Fibromatosis

A Fibromatosis is a skin disease that is characterized by overgrowth of connective tissue. In contrast to cancer, the growth is often benign. As a generalized congenital fibromatosis, however, fibromatosis can lead to death.

What is fibromatosis?

People who suffer from fibromatosis have growths in the collagenous connective tissue that are part of the neoplastic formations. The 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.

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 differentiates between different forms of the disease. For example, a growth on the gums is what is known as fibromatosis gingivae.

causes

Various causes can be considered for the development of fibromatosis. Different factors often work together. One of the most common forms of fibromatosis is nodular fasciitis.

The overgrowth of the tissue can be due to inflammation or local violence. Due to an incorrect reaction, the affected tissue grows incorrectly and thereby causes a bulge. In many cases the exact background is unclear.

Symptoms, ailments & signs

The main feature of fibromatosis is the overgrowth of connective tissue. As a rule, the bead flows smoothly into the surrounding tissue. Fibromatosis can come in several forms; they differ mainly in the place of their origin. The exact delimitation of the various forms does not always turn out to be easy in practice.

The superficial fibromatoses can manifest themselves in different parts of the body. Often times, a person will develop various superficial fibromatoses, such as bulges on the bottom of the feet (Ledderhose's disease) and nodular fasciitis on the arms.

Another form of fibromatosis is generalized congenital fibromatosis. As the name suggests, this is a congenital disease. It manifests itself in innumerable tissue growths that form knots. These nodes resemble hamartomas. Unlike most fibromatoses, generalized congenital fibromatosis can lead to death.

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

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

diagnosis

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

A subsequent examination of the tissue reveals whether it is benign or malignant. If the neoplastic formation is found to be malignant, further examinations are often required to ensure that the growth has not yet spread to other organs.

Complications

Fibromatosis can cause various complications. In generalized congenital fibromatosis, severe tissue malformations 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 during radiation therapy.

Surgical removal of the tissue growth can be associated with temporary fatigue and depressive moods. Post-bleeding, excessive scarring and renewed growth in connective tissue are also typical. In the case of aggressive fibromatosis, the removal of an overgrowth can lead to reactions of the immune system and the hormonal balance, which sometimes lead to sweating and palpitations.

In drug therapy, antiestrogens are usually prescribed, which are associated with various side effects. Menstrual cycle disorders, hot flashes and discharge are typical of drugs like tamoxifen. In addition, thromboses, polyps and tumors can rarely occur.

When should you go to the doctor?

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

Diagnosis and treatment can be done by a dermatologist. The earlier the fibromatosis is recognized and treated, the higher the chances of a positive course of the disease. During the treatment, the nodes are removed by an operation, so that those affected are mostly still dependent on a stay in hospital. Further radiation therapy may also be necessary. After successful treatment of the fibromatosis, the disease can recur, so that you must see a doctor again and treat the disease.

Doctors & therapists in your area

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, for example, it is spreading. Doctors can surgically remove the tissue growth. To do this, they take out the excess tissue. So that nothing of the overgrown tissue remains and spreads again, surgeons remove the affected tissue relatively generously. However, the fibromatosis often grows back again.

Additional radiation therapy can prove to be useful under certain circumstances - for example if the complete removal of the tissue growth is not possible or did not succeed for any reason. The radiation therapy takes place locally and exposes the affected skin area to high levels of radiation. Under normal circumstances this is harmful because it destroys the cells.

In radiation therapy, however, exactly this effect is desired: in the ideal case, 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 the 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 remained, additional radiation therapy is usually not necessary. Some fibromatoses turn out to be malignant and behave like other neoplastic formations which, as cancer, displace healthy tissue.

If the fibromatosis turns out to be malignant, quick action is often required - otherwise the tissue can metastasize and affect other organs.As a result, the affected organs suffer from functional restrictions, 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 as well. Aggressive fibromatosis can recur years after recovery. The growths then often go unnoticed and are only discovered when the disease is 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 lead to death 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 sick person.

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 be a considerable physical and mental strain on the person concerned. 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 to avoid complications.

prevention

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

Aftercare

Fibromatosis should first and foremost be treated directly by a doctor, as the options for follow-up care are very limited in this case. In the worst case, this disease can also lead to the death of the person affected if it is a malignant cancer that spreads throughout the body. For this reason, a regular examination of the body should always be carried out, even if the fibromatosis has been completely cured.

In most cases, those affected with this disease are dependent on an operation 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 have to rely on the support and help of friends and family in the treatment of fibromatosis so that there is no psychological upset or depression. Contact with other people affected by fibromatosis can also be useful and possibly make everyday life easier for those affected. Sometimes the disease reduces the life expectancy of the person affected.

You can do that yourself

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

Fibromatosis generally requires medical treatment. This can best be supported by rest and bed rest. Sometimes it is possible to support the therapy with an individually tailored diet and exercise. In general, it is advisable to avoid excessive and strong solar radiation and environmental toxins. Other potentially irritating influences, for example care products or water containing chlorine in the indoor pool, should be avoided if possible. As part of the diagnosis, the doctor will inform the person concerned which precise measures are to be taken.

Patients who are severely affected by the condition should also seek out a support group. In talking to other sufferers, strategies can be learned that make dealing with the disease easier. If the fibromatosis proves to be malignant, rapid action is required. Sufferers should immediately have an operation or radiation therapy to avoid a severe course and to rule out serious complications.