RESUMO
Desmoid tumours are deep aggressive fibromatoses that usually arise in the soft tissues of the limbs or the abdominal wall. Intra-abdominal localisation, rarely occurs and their treatment may be challenging. When necessary, surgery must be personalized to what is achievable in terms of margins while preserving functional outcomes. This condition is illustrated herein with the case of a 40-year-old female presenting an unusually large sporadic desmoid tumour with abdominal, pelvic and perineal involvement. Resection was performed without organ involvement through a combined perineal approach. Tumour resection was macroscopically completed except in the perineum, where the tumour was left (R2 resection) to preserve anal sphincter. Adjuvant treatment with tamoxifen was given to achieve local control. The hormonal treatment was well tolerated, and no recurrence was observed after 36 months of follow-up.
