects. If they know how you are feeling, they can find ways to relieve or manage your side effects to help you feel more comfortable and potentially keep any side effects from worsening.

You may find it helpful to keep track of your side effects so it is easier to talk about any changes with your health care team. Learn more about why tracking side effects is helpful.

Sometimes, side effects can last after treatment ends. Doctors call these long-term side effects. Side effects that occur months or years after treatment are called late effects. Treating long-term side effects and late effects is an important part of survivorship care. Learn more by reading the Follow-up Care section of this guide or talking with your doctor.

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Coping with emotional and social effects

You can have emotional and social effects after a desmoid tumor diagnosis. This may include dealing with a variety of emotions, such as sadness, anxiety, fear, or anger, or managing stress. Sometimes, people find it difficult to express how they feel to their loved ones. Some have found that talking to an oncology social worker, counselor, or member of the clergy can help them develop more effective ways of coping and talking about their diagnosis.

You can also find coping strategies for emotional and social effects in a separate section of this website. This section includes many resources for finding support and information to meet your needs.

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Coping with the costs of medical care

Medical treatment can be expensive. It may be a source of stress and anxiety for people with a tumor and their families. In addition to treatment costs, many people find they have extra, unplanned expenses related to their care. For some people, the high cost of medical care stops them from following or completing their treatment plan. This can put their health at risk and may lead to higher costs in the future. Patients and their families are encouraged to talk about financial concerns with a member of their health care team. Learn more about managing financial considerations in a separate part of this website.

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Coping with barriers to care

Some groups of people experience different rates of new cases of tumors and experience different outcomes from their diagnosis. These differences are called “health disparities.” Disparities are caused in part by real-world barriers to quality medical care and social determinants of health, such as where a person lives and whether they have access to food and health care. Health disparities more often negatively affect racial and ethnic minorities, people with fewer financial resources, sexual and gender minorities (LGBTQ+), adolescent and young adult populations, adults older than 65, and people who live in rural areas or other underserved communities.

If you are having difficulty getting the care you need, talk with a member of your health care team or explore other resources that help support medically underserved people.

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Talking with your health care team about side effects

Before starting treatment, talk with your doctor about possible side effects. Ask:

  • Which side effects are most likely?

  • When are they likely to happen?

  • What can we do to prevent or relieve them?

  • When and who should I call about side effects?

Be sure to tell your health care team about any side effects that happen during treatment and afterward, too. Tell them even if you do not think the side effects are serious. This discussion should include physical, emotional, social, and financial effects of having a tumor.

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Caring for a loved one with a desmoid tumor

Family members and friends often play an important role in taking care of a person with a desmoid tumor. This is called being a caregiver. Caregivers can provide physical, practical, and emotional support to the patient, even if they live far away. Being a caregiver can also be stressful and emotionally challenging. One of the most important tasks for caregivers is caring for themselves.

Caregivers may have a range of responsibilities on a daily or as-needed basis, including:

  • Providing support and encouragement

  • Talking with the health care team

  • Giving medications

  • Helping manage symptoms and side effects

  • Coordinating medical appointments

  • Providing a ride to and from appointments

  • Assisting with meals

  • Helping with household chores

  • Handling insurance and billing issues

A caregiving plan can help caregivers stay organized and help identify opportunities to delegate tasks to others. It may be helpful to ask the health care team how much care will be needed at home and with daily tasks during and after treatment. Use this 1-page fact sheet to help make a caregiving action plan. This free fact sheet is available as a PDF, so it is easy to print.

Learn more about caregiving or read the ASCO Answers Guide to Caring for a Loved One With Cancer in English or Spanish.

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Looking for More on How to Track Side Effects?

Cancer.Net offers several resources to help you keep track of your symptoms and side effects. Please note that these links will take you to other sections of Cancer.Net:

  • ASCO Answers Fact Sheets: Read 1-page fact sheets on anxiety and depression, constipation, diarrhea, and rash that provide a tracking sheet to record details about the side effect. These free fact sheets are available as a PDF, so they are easy to print, fill out, and give to your health care team.

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The next section in this guide is Follow-up Care. It explains the importance of checkups after tumor treatment is finished. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Follow-Up Care

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will read about your medical care after treatment for a tumor is completed and why this follow-up care is important. Use the menu to see other pages.

Care for people diagnosed with a desmoid tumor does not end when active treatment has finished. Your health care team will continue to check that the tumor has not come back, manage any side effects, and monitor your overall health. This is called follow-up care.

Your follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your recovery in the months and years ahead. Rehabilitation may be recommended, and this could mean any of a wide range of services, such as physical therapy, occupational therapy, career counseling, pain management, nutritional planning, and/or emotional counseling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent as possible.

For people treated for a desmoid tumor in an arm or leg, a rehabilitation program after surgery or radiation therapy can help them regain or maintain limb and joint function. Range-of-motion exercises, strengthening exercises, and a program to reduce lymphedema may be recommended. A rehabilitation medicine specialist can help patients receive the most appropriate rehabilitation after treatment. In the rare instances when treatment includes amputation, services that provide artificial limbs, called prosthetics, and additional mental health support can help manage the adjustment to life following the loss of a limb. Learn more about rehabilitation.

One goal of follow-up care is to check for new tumor growth or if the tumor has come back after surgery, called recurrence. Because desmoid tumors can be inactive, or stable, for a long period of time, your doctor will recommend a plan to monitor for new growth. A tumor recurs because small areas of tumor cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms. Desmoid tumors often recur locally, and their behavior is unpredictable. During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of tumor growth or recurrence. Your doctor will ask specific questions about your health. Some people will have imaging tests done as part of regular follow-up care, but testing recommendations depend on several factors, including the size and location of the desmoid tumor. In general, a person who received desmoid tumor treatment, with or without surgery, should have a physical exam and/or imaging tests on a regular schedule decided by their doctor. These tests will often be less frequent if there are no signs of tumor growth. In some cases, a physical examination alone, without a scan, will be performed. If the tumor is being treated with medication, blood tests may also be done.

The anticipation before having a follow-up test or waiting for test results may add stress to you or a family member. This is sometimes called “scanxiety.” Learn more about how to cope with this type of stress.

Learn more about the importance of follow-up care.

Managing long-term and late side effects

Most people expect to have side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. Other side effects called late effects may develop months or even years after treatment has ended. Long-term and late effects can include both physical and emotional changes.

Talk with your doctor about your risk of developing such side effects based on the individual factors of your tumor, your specific treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may also have certain physical examinations, scans, or blood tests to help find and manage them.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to discuss any concerns you have about your future physical or emotional health. The American Society of Clinical Oncology (ASCO) offers forms to help keep track of the treatment you received and develop a survivorship care plan when treatment is completed.

This is also a good time to talk with your doctor about who will lead your follow-up care. Some survivors continue to see their oncologist, while others transition back to the care of their primary care doctor or another health care professional. This decision depends on several factors, including treatments received, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in treating your desmoid tumor will lead your follow-up care, be sure to share your treatment summary and survivorship care plan forms with them and with all future health care providers. Details about your treatment for a desmoid tumor are very valuable to the health care professionals who will care for you throughout your lifetime.

The next section in this guide is Survivorship. It describes how to cope with challenges in everyday life after a tumor diagnosis. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Survivorship

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will read about how to cope with challenges in everyday life after a tumor diagnosis. Use the menu to see other pages.

What is survivorship?

The word “survivorship” is complicated because it means different things to different people. Common definitions include:

  • Having no signs of a tumor after finishing treatment.

  • Living with, through, and beyond a tumor. According to this definition, tumor survivorship begins at diagnosis and continues during treatment and through the rest of a person’s life.

For some, the term “survivorship” itself does not feel right, and they may prefer to use different language to describe and define their experience. Sometimes long-term treatment will be used for months or years to manage or control a desmoid tumor. Living with a tumor indefinitely is not easy, and the health care team can help you manage the challenges that come with it. Everyone has to find their own path to name and navigate the changes and challenges that are the results of their diagnosis and treatment.

Survivors may experience a mixture of feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after their diagnosis and have gained a greater acceptance of themselves. Others become very anxious about their health and uncertain about coping with everyday life. Feelings of fear and anxiety may still occur as time passes, but these emotions should not be a constant part of your daily life. If they persist, be sure to talk with a member of your health care team.

Survivors may feel some stress when their frequent visits to the health care team end after completing treatment. Often, relationships built with the health care team provide a sense of security during treatment, and people miss this source of support. This may be especially true when new worries and challenges surface over time, such as any late effects of treatment, emotional challenges including fear of recurrence, sexual health and fertility concerns, and financial and workplace issues.

Every survivor has individual concerns and challenges. With any challenge, a good first step is being able to recognize your fears and talk about them. Effective coping requires:

  • Understanding the challenge you are facing

  • Thinking through solutions

  • Asking for and allowing the support of others

  • Feeling comfortable with the course of action you choose

Many survivors find it helpful to join an in-person support group or an online community of survivors. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of your health care team, individual counseling, or asking for assistance at the learning resource center of the place where you received treatment.

A new perspective on your health

For many people, survivorship serves as a strong motivator to make lifestyle changes.

People recovering from a desmoid tumor are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, exercising regularly, and managing stress. Regular physical activity can help rebuild your strength and energy level. Your health care team can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about making healthy lifestyle choices.

It is important to have recommended medical checkups and tests (see Follow-up Care) to take care of your health.

Talk with your health care team to develop a survivorship care plan that is best for your needs.

Changing role of caregivers

Family members and friends may also go through periods of transition. A caregiver plays a very important role in supporting a person diagnosed with a tumor, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. Eventually, the need for caregiving related to the tumor diagnosis will become much less or come to an end. Caregivers can learn more about adjusting to life after caregiving.

Looking for More Survivorship Resources?

For more information about survivorship, explore these related items. Please note that these links will take you to other sections of Cancer.Net:

  • ASCO Answers Guide to Cancer Survivorship: Get this 48-page booklet that helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms. The free booklet is available as a PDF, so it is easy to print.

  • Survivorship Resources: Cancer.Net offers information and resources to help survivors cope, including specific sections for children, teens and young adults, and people over age 65. There is also a main section on survivorship for people of all ages.

The next section offers Questions to Ask the Health Care Team to help start conversations with your health care team. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Questions to Ask the Health Care Team

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will find some questions to ask your doctor or other members of the health care team, to help you better understand your diagnosis, treatment plan, and overall care. Use the menu to see other pages.

Talking often with the health care team is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your medical care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment. It may also be helpful to ask a family member or friend to come with you to appointments to help take notes.

Questions to ask after getting a diagnosis

  • Where is the desmoid tumor located?

  • How large or aggressive is the tumor? Is it affecting vital organs, tissues, or structures?

  • Has my diagnosis been reviewed by a pathologist who is experienced in the diagnosis of desmoid tumors?

  • Can you explain my pathology report (laboratory test results) to me?

  • What does my diagnosis mean?

  • Can you recommend a desmoid tumor specialist?

  • Was my case discussed in a tumor board or other multidisciplinary conference? What does this mean?

  • Do I need to be screened for familial adenomatous polyposis (FAP)?

Questions to ask about choosing a treatment and managing side effects

  • Does my desmoid tumor need to be treated right now?

  • What are my treatment options?

  • What types of research are being done for desmoid tumors in clinical trials? Do clinical trials offer additional treatment options for me?

  • How will my desmoid tumor be monitored?

  • What treatment plan do you recommend? Why?

  • What is the goal of each treatment? Is it to eliminate the tumor, help me feel better, or both?

  • What are the possible side effects of this treatment, both in the short term and the long term?

  • Who will be part of my health care team, and what does each member do?

  • Who will be leading my overall treatment?

  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?

  • Could this treatment affect my sex life? If so, how and for how long?

  • Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before treatment begins?

  • If I am worried about managing the costs of medical care, who can help me?

  • What support services are available to me? To my family?

  • If I have questions or problems, who should I call?

Questions to ask about active surveillance

  • Should I consider observation/watchful waiting instead of active treatment for this tumor? If so, why?

  • What might happen if I choose not to start treatment now?

  • How will the desmoid tumor be monitored for possible growth or changes?

  • How often will I have to be seen by a doctor?

  • What tumor changes will mean I should begin active treatment?

  • Should I have a second magnetic resonance imaging (MRI) or computed tomography (CT) scan to know if the desmoid tumor will shrink?

Questions to ask about having surgery

  • Should I have a biopsy before surgery?

  • Is the goal of the surgery to remove all of the tumor? Why or why not?

  • What is the likelihood that the tumor will recur after surgery?

  • What will happen during this surgery?

  • How long will the operation take?

  • How long will I be in the hospital?

  • How much experience does this surgeon have with desmoid tumors? How many desmoid tumor operations has this surgeon done in the past year?

  • What are the possible side effects of my surgery, both in the short term and long term?

  • Who should I contact about any side effects I experience? And how soon?

  • Do I need any other type of treatment before the surgery? After the surgery?

  • Will I need rehabilitation services after the surgery?

Questions to ask about having radiation therapy

  • What is the goal of radiation therapy?

  • What type of radiation therapy is recommended?

  • How experienced is the radiation oncologist with desmoid tumor? How many people with desmoid tumor has this doctor treated in the past year?

  • How many treatments will I need, and how long will each one take?

  • What side effects can I expect during treatment?

  • Who should I contact about any side effects I experience? And how soon?

  • What are the possible long-term or late effects of having this treatment?

  • What can be done to prevent or to treat side effects?

Questions to ask about having therapies using medication

  • What type of medication is recommended?

  • What is the goal of each medication?

  • How will it be delivered? Through an IV or a pill?

  • Will I receive this treatment at a hospital or clinic? Or will I take it at home?

  • If given by IV, how many treatments will I need? How long will each take?

  • How experienced is the medical oncologist with desmoid tumors? How many people with desmoid tumor has this doctor treated in the past year?

  • Should I consider a clinical trial?

  • What side effects can I expect from each medication?

  • Who should I contact about any side effects I experience? And how soon?

  • What are the possible long-term or late effects of having this type of treatment?

  • What can be done to prevent or relieve side effects?

Questions to ask about planning follow-up care

  • What is the chance that the desmoid tumor will come back? Should I watch for specific signs or symptoms?

  • What long-term side effects or late effects are possible based on the treatment I received?

  • What follow-up tests will I need, and how often will those tests be needed?

  • How do I get a treatment summary and survivorship care plan to keep in my personal records?

  • When should I return to my primary care doctor for regular medical care?

  • Who will be leading my follow-up care?

  • What survivorship support services are available to me? To my family?

The next section in this guide is Additional Resources. It offers more resources on this website that may be helpful to you. Use the menu to choose a different section to read in this guide.

Desmoid Tumor - Additional Resources

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about tumor care and treatment. This is the final page of Cancer.Net’s Guide to Desmoid Tumor. Use the menu to go back and see other pages.

Cancer.Net includes many other sections about the medical and emotional aspects of a tumor for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond.

Here are a few links to help you explore other parts of Cancer.Net:

This is the end of Cancer.Net’s Guide to Desmoid Tumors. Use the menu to choose a different section to read in this guide.