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 years of age 7. There may be 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.
Recognised associations include:
- surgical or accidental trauma
- oestrogenic hormone use
- Gardner syndrome
- familial adenomatous polyposis
Radiographs may show a soft-tissue mass, localised periosteal thickening, or direct bony destruction/invasion 4.
Signal characteristics of the lesion include 7:
- T1: homogeneous iso-intensity or mild hyperintensity
- T2/STIR: high heterogenous 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
May show arterial stretching, neovascularity, and tumour staining 4.
Treatment and prognosis
It does not have any propensity for metastasis although 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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- 8. Mcdonald ES, Yi ES, Wenger DE. Best cases from the AFIP: extraabdominal desmoid-type fibromatosis. Radiographics. 28 (3): 901-6. doi:10.1148/rg.283075169 - Pubmed citation
- 9. Murphey MD, Ruble CM, Tyszko SM et-al. From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 2009;29 (7): 2143-73. doi:10.1148/rg.297095138 - Pubmed citation