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 that arises from the fascial and musculo-aponeuroticmusculoaponeurotic coverings, sometimes at the site of a traumatic or post-surgical scar.

Terminology

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.

Epidemiology

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

Pathology

Being non-encapsulated, poorly circumscribed, and infiltrative, aggressive fibromatosis fibromatosis grows insidiously and invades locally. The lesions may 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 fibroblasts embedded in an abundant collagenous matrix with increased increased cellularity at the periphery. Cytologic features of malignancy are lacking. Its exact cause is unknown.

Associations

Recognised associations include:

• surgical or accidental trauma
• pregnancy
• oestrogenic hormone use
• Gardner syndrome
• familial adenomatous polyposis

Radiographic features

Plain filmradiograph

Radiographs may show a soft-tissue mass, localised periosteal thickening, or frankdirect bony destruction/invasion⁴.

MRI

Signal charactersiticscharacteristics of the lesion include ⁷:

• T1: homogeneous iso-intensity or mild hyperintensity
• T2/STIR: high heterogenous high signal
• T1 C+ (Gd): typically enhances avidly
• GE: peripheral areas of smooth low/low 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.

Treatment and prognosis

It does not not have any propensity for metastasis although can aggressively invade structures. The optimal management for aggressive fibromatosis depends on tumortumour location and extent. Surgical resection may be offered although the likelihood 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%.

See also

• WHO 2002 classification of soft tissue tumours