Aggressive fibromatosis of the abdominal wall, limbs and limb girdles

Abstract

Abstract Background Aggressive fibromatosis (AF) is a rare soft-tissue tumour. It is histologically benign but locally aggressive and destructive. Surgery is the mainstay of treatment. This was a review of patients treated at the Royal Marsden Hospital between 1986 and 2003. Methods Records of patients with abdominal wall (23) and limb or limb girdle (86) AF were studied to determine patient, tumour and treatment characteristics and outcome. Children and patients with intra-abdominal AF were excluded. The data were analysed by Cox regression to calculate univariate hazard ratios. Results Twenty-one (19·3 per cent) of 109 patients developed recurrence after a median follow-up of 39 months. Recurrence did not correlate with surgical margin or any other variable. Conclusion Wide excisional, function-preserving surgery is the goal in treatment of AF. Surgery for recurrent disease is often curative. Tumours are frequently irresectable at presentation and an initial period of expectant observation is advisable because growth arrest is a common feature of the disease.