Connective tissue cancer is an umbrella term for both benign and malignant tumors of the connective tissue. They develop from special connective tissue cells, the fibroblasts, which are physiologically responsible for building connective tissue. Thus, when these cells degenerate, unimpeded production and proliferation of connective tissue occurs. Depending on whether the cancer is benign or malignant one speaks of either a fibroma or a fibrosarcoma. Benign fibroids can be further subdivided into hard fibromas or soft fibromas.
The exact cause of the development of connective tissue cancer is not yet known. One possibility for the development of a benign fibroma is a mistake in the embryonic development of body tissue.
With regard to malignant fibrosarcomas, further, following causes are discussed:
Contact with chemicals, such as weedkillers or dioxins, which may be produced by the incineration of household waste, may be the cause of the development of malignant fibrosarcomas.
Also found are malignant connective tissue tumors in certain genetic diseases such as neurofibromatosis type 1 (Recklinghausen disease) or Li-Fraumeni syndrome. After radiation therapy in the context of cancer, the increased occurrence of, inter alia, fibrosarcomas was also observed.
There is also the possibility that a benign fibroid develops into a malignant fibrosarcoma; However, this is very rare and mostly associated with genetic diseases such as Morbus Recklinghausen. Infants rarely develop a form of congenital conjunctival cancer.
The diagnosis of a fibroma is made clinically, usually by the dermatologist (dermatologist), to whom the patients have turned because of a visually striking, usually painless skin change.
If there is a suspicion of malignancy, a tissue sample is taken during the surgical removal (biopsy) and then examined. Imaging of fibrosarcoma is followed by imaging techniques such as computed tomography (CT) or magnetic resonance imaging (MRI) to further assess the tumor.
First, connective tissue cancer is characterized by a painless, enlarged area under the skin.
Benign fibroids also have specific locations on the body. Soft fibromas are particularly common in the neck, armpit, groin, and women below the breast. They are skin colored.
On the other hand, hard fibroids are often found around the legs and look darker due to embedded color pigments. Hard fibroids can be identified by the so-called Fitzpatrick sign, in which the affected area is compressed with the thumb and forefinger, whereupon it then sinks into the skin. In the course of their development, fibroids terminate their further growth and retain their existing size.
Among 10% of cases of connective tissue tumors are malignant fibrosarcomas. They are more difficult to detect and are often diagnosed as incidental findings as part of a tissue sample (biopsy). Like fibroids, fibrosarcomas are also painless for a long time, but gradually increase in size over time. From a size of 5 cm you should think of a fibrosarcoma. In the case of very large tumors, functional failures of the affected part of the body may also occur.
For benign fibroids no further action is needed. In otherwise healthy patients with no preexisting genetic disease, there is no risk that the fibroma will malignantly change. If the affected area of the skin is found to be disturbing to the patient, the fibroma can be surgically removed. This is done by the dermatologist in a small, outpatient procedure.
In the case of a malignant fibrosarcoma, surgical treatment is necessary. The sarcoma must be surgically removed on a large scale, as this type of cancer tends to grow again in certain areas, so-called local recurrences. Chemotherapy may be used prior to surgery to reduce the size of the tumor before removal, thus improving the postoperative outcome. Both before and after surgery, radiotherapy can also be given.
Since the benign fibroma requires no therapy, the prognosis is accordingly good.
In the case of malignant fibrosarcoma, the chances of recovery depend on how far the tumor has already grown in the patient's body. If there are no daughter tumors (metastases), the tumor has not grown large or could be reduced in size by therapy before surgery, the chance of completely removing the cancer is good.
However, the fibrosarcoma also rapidly forms metastases. If these are already present, complete healing is usually no longer possible.