What is Fibromatosis?
Fibromatosis is a term that indicates a set of soft tissue tumors that share specific characteristics, among which stand out a rather aggressive behavior that manifests itself with frequent recurrences, but the absence of malignancy; these are well-differentiated tumors that do not form metastasisalthough it must be said that desmoid tumors can be locally aggressive, even pressing dangerously on organs and other nearby structures (including blood vessels and nerves).
Fibromatosis, regardless of its form and location, is characterized by the appearance of fibroids, benign tumors that are the result of excessive fibrous growth of the dermal and subcutaneous connective tissue.
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Classification
The most common form of classification of fibromatosis is based on the age of onset and, secondly, on the depth of the affected tissue (source MyP table):
| Youth | adult | |
| Superficial forms | deep form | |
| Congenital generalized fibromatosis (myofibromatosis) | Palmar fibromatosis (Dupuytren’s disease) and plantar fibromatosis (Ledderhose disease) | Desmoid tumors:
|
| Fibroma aponeurotico | Penile fibromatosis (Peyronie’s disease) | |
| Infantile digital fibromatosis | Garrod Bearings | |
| Aggressive infantile fibromatosis | Dermatofibroma | |
| Neck fibromatosis | Nodular fasciitis | |
| Lenticular dermatofibrosis (Buschke-Ollendorf syndrome) | Elastofibroma | |
However, the difference between superficial and deep fibroids goes beyond mere location, because:
- surface forms have
- slow growth
- small size
- limited aggression
- while the deep forms
- show rapid growth,
- reach large sizes
- they often involve deeper structures (such as muscles in the trunk and extremities).
With regard to the type of lesion, it is then possible to make a distinction into:
- soft fibroids (composed mostly of cells)
- hard fibroids (composed mainly of fibers).
Note that the term fibroma generally indicates a benign tumor, unlike fibrosarcoma which is instead the malignant form.
Cause
As with nearly all cancers, the exact cause is unclear; if for some forms (such as desmoid tumors) specific events are hypothesized (such as trauma or hormonal changes, such as pregnancy) that act on a genetic predisposition, for other forms it is more difficult to make hypotheses.
For superficial fibromatoses such as palmar, plantar, and penile fibromatosis, an association with other health conditions is often seen, such as diabetesliver disease and high pressure.
Treatment
Treatment is mainly surgical, but highly variable depending on the location and shape of the fibroid.