[Agressive fibromatosis]

Aggressive fibromatosis is a rare soft tissue tumour with a high tendency to local recurrence. The patients often present with a deeply sited, firm mass with or without pain. Genetic, physical and endocrine factors seem to be important in the development of the disease. Patients with Gardner's syndrome and familial adenomatous polyposis have a higher risk of developing aggressive fibromatosis and often develop tumours in the mesentery, retroperitoneally or in the abdominal wall. Infantile fibromatosis is a variant of aggressive fibromatosis. The standard treatment of aggressive fibromatosis is surgical resection with wide free margin, in some cases supplemented with radiotherapy. Chemotherapy and hormonal treatment of non-resectable tumours has been described without convincing results and more data from clinical studies are needed.