The outcome of extra-abdominal fibromatosis treated at a tertiary referral centre

Abstract Purpose To review the outcomes of patients with extra-abdominal fibromatosis treated at a tertiary referral centre. Methods A retrospective review of a series of 72 patients with fibromatosis treated at the Royal National Orthopaedic Hospital (RNOH) between 1980 and 2009, with a median follow up of 4 years (1–17 years). Results Forty patients were primary referrals, and 32 more had operations at the referring hospital. Five were treated non-operatively; 48 patients were treated by operation alone and 19 patients underwent surgery supplemented by adjuvant therapy. Recurrence was seen in 24 of the operation alone group and 10 in the operation and adjuvant therapy group. The rate of recurrence was lower with complete excision. However, complete excision was impossible in some cases because of extension into the chest or spinal canal, or involvement with the axial vessels and lumbosacral or brachial plexus. Conclusion We suggest that operative excision should seek to preserve function and that supplementary adjuvant therapy may reduce the risk of recurrence, although excision margin appears to be the most important factor. The aggressive, infiltrative behaviour of deep fibromatoses and the associated genetic mutations identified, clearly distinguish them from the superficial fibromatoses and makes their treatment more difficult and dangerous, especially where vital structures are involved.