Diseases

Fibromatosis – Causes, Symptoms & Treatment

Fibromatose

Fibromatosis is a skin disease characterized by proliferation of connective tissue. The growth, unlike cancer, is often benign. However, as a generalized congenital fibromatosis, fibromatosis can lead to death.

What is fibromatosis?

People suffering from fibromatosis show growths in the collagenous connective tissue, which are classified as neoplastic formations. Neoplastic formations also include cancers and other forms of uncontrolled cell growth. However, fibromatosis does not have the same damaging potential, but in most cases is considered benign or at least only slightly malignant.

However, certain forms of fibromatosis are a fundamental exception. Fibromatosis can manifest itself in different parts of the human body. Depending on the location of the growth, medicine distinguishes different forms of the disease. For example, a growth on the gums is called fibromatosis gingivae .

causes

There are various possible causes for the development of fibromatosis. Various factors often work together. One of the most common forms of fibromatosis is nodular fasciitis .

The proliferation of the tissue can be attributed to inflammation or local violence, among other things. Due to an incorrect reaction, the affected tissue grows incorrectly and thus causes a bulge. The exact background is unclear in many cases.

Symptoms, Ailments & Signs

The main feature of fibromatosis is the proliferation of connective tissue. As a rule, the bulge flows smoothly into the surrounding tissue. Fibromatosis can occur in different forms; they differ mainly in the place of their origin. In practice, it is not always easy to define the different forms precisely.

The superficial fibromatoses can manifest themselves on different parts of the body. Often a person will develop various superficial fibromatoses, for example bulges on the bottom of the feet ( Ledderhose disease ) and nodular fasciitis on the arms.

Another form of fibromatosis is generalized congenital fibromatosis. As the name suggests, it is a congenital disease. It manifests itself in countless tissue proliferations that form knots. These nodules resemble hamartomas. Unlike most fibromatosis, generalized congenital fibromatosis can be fatal.

Aggressive fibromatosis also forms proliferations of collagenous connective tissue. In aggressive fibromatosis, these growths grow very slowly but steadily. In this way, the connective tissue develops into a bulge. Medicine also refers to this type of growth as desmoid.

In aggressive fibromatosis, they appear as extra-abdominal desmoids: they are often found on the trunk and less frequently on the extremities. This distinguishes them from the abdominal desmoids, which can form on the abdomen. Aggressive fibromatosis often recurs and is also called highly differentiated fibrosarcoma because of its appearance.

Diagnose

To diagnose fibromatosis, doctors first look at the appearance of the growth. In contrast to other skin phenomena, fibromatosis is not sharply demarcated from the surrounding tissue. The tissue overgrowth can often be removed with an outpatient procedure.

A subsequent examination of the tissue provides information about whether it is benign or malignant. If the neoplastic formation is found to be malignant, further investigation is often necessary to ensure that the growth has not spread to other organs.

complications

Fibromatosis can cause various complications. In the case of generalized congenital fibromatosis, severe malformations in the tissue can occur. Depending on the position and size of the growths, chronic paralysis, circulatory disorders and nerve damage can occur. In the long term, congenital fibromatosis can lead to death.

If other organs are already affected, various functional disorders can occur, which progress as the tumor grows. Organ failure can occur later . Treating fibromatosis also carries risks. Hair loss, diarrhea , headaches and other typical complications can occur as part of radiation therapy.

Surgical removal of the tissue growth can be accompanied by temporary fatigue and depressive moods. Postoperative bleeding, excessive scarring and the renewed formation of growths in the connective tissue are also typical. In the case of an aggressive fibromatosis, after the removal of a growth, reactions of the immune system and the hormone balance can occur, which sometimes lead to sweating and tachycardia.

In drug therapy, antiestrogens are usually prescribed, which are associated with various side effects. Cycle disorders, hot flashes and discharge are typical of drugs such as tamoxifen . In rare cases, thrombosis, polyps and tumors can also occur.

When should you go to the doctor?

Since fibromatosis can, in the worst case, lead to the death of the person concerned, it must be treated in any case. As a rule, a doctor should always be consulted if there are growths in the tissue. These growths can appear on different parts of the body and cause different skin conditions. In most cases, however, they are easy to recognize because they form large knots. Should these knots form, a doctor must be consulted immediately. The growths rarely appear on the extremities.

Diagnosis and treatment can be done by a dermatologist . The earlier fibromatosis is recognized and treated, the higher the chances of a positive course of the disease. During treatment, the knots are surgically removed, so those affected usually have to stay in the hospital. Radiation therapy may also be necessary. After successful treatment of fibromatosis, the disease can recur, so that a doctor must be consulted again and the disease must be treated.

Treatment & Therapy

In many cases, close observation of the fibromatosis is important. In this way, patients and doctors can see how the growth behaves and whether it is spreading, for example. Doctors can surgically remove the tissue growth. To do this, they remove the superfluous tissue. So that nothing of the proliferating tissue remains and spreads again, surgeons remove the affected tissue relatively generously. However, the fibromatosis often grows back.

Additional radiotherapy can be useful under certain circumstances – for example, if complete removal of the tissue proliferation is not possible or not possible for some reason. The radiation therapy is carried out locally and exposes the affected skin area to high levels of radiation. Under normal circumstances, this is harmful to health because it destroys the cells.

However, this is precisely the effect that is desired in radiation therapy: ideally, the proliferating cells of the tissue die off completely, while the healthy tissue is spared as much as possible. However, just like the surgical removal of fibromatosis, radiation therapy always affects healthy tissue a little.

If doctors have already completely removed the fibromatosis in one operation and there is a high probability that no proliferating tissue will remain, additional radiotherapy is usually not necessary. Some fibromatoses turn out to be malignant and behave like other neoplastic formations, crowding out healthy tissue as cancer.

If the fibromatosis turns out to be malignant, rapid action is often required – otherwise the tissue can metastasize and affect other organs. As a result, the affected organs suffer from functional limitations, which often progress as the tumor grows and can lead to organ failure. However, such forms of fibromatosis are very rare.

Outlook & Forecast

If it is the malignant form of fibromatosis, the disease can be fatal in the worst case. There is a risk that the growths will spread throughout the body and eventually affect the organs. Aggressive fibromatosis can recur years after recovery. The growths then often go unnoticed and are only discovered when the disease is already well advanced.

The affected organs are restricted in their function, which can lead to physical complaints and ultimately to organ failure. Generalized congenital fibromatosis can be fatal if left untreated. If diagnosed and treated early, the chances of recovery are relatively good. The drug treatment is lengthy, but does not result in any health risks for the patient.

If other organs are already affected at the time of treatment, radiation therapy must be carried out. This always represents a considerable burden and can put a considerable physical and mental strain on those affected. A malignant fibromatosis therefore has a rather negative prognosis, while the benign form can usually be treated well. Both forms must be recognized and treated promptly in order to avoid complications.

prevention

The development of fibromatosis is very complex and often ambiguous. Various factors play a role in its development: local violence, genetic aspects and inflammation are difficult to avoid. Avoiding excessive and strong solar radiation as well as environmental toxins could contribute to general prevention.

aftercare

A fibromatosis should primarily be treated directly by a doctor, since the options for aftercare are very limited in this case. In the worst case, this disease can also lead to the death of the person concerned if it is a malignant cancer that spreads throughout the body. For this reason, a regular examination of the body should also always be carried out, even if the fibromatosis has been completely cured.

In most cases, those affected by this disease are dependent on a surgical procedure in which the growths are completely removed. The patient should always rest after an operation and also take care of his body. Radiation therapy or chemotherapy may also be necessary.

It is not uncommon for those affected to be dependent on the support and help of friends and family when treating fibromatosis so that mental disorders or depression do not occur. Contact with other people affected by fibromatosis can also be useful and may also make everyday life easier for those affected. Sometimes the life expectancy of those affected is reduced by the disease.

You can do that yourself

Patients affected by fibromatosis should see a doctor regularly with the condition. In this way, if the growth spreads, the appropriate measures can be taken to avoid further complications.

Fibromatosis generally requires medical attention. This is best supported by rest and bed rest . Sometimes it is possible to support the therapy with an individually tailored diet and physical exercise. In general, it is advisable to avoid excessive and strong solar radiation as well as environmental toxins. Other potentially irritating influences, such as care products or chlorinated water in the indoor pool, should be avoided if possible. The doctor will inform the person concerned of the exact measures to be taken as part of the diagnosis.

Patients who suffer severely from the disease should also visit a support group. Strategies can be learned by talking to other affected people, which make it easier to deal with the disease. If the fibromatosis turns out to be malignant, rapid action is required. Affected persons should have an operation or radiotherapy carried out immediately in order to avoid a severe course and to rule out serious complications.

Lisa Newlon
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Hello! I am Lisa Newlon, and I am a medical writer and researcher with over 10 years of experience in the healthcare industry. I have a Master’s degree in Medicine, and my deep understanding of medical terminology, practices, and procedures has made me a trusted source of information in the medical world.