Extra-abdominal desmoid tumour of the breast: review of the primary management and the implications for breast reconstruction.

This case report illustrates the presentation and management of an extra-abdominal desmoid tumour of the breast. A review of the literature describing the aetiology, pathology and risk of recurrence was undertaken to determine how current understanding of this rare tumour may affect the management of patients, should they require breast reconstruction after radical excision of the primary tumour. The natural progression of the disease is variable and there are no markers predictive of recurrence or regression. Primary lesions should be assessed with respect to their anatomical site of origin (i.e. whether they arise within the breast or invade the breast from the underlying musculo-aponeurotic tissue) and the extent of local invasion. Radical excision of the tumour with clear histological margins decreases the likelihood of recurrence. Tumours arising from the musculo-aponeurotic system have increased risks of recurrence and of developing multifocal primary tumours in specific anatomical territories. Local recurrences should be assessed for extent and anatomical distribution, and radical excision performed as for a primary tumour. Radiotherapy can be used as an alternative treatment if radical excision of a primary or recurrent tumour would cause severe functional loss or mutilation. Radiotherapy can be used for positive histological margins following tumour excision. There is a higher risk of recurrence in the first 3 years after primary excision, and breast reconstruction may be best delayed for this period. Surgical trauma has been implicated in the aetiology of recurrence and the patient should be informed of this prior to breast reconstruction. Copyright 2001 The British Association of Plastic Surgeons.