Fibromatosis

Author: Vanessa Ngan, Staff Writer, 2006.


Fibromatosis — codes and concepts
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What is fibromatosis?

Fibromatosis is a condition where fibrous overgrowths of dermal and subcutaneous connective tissue develop tumours called fibromas. These fibromas are usually benign (non-cancerous).

What is the classification of fibromatosis?

There appear to be many different ways to classify fibromatosis. One classification system used is based on age (i.e.: juvenile vs adult fibromatoses) and localisation (ie, superficial vs deep fibromatoses).

JuvenileAdult
Superficial (fascial) fibromatoses
Deep (musculoaponeurotic) fibromatoses
  • Desmoid tumours (aggressive fibromatoses)
    • Extra-abdominal fibromatosis
    • Abdominal fibromatosis
    • Intra-abdominal fibromatosis (eg, pelvic fibromatosis)

See also elastofibroma pathology.

What are the features of fibromatosis?

The following table lists the distinguishing features between superficial and deep fibromatoses.

Superficial fibromatosesDeep fibromatoses
  • Slow growing tumour
  • Small size
  • Arise from fascia or aponeurosis
  • Less aggressive
  • Rapidly growing pseudotumour
  • Usually, they reach a large size
  • Often involve deeper structures (muscles of the trunk and extremities)

Whilst most fibromatoses are benign tumours and do not metastasise (spread to other parts of the body), the desmoid tumours although they do not metastasise like malignant cancers can be locally aggressive. They can grow quickly into large tumours that can obstruct vital structures such as major blood vessels, nerves and organs.

Fibromatosis

What causes fibromatosis?

The cause of fibromatosis remains unclear. In some types of fibromatosis such as desmoid tumours, it is thought that the condition may be related to trauma, hormonal factors, or have a genetic association. Superficial fibromatoses such as palmar, plantar and penile fibromatosis have sometimes been linked to certain diseases such as diabetes, liver disease and hypertension.

What is the treatment for fibromatosis?

Management of fibromatosis depends on individual disease. For further information on the different types of fibromatoses select from the list below.

 

Related information

 

References

  • Book: Textbook of Dermatology. Ed Rook A, Wilkinson DS, Ebling FJB, Champion RH, Burton JL. Fourth edition. Blackwell Scientific Publications.

On DermNet NZ

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