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{{Desmoid tumor}}
{{Desmoid tumor}}
{{CMG}} {{AE}}{{S.M.}}{{Faizan}}
{{CMG}} {{AE}}{{S.M.}}
==Overview==
==Overview==
[[Desmoid tumor]] commonly develops by division of [[myofibroblast]] [[Cells (biology)|cells]] in the [[fibrous]] ([[Connective tissue|connective) tissue]] of the [[body]] that forms [[tendons]] and [[ligaments]], usually in the [[arm]]<nowiki/>s, [[legs]], [[abdomen]], [[head]] and [[neck]] or any part of the [[body]]. These [[tissues]] of the [[body]] connect, support, and surround other body parts and [[organs]].
==What is desmoid tumor?==
==What is desmoid tumor?==
*Desmoid tumors grow from the connective tissue in your body.
*[[Desmoid tumor]]<nowiki/>s 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.
*They grow from [[fibroblast]] [[Cells (biology)|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.
*[[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.
*[[Desmoid tumor]]<nowiki/>s are also known as [[aggressive fibromatosis]] or [[Desmoid tumor|desmoid]]-type [[fibromatosis]]
*A desmoid tumor can occur anywhere in the body since connective tissue is found everywhere in your body.
*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.
*[[Desmoid tumor]]<nowiki/>s are often found in the [[abdomen]]
*They can also be found in the shoulders, upper arms, and thighs.
*They can also be found in the [[shoulders]], upper [[arm]]<nowiki/>s, and [[thighs]]
*Desmoid tumors are benign, which means they are not cancer.
*[[Desmoid tumor]]<nowiki/>s 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.
*While the [[Cells (biology)|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.
*[[Desmoid tumor]]<nowiki/>s 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.  
*The quicker they grow the more serious they are
*Desmoid tumors are most common in people between the ages of 15 and 60 years. They are more common in females than males.
*[[Desmoid tumor]]<nowiki/>s can be difficult to completely remove with [[surgery]]  
*[[Desmoid tumor]]<nowiki/>s 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?==
==How common is desmoid tumor?==
Out of one million people worldwide, two to four are diagnosed with a desmoid tumor per year.
Out of one million people worldwide, two to four are diagnosed with a [[desmoid tumor]] per year


==How is desmoid tumor diagnosed?==
==How is desmoid tumor diagnosed?==
*Some people with desmoid tumors have symptoms, but others don’t.
*Some people with [[desmoid tumor]]<nowiki/>s have [[symptoms]], but others don’t  
*Symptoms can include:
*[[Symptoms]] can include:
**Pain
**[[Pain]]
**Swelling in the area of the tumor
**[[Swelling]] in the area of the [[tumor]]
**Sleep loss
**[[Sleep]] loss
*Difficulty moving
*Difficulty [[moving]]
*For people without symptoms, these tumors can be discovered if the person has an imaging test for another reason.
*For people without [[symptoms]], these [[tumors]] can be discovered if the person has an [[imaging]] test for another reason


==Imaging==
==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.
*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==
==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  
*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  
*An expert, called a [[pathologist]], will study the [[Cells (biology)|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
*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?==
==How is desmoid tumor treated?==
*Treatment for each patient will be unique.
*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 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.
*You can contact MyPART for help finding experts near you
*Desmoid tumors can be hard to predict.
*[[Desmoid tumor]]<nowiki/>s 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.
*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:
*Treatment options to discuss with your doctor include:
**'''Watch and wait'''
**'''Watch and wait'''
***In some cases, the tumor grows very slowly, or even shrinks without any treatment.
***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.
***In this case it may be safest for your doctor to check your [[tumor]] regularly without treating it
**'''Surgery'''
**'''Surgery'''
***Surgery has been a standard treatment for desmoid tumors in the past, but this may be changing.
***[[Surgery]] has been a standard treatment for [[desmoid tumor]]<nowiki/>s 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.
***Because the [[tumor]] often returns to the same location after [[surgery]], doctors are looking for other treatment options
**'''Radiation therapy'''
**'''Radiation therapy'''
***Radiation therapy is a treatment option for some desmoid tumors.
***[[Radiation therapy]] is a treatment option for some [[desmoid tumor]]<nowiki/>s
***Because radiation therapy can cause other cancers in the future, it is important to discuss with your doctor.
***Because [[radiation therapy]] can cause other [[cancers]] in the future, it is important to discuss with your doctor
**'''Chemotherapy'''
**'''Chemotherapy'''
***There is no standard chemotherapy for desmoid tumors.
***There is no [[standard]] [[chemotherapy]] for [[desmoid tumor]]<nowiki/>s
***But, promising new drugs have been shown to shrink these 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.
*It is important to discuss these different options with your doctor and seek second opinions if possible


==Do desmoid tumors run in families?==
==Do desmoid tumors run in families?==
*In 5% to 10% of cases, desmoid tumors may run in families.
*In 5% to 10% of cases, [[desmoid tumor]]<nowiki/>s may run in families  
*Gardner syndrome  is a rare syndrome that runs 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.
*People with this [[syndrome]] have a high risk of [[colorectal cancer]] and [[desmoid tumor]]


==How does desmoid tumor form?==
==How does desmoid tumor form?==
*Scientists are always working to understand how tumors form, but it can be hard to prove.
*[[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.
*We know that some people with a [[family history]] of [[desmoid tumor]]<nowiki/>s 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.
*This change causes too much of a [[protein]] called [[beta-catenin]] in parts of your [[Cells (biology)|cells]]
*Too much beta-catenin can cause cells to grow when they shouldn’t.
*Too much [[beta-catenin]] can cause [[Cells (biology)|cells]] to grow when they shouldn’t  
*In some desmoid tumors, there is too much beta-catenin, even though APC is normal.
*In some [[desmoid tumor]]<nowiki/>s, 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.
*[[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 tumor]]<nowiki/>s


==What is the prognosis for someone with a desmoid tumor?==
==What is the prognosis for someone with a desmoid tumor?==
*The estimate of how a disease will affect you long-term is called prognosis.
*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:
*Every person is different and [[prognosis]] will depend on many factors, such as:
 
**Where the [[tumor]] is in your [[body]]
==Where the tumor is in your body==
**How fast the [[tumor]] grows
*How fast the tumor grows
**How much of the [[tumor]] was taken out during [[surgery]]
*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  
*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]]
*NCI also has resources to help you understand cancer prognosis.
*Doctors estimate [[desmoid tumor]] [[survival rates]] by how groups of people with [[desmoid tumor]]<nowiki/>s have done in the past  
*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
*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
*They also don’t take into account newer treatments being developed.
*Most [[desmoid tumor]]<nowiki/>s don’t change how long you will live, but they are very difficult to get rid of and can be [[painful]] to live with  
*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 tumor]]<nowiki/>s growing in the [[abdomen]] can cause problems, such as blocking your [[intestines]]
*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
*It is important that your doctor monitor the growth of these tumors carefully.


==Source==
==Source==

Latest revision as of 20:12, 5 March 2019

Desmoid tumor Microchapters

Home

Patient Information

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]

Overview

Desmoid tumor commonly develops by division of myofibroblast cells in the fibrous (connective) tissue of the body that forms tendons and ligaments, usually in the arms, legs, abdomen, head and neck or any part of the body. These tissues of the body connect, support, and surround other body parts and organs.

What is desmoid tumor?

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?

Imaging

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

Do desmoid tumors run in families?

How does desmoid tumor form?

What is the prognosis for someone with a desmoid tumor?

Source

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