The Fibromatosis /Desmoid Tumour Site

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The purpose of this website is to present information about a medical condition known as  fibromatosis.  One type of fibromatosis is also known as aggressive fibromatosis or desmoid tumour.  Aggressive fibromatosis is a rare condition and there is relatively little information about it on the internet.  After being diagnosed with this condition myself, I decided that an information page with simple, non-technical information about the condition was needed.
Please note that I am not a member of the medical profession and I do not recommend any specific course of treatment for this condition.  Please consult your doctor. 
Please also note that I have no control over the advertising that appears at the top of the webpage.  The ads are placed there by the web page hosting company and I have nothing to do with it.

I've put together some of the questions that I had about fibromatosis, and the information I was able to find out.

What is Aggressive Fibromatosis?
Aggressive fibromatosis is a rare type of non-malignant tumour.  Aggressive fibromatosis is also known as a 'desmoid' tumour.  Some forms of fibromatosis can be locally aggressive (fast-growing and difficult to get rid of). 
Is it a type of cancer?
Well..... sort of.  The World Health Organization classifies desmoid tumours as somewhere in between truly benign tumours and the malignant tumours. Desmoids are not truly benign because they are locally aggressive, but they do not metastasize like malignant cancers.  The bottom line is that whatever you call them, desmoids can behave like cancer.
Is it serious?  Will it kill me?
Reviews of cases of aggressive fibromatosis have found 5-year survival rates are over 90%.  People with desmoid tumours rarely die from the tumours themselves.  In a group of studies with a total of 424 patients, there were only 5 reported tumour deaths.
However, major surgery can be required to treat this form of tumour, depending on its size and location when it is diagnosed.  Surgery, radiation and chemotherapy also have their own risks and chances of complications developing.  Tumours growing on vital structures such as major blood vessels, organs, major nerves or the brain will be more difficult to treat without risking damage to surrounding tissues.  Rarely, if a tumour does not respond to other treatment methods, amputation of the affected limb may be the only way to stop tumour growth.

Is this a malignant tumour?

 No, there is no evidence of metastasis (i.e., tumour cells do not migrate and start new tumours elsewhere in the body).


How rare is aggressive fibromatosis?

Aggressive fibromatosis has been estimated as making up 0.03% of all tumours.



What causes aggressive fibromatosis?

 Causes of this tumour are still under investigation.  In some forms, they appear to be inherited as several family members may be diagnosed with them.  Some tumours develop in surgical scars after surgery.  Desmoid tumours may also develop in association with pregancy.  Adult-onset desmoids are mostly found in women of childbearing age, but the disease can occur in both sexes, at any age.


Researchers have discovered a number of different mutations within desmoid tumour cells.  However, what causes the mutations is not known.


Can children get this disease?

Children have been diagnosed with several types of fibromatosis, including infantile myofibromatosis, which affects soft tissue, internal organs and bone, juvenile aponeurotic fibroma, which affects the hands and feet, juvenile hyaline fibromatosis which affects the skin, and infantile desmoid type fibromatosis which affects the musculature.


What is FAP?

FAP stands for familial adenomatous polyposis.  It is a rare genetic condition that results in hundreds of polyps forming in the colon (large intestine).  Colon cancer from one or more of those polyps is considered inevitable unless the colon is surgically removed with a colostomy.  The risk of desmoid tumors in individuals with FAP is 852 times the risk in the general population.  Desmoid tumours usually develop in the abdomen of people with FAP.


Where do desmoid tumours occur in the body?

Desmoids occur in limbs (69%) (girdle area), in the neck (19%), the trunk (12%) or the abdomen (2%). They may also occur in the jaw, head or eye.


Is this a fast-growing tumour?

Sometimes.  Some tumours grow very quickly, and others can stay the same size for months or even years. If the tumour is very slow growing and is not causing any other problems, sometimes doctors choose to just monitor it carefully and defer treatment.


How is aggressive fibromatosis treated?

The current methods of treating aggressive fibromatosis usually involve surgical removal of the tumour, often followed by radiation to kill any remaining tumour cells.  Chemotherapy and treatments with hormones or a drug called Tamoxifen may also be used.  Desmoid tumours are very individual in their responses to treatment, and no one treatment alone seems to have a high success rate.  What works for one person may not work at all for another.  Often, more than one treatment type is needed (i.e. surgery + radiation, or surgery + chemo, or chemo + radiation).  If the tumour is growing on or near vital structures, your doctor may choose not to attempt surgery but to use radiation or chemotherapy alone.


Check the 'Links' page for links to medical articles on the treatment of desmoids. 


This is not a malignant tumour - why is radiation sometimes used to treat it?

 Desmoid tumours are very prone to local recurrence - after being surgically removed, they have a nasty tendency to grow back in the same area.  Your doctor may use radiation or chemotherapy to lower the chances of recurrence. 


Will this tumour grow back?

Recurrence of desmoid tumours has been found to occur in 50% of cases, on average, 3 years after the original diagnosis.  It may recur multiple times.  Desmoid tumours in the head and neck recur, on average, in 70% of cases.


Tumours can also recur after being treated with radiation.  In those cases, the tumour usually recurs at the edge of or outside of the radiation field.


I've been diagnosed with aggressive fibromatosis.  What should I do?

Discuss your treatment options with your doctor.  Just make absolutely sure that your doctor is aware how often these tumours recur, and that a follow-up schedule such as a periodic MRI is developed so your doctor can check if your tumour is growing back.  An MRI to check for recurrence may be warranted as early as 3 months or less after surgical removal, if your tumour is particularly fast-growing.  There are links to medical articles elsewhere on this site if you or your doctor want more detailed information.  You may wish to ask your doctor to refer you to a specialist with experience in treating desmoid tumours, as the condition is rare enough that most general practitioners never see it.   In Canada, you can ask for a referral to the closest Cancer Centre.