Norton SM, McDermott A, Sehgal R and O’Donoghue G
Introduction: Desmoid fibromatosis (DF) or Desmoid tumours are rare, benign musculoaponeurotic tumours. DF have no potential for metastatic spread but are locally aggressive and spread along fascial planes, leading to extensive patient morbidity. While germline Adenomatous Polyposis Coli (APC) mutations are associated with DF, it can also occur sporadically. Debate regarding the most appropriate treatment options exists in literature.
Case summary: A 38 year old lady presented with a left hypochondrial swelling, night sweats and weight loss to the General Surgical Outpatients Department at our institution. This was noticed after the birth of her second child. No relevant family history was noted. A pre-operative ultrasound demonstrated a fusiform nodule within the left anterior abdominal transversalis oblique muscle, which had a focal hypervascular nidus. Surgical excision of the mass was performed under general anaesthetic and histologically the tumour was found to contain spindled myofibroblastic cells arranged in fascicles with perivascular lymphoid infiltrates. The diagnosis was DF with a positive margin status. Given the high risk of recurrence of this tumour type, the decision was made to undertake a further resection.
Conclusion: DF is known to be a locally aggressive benign tumour of mesenchymal origin. The benefits and disadvantages of treatment options are debated within literature. Surgery is reported to have better or similar local control rates to radiotherapy but without radiation related complications. Given that DF is known to be locally aggressive, affecting young people and with a female preponderance, we believe that surgical excision with clear margins is the most appropriate treatment of choice.
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