Aggressive Fibromatosis


Also found in: Dictionary, Encyclopedia, Wikipedia.

Aggressive Fibromatosis

A common (50% of fibroproliferative lesions of musculoaponeuroses of childhood), slowly infiltrative, non-tender, non-metastasising, but locally aggressive myofibroblastic neoplasm of younger (mean age 40) adults that is the most common tumour of mesentery. It arises in the stomach, GI tract, or abdomen and may extend into liver, pancreas, or retroperitoneum, as well as in deep soft tissues (shoulder, pelvic girdle, chest wall, trunk, back, legs, head and neck, breast and spermatic cord) of children to young adults.
Clinical findings Obstruction of bowel, ureter, and blood vessels; fistula formation.
Management Surgery with wide margins, but not always possible or successful if attempted. Radiotherapy may or may not help. Chemotherapy (imatinib/Glivec/Gleevec) or endocrine therapy may or may not help
Prognosis Post-excisional recurrences range from 20–90%; successful therapy requires adequate margins. Young age at diagnosis, mesenteric location and Gardner’s syndrome carry a higher risk of recurrence.
References in periodicals archive ?
Aggressive fibromatosis of the breast: a case report and literature review.
Differential considerations of adenomyoepitheliom: Lobulated-shape, well-circumscribed solid mass on mammography * fibroadenoma * malignant myoepithelioma * spindle-cell carcinoma * aggressive fibromatosis * other myofibroblastic lesions
1) They include a number of tumors with similar characteristics, such as fibromas, aggressive fibromatosis, rhabdosarcomas, and fibrosarcomas.
Aggressive fibromatosis has virtually no metastatic potential.
These features suggested a diagnosis of aggressive fibromatosis.
Aggressive fibromatosis presents as a painless mass, creating vascular and neurologic signs of pressure.
Aggressive fibromatosis (non-familial desmoid tumour): Therapeutic problems and the role of adjuvant radiotherapy.
We describe two cases of aggressive fibromatosis of the parapharyngeal space, and we review the available treatment options.
2) Only two cases of aggressive fibromatosis of the parapharyngeal space have been reported previously.
The pathology of the excised tumor was consistent with aggressive fibromatosis in that dense fibrous tissue focally infiltrated between the muscle fibers (figure 1, B).
On pathologic analysis, the tumor was consistent with aggressive fibromatosis (figure 2, B).

Full browser ?