Aggressive fibromatosis

Aggressive fibromatosis is a type of musculoskeletal fibromatosis. While it is a non-metastasising fibrous lesion, it is thought to be a true neoplasm that arises from the fascial and musculoaponeurotic coverings, sometimes at the site of a traumatic or post-surgical scar.

The term aggressive fibromatosis is occasionally used synonymously with desmoid tumours by some authors 5. This article will focus in the musculoskeletal presentation of this tumour; for the abdominal presentation, please refer to the latter.

According to one the study, the mean age at presentation is ~40 7.  There may be a slight female predilection (male to female ratio of 1:1.2).

Being non-encapsulated, poorly circumscribed, and infiltrative, aggressive fibromatosis grows insidiously and invades locally. The lesions may become quite large and adhere to neighbouring structures, such as neurovascular bundles. The lesions grossly resemble scar tissue and are composed of well-differentiated fibroblasts embedded in an abundant collagenous matrix with increased cellularity at the periphery. Cytologic features of malignancy are lacking. Its exact cause is unknown.

Associations

Recognised associations include:

Plain radiograph

Radiographs may show a soft-tissue mass, localised periosteal thickening, or direct bony destruction/invasion 4.

MRI

Signal characteristics of the lesion include 7:

  • T1: homogeneously isointense or mildly hyperintense
  • T2/STIR: high heterogeneous signal
  • T1 C+ (Gd): typically enhances avidly
  • GE: peripheral areas of smooth low signal intensity that do not represent calcification (check radiographs) or haemorrhage (check gradient images/scouts for blooming) are characteristic
Angiography

May show arterial stretching, neovascularity, and tumour staining 4.

It does not have any propensity for metastasis although it can aggressively invade structures. The optimal management for aggressive fibromatosis depends on tumour location and extent. Surgical resection may be offered although the likelihood of local recurrence after surgery is high, particularly if margins are positive. Moderate-dose radiotherapy alone for gross disease or after a microscopically incomplete resection yields local control rates of approximately 75-80% 3. Treatment with pharmacologic agents results in objective response rates of approximately 40-50%.

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Article information

rID: 17235
Tag: cases
Synonyms or Alternate Spellings:
  • Aggressive fibromatosis (AF)

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