Fibromatosis

Fibromatosis General Information 

Fibromatosis is a soft tissue benign tumor that sometimes has a very (highly) aggressive course (just like various other benign tumors). This medical condition is different from neurofibromatosis (a dominant genetic disorder that determines the appearance of tumors along different types of nerves). This one, for example, has many local recurrences. The patients suffering from Fibromatosis complain of extreme pain and can remain disfigured. This happens if the tumor is not completely excised. These types of tumor have many characteristics; for example, the clinical and cytological malignant features are absent, the clinical behavior is always extremely aggressive and they present a growth pattern that is described as infiltrative. In Fibromatosis, the fibroblasts are very well differentiated and often proliferate. When these tumors have the tendency of infiltrating very deep into the patient’s skeletal muscles, specialists refer to them as musculoaponeurotic tumors. As for desmoid tumors, they are present in a pregnant woman, on the walls of her abdomen. This medical disorder develops in patients with Gardner's syndrome as multiple fibromatoses. This terrible disease has no predilection for a specific gender although it is more common in women who gave birth and occurs mainly in young adults and children, being rarer among the middle-aged persons. This disease has a worldwide distribution and it affects about 0.05 of the globes population. More prone to develop it are those persons who present polyposis of the colon (part of the large intestine). Because of its aggressive effects, it is of high importance that the condition is treated on time.

Fibromatosis Symptoms

Fibromatosis Treatment

If you have been diagnosed with Fibromatosis, you should first have an open talk with your health care provider. Because these tumors often recur with major problems, it is very important to know all the available prophylaxis options. Your doctor should develop a follow-up schedule (for example a periodic MRI) so that he could regularly check if the tumor can or does come back. Fibromatosis is extremely rare so that you should make sure that your doctor is well informed in what concerns your disease. To be more sure and if you feel it necessary, you are allowed to ask your health care provider to consult with a specialist who is more experienced in dealing with this kind of medical conditions and who can offer you more detailed information. Most of the general health care providers do not have the necessary studies to accurately diagnose this particular medical condition. We strongly advise you to go to the closest cancer center in your town. The diagnosis is established after a biopsy of the tumor is performed. Through a surgical procedure, the medical professional will remove a small sample of the tumor. Some of the doctors also prefer to perform CT scans (computerized tomography), MRI (magnetic resonating images) or X-rays once the medical examination is completed. Once the diagnosis of Fibromatosis is set up, the specialist must quickly proceed with the appropriate treatment.

The preferred method of dealing with Fibromatosis is the surgery which consists in the removal of the tumor. Any therapy against this type of tumor contains radiations or a prompt wide excision that includes a thin margin of the adjacent healthy tissue. The radiation is usually used after the surgery in order to kill the tumor cells that may have remained after the operation. The local behavior of Fibromatosis is quite aggressive and the rate or recurrence is over 15%. Any recurrences of the medical condition are treated with re-excision. Although the effects of this type of tumor on a patient’s life are sometimes devastating because of the infiltrative and very aggressive behavior, these tumors rarely lead to death; the risk is either minimal or even nonexistent. Chemotherapy is also applied, as well as treatments with drugs (especially tamoxifen) and hormones. The response to all these methods of treatment varies from tumor to tumor because this disease is individualized in what concerns the response to a certain therapy. Doctors can not tell for sure to a patient how long would it take until he is completely cured or even if the therapy will give any results. Not always a treatment that has given good responses to a patient has the same result in another patient. Most patients are generally prescribed a combination of treatments, for example: surgery and chemotherapy, chemotherapy and radiation, surgery and radiation or all these three together. Ask your physician for further information.