An Introduction to Aggressive Fibromatosis

Aggressive Fibromatosis is also referred to as a “Desmoid tumour”, Desmoid tumours are a rare type of benign tumour, however just because it’s not malignant does not mean it’s an easy ride, however hopefully this information and this site will help to both educate you and support you through your diagnosis.  Desmoid tumours are aggressive, meaning they can enter and even take over surrounding tissue, muscle and even in some cases bone. The difference between Desmoid tumours and Cancer is the ability to metastasize, meaning that unlike Cancer, Desmoid tumours do not spread throughout the body but instead can reoccur and create destruction in the affected area. Desmoid tumours tend to occur most in the limbs, the neck or the abdomen; however there have been cases of tumours in other areas of the body.  What causes these tumours is still currently unknown, however there are varying reasons that have been shown to be a cause to these types of tumours, for example they can be passed down from a previous generation, they could also occur as a result of a scar from a previous or recent trauma, cases have also suggested an association with pregnancy.

How AF is diagnosed

Usually a lump can be felt externally through the skin, the next step after that is often an ultrasound scan which determines if the mass is solid, after that a CT or MRI scan can be used to see if the tumour is attached to any muscles or bones and this is also used to work out if surgery could safely remove it from the body. Finally a biopsy is usually taken, there are different types of biopsy, some take a large amount of the tumour whereas some just take a small sample.

Treatment Options

From what I have experienced so far, I have been told there are possible routes to now choose to go down, all with their own benefits and risks. The first option is surgery and this is the only option that removes the tumour, hopefully in its entirety,  the surgery option is however subject to a “window of surgical opportunity” in other words, there may only be a certain period of time in which the surgeons are confident of completely and safely removing the tumour, any changes in circumstance could affect how confident the surgeons are and therefore surgery may not always be a viable option. The main drawback with choosing the surgery is the high reoccurrence rate if any residual tumour is left behind.
The second option is Radiation Therapy, this is considered a highly effective option for those patients who have been told that surgery is not an option, and it is also used in conjunction with either chemotherapy or surgery as a way to further destroy the tumour cells. Typically radiation treatment is scheduled for 6 to 8 weeks however this can be, and for many patients has been extended.
Another option is to refrain from any medical treatment at all, some studies have shown that eventually the tumour can just burn itself out and stabilise, some patients even report remission. I have discussed a report on this technique in the “Recent Publications and Research” section of this website and I have included the link for the original report.
Yet another option would be Chemotherapy,   this technique is sometimes used when patients do not respond to the medical treatment available (usually Tamoxifen and Sulindac.) Low doses of chemotherapy have shown response from tumours, more so in younger patients and children. More aggressive desmoid tumours however can be treated with a higher dose of chemotherapy. Chemotherapy, as mentioned above, can be used in conjuction with other available options. I will be aiming to discuss a report on the effectiveness of chemotherapy on desmoid tumours in the near future.
The last option is drug treatment, the two main drugs prescribed are Tamoxifen and Sulindac, these drugs aim to stabilise the tumour in order to restrict any further growth, tumour shrinkage is not named as an aim with these drugs, however some patients have reported a decrease in size after taking these on a regular basis.