Aggressive Fibromatosis


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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 ?
One retrospective analysis of patients with aggressive fibromatosis during a 5-year period (2008 to 2012) concluded that in the treatment of aggressive fibromatosis, preservation of form and function should be given greater priority in all age groups.
Aggressive fibromatosis (desmoid tumor) is amonoclonal disorder.
Matrix metalloproteinase activity modulates tumor size, cell motility, and cell invasiveness in murine aggressive fibromatosis.
Magnetic resonance imaging in aggressive fibromatosis.
Head-and-neck aggressive fibromatosis usually occurs in children under 20 years of age.
Differential considerations of adenomyoepitheliom: Lobulated-shape, well-circumscribed solid mass on mammography * fibroadenoma * malignant myoepithelioma * spindle-cell carcinoma * aggressive fibromatosis * other myofibroblastic lesions
Thus the histological features favored the final diagnosis of aggressive fibromatosis over inflammatory myofibroblastic tumor.
1) They include a number of tumors with similar characteristics, such as fibromas, aggressive fibromatosis, rhabdosarcomas, and fibrosarcomas.
Aggressive fibromatosis often recur after surgery, particularly after marginal or intralesional excision.
Aggressive fibromatosis (non-familial desmoid tumour): Therapeutic problems and the role of adjuvant radiotherapy.
INTRODUCTION: Aggressive fibromatosis is a rare condition marked by the presence of desmoids tumors, which are benign, slow growing musculo- aponeurotic tumors without any metastatic potential.
We describe two cases of aggressive fibromatosis of the parapharyngeal space, and we review the available treatment options.

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