Fibromatosis (ICD code: M72) is a proliferation of collagenous connective tissue. Connective tissue is loose to taut tissue consisting of
- interstitial cell mass
- fatty tissue and
- collagen
It also includes the supporting and protective sheaths around muscles and organs.
Fibromatoses can occur in many different parts of the body. Like cancers, they are neoplastic diseases. Neoplastic means that disordered, independent and excessive tissue growths occur.
The proliferation caused by fibromatosis is benign, i.e. no malignant cancer cells are present. Malignant cells can form metastases that spread to other organs. Degeneration, i.e. a change from a benign growth to a malignant tumor, is also very rare.
However, fibromatosis sometimes grows very aggressively and ulcer-like. It can grow into the surrounding tissue and damage important structures there. For example, the following can be affected
In many cases, myofibroblasts are involved in the development of fibromatosis. Myofibroblasts are cells that are classified between muscle cells (myoblasts) and cells of the connective tissue (fibroblasts).
Structure of the skin in layers - the connective tissue is located as intermediate tissue in the dermis and subcutis © bilderzwerg / Fotolia
There are various possible causes for the development of fibromatosis. Possible causes include inflammation or injury. The connective tissue tries to repair the local tissue damage, but forms too many cells and growths occur.
In many cases, however, there is no clear explanation for the development of fibromatosis. There are some fibromatoses that are congenital or can be inherited.
Fibromatoses are generally differentiated according to the part of the body where they occur. For example, it can be a superficial or a deep fibromatosis. There are a large number of fibromatoses, and a complete treatment of all subtypes is not possible within the scope of this article and would go beyond its scope. This is due to the fact that connective tissue can occur in almost any part of the body and therefore the number of fibromatoses and different forms of manifestation is extremely large.
Are all fibromatoses visible from the outside?
Fibromatoses are only visible from the outside if they have formed in the superficial tissues. This includes the skin in particular, but also the layers directly beneath the skin (subcutaneous tissue) as well as mucous membranes and body orifices. Fibromatoses that have formed in deeper structures are not visible from the outside and are only diagnosed by corresponding clinical complaints and symptoms.
Fibromatoses are therefore divided into superficial and deep manifestations.
What are superficial fibromatoses?
Superficial fibromatoses include the following clinical pictures:
- Ledderhose disease (plantar fibromatosis of the sole of the foot)
- Dupuytren's disease (fibromatosis in the palm of the hand)
- Peyronie's disease (induratio penis plastica, connective tissue disease of the penis)
- Desmoid tumor (aggressive fibromatosis on the sheaths = fasciae of the muscles)
- Fibromatosis colli (often occurs in small children on the neck and can result in torticollis )
- Nodular fasciitis (lump formation of the upper extremities)
- Jones syndrome (fibromatosis of the gums)
What are deep fibromatoses?
Deep fibromatoses include the following diseases, among others:
- Sclerosing mediastinitis (a hardening inflammation of the connective tissue in the mediastinum of the chest cavity)
- Sclerosing mesenteritis (inflammation in the small intestine, affecting the connective tissue interspersed with fatty tissue)
- Ormond's disease (retroperitoneal fibromatosis, a connective tissue growth in the posterior abdominal cavity and lower back)
Ledderhose disease with characteristic nodule on the sole of the foot © Farantsa | AdobeStock
Different symptoms occur depending on the location of the fibromatosis.
Superficial fibromatoses often develop on the underside of the feet or in the palms of the hands. The accompanying symptoms can then be directly felt and often also seen. The main symptoms are
- a feeling of pressure or tension
- pain and
- skin irritation
occur. Small or larger lumps can also develop. The tissue changes cannot be clearly distinguished from the surrounding tissue, but are clearly visible.
Nodular fasciitis is diagnosed very frequently. This is a form of superficial fibromatosis and usually manifests itself in the formation of individually growing nodules in the area of the muscle sheaths (fascia). This often results in small, tumor-like nodules on the upper extremity.
Symptoms of deep fibromatosis depend on the location in the body:
- Digestive problems with fibromatosis in the abdomen
- Back pain and kidney damage in fibromatoses in the lower posterior abdomen (Ormond's disease).
- Shortness of breath with fibromatosis in the chest area
The diagnosis of fibromatosis can be difficult under certain circumstances. The symptoms can sometimes also indicate other diseases, so that further examinations are still necessary in the event of conspicuous skin findings.
In addition to the blood test, this also includes examining the suspicious tissue structures under the microscope. This requires a tissue sample(biopsy), from which a histological determination of the growths is then made. During the examination, the structure and composition of the tissue is assessed. This usually makes it possible to determine whether fibromatosis is present and what type of disease it is.
If fibromatosis is strongly suspected or confirmed, the tissue growth is then surgically removed. This removed tissue is then also prepared for histological examination by cutting it into small pieces and thin slices. The specialists (pathologists) then determine whether it is a benign or malignant tumor and what degree of malignancy the growth has.
In medical terminology, malignancy refers to the malignancy of a disease. A finding with a higher degree of malignancy means that the disease has a greater potential to destroy the organism.
Rapid treatment is therefore essential for the patient. Fibroblastic-myofibroblastic tumors, for example, but also malignant ulcers can occur. In the case of a malignant tumor, however, the doctor does not speak of fibromatosis, but of fibrosarcoma, depending on the findings.
If malignant cells are discovered, further examinations are usually necessary. The blood and the function of the internal organs are checked, also to detect possible pre-existing conditions. Using imaging, such as
must almost invariably be used to check whether the growth has spread, i.e. formed metastases.
The treatment of fibromatosis depends on which variant is involved. In many cases, the connective tissue proliferation can initially be observed. Of particular interest here is whether and how quickly the proliferation grows, as well as the degree of pain.
Surgical removal may be advisable if the pain increases or for cosmetic reasons, and is highly recommended if the growth increases rapidly. The diseased tissue is cut out relatively generously in order to remove it safely and completely.
The diseased tissue must be completely removed. If individual cells or cut edges remain, the disease often recurs (recurrence).
If it is not possible to remove all of the diseased tissue, radiotherapy can be helpful. Radiotherapy is carried out locally, i.e. only at the site of tissue proliferation, and aims to destroy the diseased cells. However, as with surgical removal, this can often result in additional damage to healthy tissue. However, this is often necessary in order to prevent recurrence.