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==Overview==
==Overview==
==Patient information==
==Patient information==
Desmoid tumors (also called aggressive fibromatosis) are benign, slowly growing fibroblastic neoplasms with no metastatic potential but a propensity for local recurrence, even after complete surgical resection. Despite being histologically benign, they are locally infiltrative and can cause death through destruction of adjacent vital structures and organs. (See 'Introduction' above.)
What is desmoid tumor?
Desmoid tumors grow from the connective tissue in your body. They grow from fibroblast cells, which make up connective tissue and are also important for wound healing. Connective tissue supports and connects your bones, ligaments, and muscles, and helps hold your organs in place. Desmoid tumors are also known as aggressive fibromatosis or desmoid-type fibromatosis.


●The risk of desmoids is increased in patients with familial adenomatous polyposis (FAP). Given the association with FAP, at some institutions, colonoscopy is recommended for all patients with desmoid tumors, particularly intraabdominal. (See 'FAP and Gardner syndrome' above.)
A desmoid tumor can occur anywhere in the body since connective tissue is found everywhere in your body. Desmoid tumors are often found in the abdomen. They can also be found in the shoulders, upper arms, and thighs.


●Treatment of desmoids can be a clinical challenge, particularly in patients with FAP who tend to develop intraabdominal desmoids at sites of prior surgery. Because of their locally aggressive behavior and tendency to relapse with more aggressive disease, multimodality treatment is often required for these benign lesions and is best delivered within the context of a multidisciplinary team specializing in sarcoma treatment.
Desmoid tumors are benign, which means they are not cancer. While the cells of the desmoid tumor do not travel to parts the body like cancer can, they can invade nearby tissue and are often very painful. Desmoid tumors can grow slowly or very quickly. The quicker they grow the more serious they are. Desmoid tumors can be difficult to completely remove with surgery.


●Extraabdominal and abdominal desmoids tend to occur in women during or following pregnancy. If a woman had a desmoid arise during a prior pregnancy and the desmoid was resected, the recurrence risk during future pregnancies is low. If woman had an existing desmoid (pregnancy-associated or predating any pregnancy) that was managed with watchful waiting, that desmoid can grow during a subsequent pregnancy, but not always. The risk to the pregnancy is very low, and the course of the pregnancy may be relatively normal. (See 'Pregnancy' above.)
Desmoid tumors are most common in people between the ages of 15 and 60 years. They are more common in females than males.


Treatment — The natural history of desmoid tumors is prolonged, variable, and unpredictable, and it is not clear that any intervention improves survival. (See 'Natural history' above.)
How common is desmoid tumor?
Out of one million people worldwide, two to four are diagnosed with a desmoid tumor per year.


Extraabdominal and abdominal wall tumors
How is desmoid tumor diagnosed?
Some people with desmoid tumors have symptoms, but others don’t. Symptoms can include pain, swelling in the area of the tumor, sleep loss, or difficulty moving. For people without symptoms, these tumors can be discovered if the person has an imaging test for another reason.


●Observation is an appropriate option in asymptomatic patients who may be reliably followed. If desmoids remain unchanged or shrink, observation may be continued. If the tumor increases in size or becomes symptomatic, if there is imminent risk to adjacent structures, or if the desmoid creates cosmetic concerns, treatment should be pursued. (See 'Initial observation' above.)
Imaging: If you have symptoms of a desmoid tumor, your doctor may use imaging scans such as CT, MRI, or ultrasound to see where the tumor is and how big it is.


●We suggest complete surgical excision as the treatment of choice for a potentially resectable extraabdominal (extremity, trunk, breast) or abdominal wall desmoids in a patient who is medically able to tolerate surgery and if resection can be accomplished without major functional or cosmetic deficit (Grade 2B). Controversy remains as a number of patients will fare well without surgical intervention, so a more conservative approach appears rational for relatively static lesions. (See 'Surgery' above.)
Biopsy: To check if the tumor is a desmoid tumor your doctor will do a biopsy, taking a small amount of the tumor out with a needle. An expert, called a pathologist, will study the cells under the microscope to see what kind of tumor it is. Depending on where the tumor is in the body, it may be difficult for the doctor to take a biopsy.


●Primary radiation therapy (RT) is an appropriate option for patients who need treatment but are not good surgical candidates, those who decline surgery, and those for whom surgical morbidity would be excessive. An alternative approach is initiation of systemic treatment in these patients, especially if they are young and there are concerns about the potential for late toxicity from RT. (See 'Radiation therapy' above.)
How is desmoid tumor treated?
Treatment for each patient will be unique. You should go to an expert in sarcoma treatment to decide the best approach for your tumor. You can contact MyPART for help finding experts near you.


●We recommend not pursuing adjuvant RT in patients with microscopically negative surgical margins, regardless of tumor size (Grade 1C). We suggest not pursuing adjunctive RT following resection of a primary desmoid tumor with microscopically positive margins (Grade 2C). Since fewer than 50 percent of these patients will develop a recurrence, patients who remain progression-free will be spared the long-term sequelae of RT. (See 'Postoperative RT' above.)
Desmoid tumors can be hard to predict. They can shrink and go away on their own, they can remain the same size, or they can grow quickly.


●For gross residual disease of a primary desmoid, we suggest observation or RT rather than more radical resection (Grade 2B). Due to the very high local control rates with RT, additional surgery is warranted only if there is strong confidence in obtaining clear margins, and functional loss is minimal and acceptable. Systemic therapy is also effective in controlling extremity desmoids in a number of patients, precluding the need for further surgery.
Treatment options to discuss with your doctor include:


●For a recurrent desmoid tumor, our preference is observation or surgical resection. However, systemic therapy or RT alone are reasonable alternative treatments in selected patients who are thought to have a higher morbidity from repeat operation and an increased probability of positive margins. For patients who undergo surgery for a recurrent desmoid, we suggest observation or postoperative RT if the margins are positive (Grade 2C). (See 'Management of recurrent disease' above.)
Watch and wait: In some cases, the tumor grows very slowly, or even shrinks without any treatment. In this case it may be safest for your doctor to check your tumor regularly without treating it.


Intraabdominal desmoid, Gardner syndrome
Surgery: Surgery has been a standard treatment for desmoid tumors in the past, but this may be changing. Because the tumor often returns to the same location after surgery, doctors are looking for other treatment options.


●For patients who have large intraabdominal desmoid tumors, particularly in the setting of Gardner syndrome, surgery is still a standard approach for resectable tumors. However, the infiltrative nature of the desmoid in this situation often precludes surgery, and the surgical margins are often positive. Medical therapy in lieu of surgery is a viable option for patients with more difficult tumors such as those involving the mesentery, major vessels, or other critical structures. (See 'Intraabdominal desmoids' above and "Desmoid tumors: Systemic therapy".)
Radiation therapy: Radiation therapy is a treatment option for some desmoid tumors. Because radiation therapy can cause other cancers in the future, it is important to discuss with your doctor.


●Surgery with or without RT is an appropriate option if there is no response to medical therapy. In this situation, consideration should be given to use of intraoperative electron beam therapy as a component of the treatment [138].
Chemotherapy: There is no standard chemotherapy for desmoid tumors. But, promising new drugs have been shown to shrink these tumors


●Management of recurrence of an intraabdominal tumor in patients with Gardner syndrome is challenging because recurrences tend to become more frequent and aggressive with each surgical intervention. Most of these patients are managed with systemic therapy rather than additional local measures. (See 'Management of recurrent disease' above and "Desmoid tumors: Systemic therapy".)
It is important to discuss these different options with your doctor and seek second opinions if possible.


Posttreatment surveillance
Do desmoid tumors run in families?
In 5% to 10% of cases, desmoid tumors may run in families. Gardner syndrome  is a rare syndrome that runs in families. People with this syndrome have a high risk of colorectal cancer and desmoid tumor.


●There is no standard protocol for follow-up of patients with desmoid tumors. However, we typically follow patients by clinical examination and radiographic studies (where appropriate) every six months for the first three years, every 12 months to year 6, and then biannually. (See 'Posttreatment surveillance' above.)
How does desmoid tumor form?
Scientists are always working to understand how tumors form, but it can be hard to prove. We know that some people with a family history of desmoid tumors have a change in a gene called adenomatous polyposis coli, or APC. This change causes too much of a protein called beta-catenin in parts of your cells. Too much beta-catenin can cause cells to grow when they shouldn’t. In some desmoid tumors, there is too much beta-catenin, even though APC is normal.


APC and beta-catenin are important in many common cancers, such as colon cancer, and scientists are trying to use this information to develop better cancer treatments, which will help people with desmoid tumors.


What is the prognosis for someone with a desmoid tumor?
The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as:


Where the tumor is in your body
How fast the tumor grows
How much of the tumor was taken out during surgery
If you want information on your prognosis, it is important to talk to your doctor. NCI also has resources to help you understand cancer prognosis.
Doctors estimate desmoid tumor survival rates by how groups of people with desmoid tumors have done in the past. Because there are so few desmoid tumor patients these rates may not be very accurate. They also don’t take into account newer treatments being developed.
Most desmoid tumors don’t change how long you will live, but they are very difficult to get rid of and can be painful to live with. Desmoid tumors growing in the abdomen can cause problems, such as blocking your intestines. It is important that your doctor monitor the growth of these tumors carefully.
==Source==
NATIONAL CANCER INSTITUTE - CANCER.GOV https://www.cancer.gov/nci/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor




Revision as of 16:32, 4 March 2019

Desmoid tumor Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Desmoid tumor from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]Faizan Sheraz, M.D. [3]

Overview

Patient information

What is desmoid tumor? Desmoid tumors grow from the connective tissue in your body. They grow from fibroblast cells, which make up connective tissue and are also important for wound healing. Connective tissue supports and connects your bones, ligaments, and muscles, and helps hold your organs in place. Desmoid tumors are also known as aggressive fibromatosis or desmoid-type fibromatosis.

A desmoid tumor can occur anywhere in the body since connective tissue is found everywhere in your body. Desmoid tumors are often found in the abdomen. They can also be found in the shoulders, upper arms, and thighs.

Desmoid tumors are benign, which means they are not cancer. While the cells of the desmoid tumor do not travel to parts the body like cancer can, they can invade nearby tissue and are often very painful. Desmoid tumors can grow slowly or very quickly. The quicker they grow the more serious they are. Desmoid tumors can be difficult to completely remove with surgery.

Desmoid tumors are most common in people between the ages of 15 and 60 years. They are more common in females than males.

How common is desmoid tumor? Out of one million people worldwide, two to four are diagnosed with a desmoid tumor per year.

How is desmoid tumor diagnosed? Some people with desmoid tumors have symptoms, but others don’t. Symptoms can include pain, swelling in the area of the tumor, sleep loss, or difficulty moving. For people without symptoms, these tumors can be discovered if the person has an imaging test for another reason.

Imaging: If you have symptoms of a desmoid tumor, your doctor may use imaging scans such as CT, MRI, or ultrasound to see where the tumor is and how big it is.

Biopsy: To check if the tumor is a desmoid tumor your doctor will do a biopsy, taking a small amount of the tumor out with a needle. An expert, called a pathologist, will study the cells under the microscope to see what kind of tumor it is. Depending on where the tumor is in the body, it may be difficult for the doctor to take a biopsy.

How is desmoid tumor treated? Treatment for each patient will be unique. You should go to an expert in sarcoma treatment to decide the best approach for your tumor. You can contact MyPART for help finding experts near you.

Desmoid tumors can be hard to predict. They can shrink and go away on their own, they can remain the same size, or they can grow quickly.

Treatment options to discuss with your doctor include:

Watch and wait: In some cases, the tumor grows very slowly, or even shrinks without any treatment. In this case it may be safest for your doctor to check your tumor regularly without treating it.

Surgery: Surgery has been a standard treatment for desmoid tumors in the past, but this may be changing. Because the tumor often returns to the same location after surgery, doctors are looking for other treatment options.

Radiation therapy: Radiation therapy is a treatment option for some desmoid tumors. Because radiation therapy can cause other cancers in the future, it is important to discuss with your doctor.

Chemotherapy: There is no standard chemotherapy for desmoid tumors. But, promising new drugs have been shown to shrink these tumors

It is important to discuss these different options with your doctor and seek second opinions if possible.

Do desmoid tumors run in families? In 5% to 10% of cases, desmoid tumors may run in families. Gardner syndrome is a rare syndrome that runs in families. People with this syndrome have a high risk of colorectal cancer and desmoid tumor.

How does desmoid tumor form? Scientists are always working to understand how tumors form, but it can be hard to prove. We know that some people with a family history of desmoid tumors have a change in a gene called adenomatous polyposis coli, or APC. This change causes too much of a protein called beta-catenin in parts of your cells. Too much beta-catenin can cause cells to grow when they shouldn’t. In some desmoid tumors, there is too much beta-catenin, even though APC is normal.

APC and beta-catenin are important in many common cancers, such as colon cancer, and scientists are trying to use this information to develop better cancer treatments, which will help people with desmoid tumors.

What is the prognosis for someone with a desmoid tumor? The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as:

Where the tumor is in your body How fast the tumor grows How much of the tumor was taken out during surgery If you want information on your prognosis, it is important to talk to your doctor. NCI also has resources to help you understand cancer prognosis.

Doctors estimate desmoid tumor survival rates by how groups of people with desmoid tumors have done in the past. Because there are so few desmoid tumor patients these rates may not be very accurate. They also don’t take into account newer treatments being developed.

Most desmoid tumors don’t change how long you will live, but they are very difficult to get rid of and can be painful to live with. Desmoid tumors growing in the abdomen can cause problems, such as blocking your intestines. It is important that your doctor monitor the growth of these tumors carefully.

Source

NATIONAL CANCER INSTITUTE - CANCER.GOV https://www.cancer.gov/nci/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor

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