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{{Desmoid tumor}}
{{CMG}} {{AE}}{{S.M.}}
==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?==
*[[Desmoid tumor]]<nowiki/>s grow from the [[connective tissue]] in your [[body]]
*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
*[[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]]
*[[Desmoid tumor]]<nowiki/>s are often found in the [[abdomen]]
*They can also be found in the [[shoulders]], upper [[arm]]<nowiki/>s, and [[thighs]]
*[[Desmoid tumor]]<nowiki/>s are [[benign]], which means they are not [[cancer]]
*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 tumor]]<nowiki/>s can grow slowly or very quickly
*The quicker they grow the more serious they are 
*[[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?==
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 tumor]]<nowiki/>s have [[symptoms]], but others don’t
*[[Symptoms]] can include:
**[[Pain]]
**[[Swelling]] in the area of the [[tumor]]
**[[Sleep]] loss
*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 (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]]
==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 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
*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 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
**'''Radiation therapy'''
***[[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
**'''Chemotherapy'''
***There is no [[standard]] [[chemotherapy]] for [[desmoid tumor]]<nowiki/>s
***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 tumor]]<nowiki/>s 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 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 (biology)|cells]]
*Too much [[beta-catenin]] can cause [[Cells (biology)|cells]] to grow when they shouldn’t
*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 tumor]]<nowiki/>s
==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 tumor]]<nowiki/>s 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 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
*[[Desmoid tumor]]<nowiki/>s 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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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

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