A REVIEW OF EXTRA-ABDOMINAL FIBROMATOSIS TREATED AT THE ROYAL NATIONAL ORTHOPAEDIC HOSPITAL

Abstract

Objective: This study was performed to review the current treatment and outcome of extra abdominal fibromatosis in our hospital, supplemented by a current review of the literature.

Method: A retrospective study of 72 patients with fibromatosis seen at the Royal National Orthopaedic Hospital (RNOH) between 1980 and 2009 was performed. Patients were identified using the databases at the peripheral nerves injury (PNI) unit and the histopathology department. Medical and radiological records were reviewed.

Results: There were 72 patients treated at the Sarcoma and PNI units. 40 patients were primary referrals, and 32 more had operations at the referring hospital. An operation was not carried out in 5 patients. 48 patients were treated by operation alone and this was supplemented by adjuvant therapy in 19 patients. Recurrence was seen in 24 (50.0%) of the operation alone group and 10 (52.6%) 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. We agree with the recent recommendation that these lesions should be treated in regional soft tissue sarcoma units.