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-ab dominal desmoid tumour of the breast. A review of the literature describing the aetiology, pathology and risk of recurrence was undertaken to determin e how current understanding of this rare tumour may affect the management o f patients, should they require breast reconstruction after radical excisio n of the primary tumour. The natural progression of the disease is variable and there are no markers predictive of recurrence or regression. Primary l esions 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 und erlying musculo-aponeurotic tissue) and the extent of local invasion. Radic al excision of the tumour with clear histological margins decreases the Lik elihood of recurrence. Tumours arising from the musculo-aponeurotic system have increased risks of recurrence and of developing multifocal primary tum ours in specific anatomical territories. Local recurrences should be assess ed for extent and anatomical distribution, and radical excision performed a s for a primary tumour. Radiotherapy can be used as an alternative treatmen t if radical excision of a primary or recurrent tumour would cause severe f unctional loss or mutilation. Radiotherapy can be used for positive histolo gical margins following tumour excision. There is a higher risk of recurren ce in the first 3 years after primary excision, and breast reconstruction m ay be best delayed for this period. Surgical trauma has been implicated in the aetiology of recurrence and the patient should be informed of this prio r to breast reconstruction. (C) 2001 The British Association of Plastic Sur geons.