Desmoid tumours are a rare fibroblastic proliferation of monoclonal origin, arising in deep soft-tissues. Histologically, they are characterized by locally aggressive behaviour and an inability to metastasize, and clinically by a heterogeneous and unpredictable course. Desmoid tumours can occur in any anatomical site, but commonly arise in the limbs. Despite their benign nature, they can be extremely disabling and sometimes life-threatening, causing severe pain and functional limitations. Their surgical management is complex and challenging, due to uncertainties surrounding the biological and clinical behaviour, rarity, and limited available literature. Resection has been the first-line approach for patients with a desmoid tumour but, during the last few decades, a shift towards a more conservative approach has occurred, with an initial ‘wait and see’ policy. Many medical and regional forms of treatment are also available for the management of this condition, and others have recently emerged with promising results. However, many areas of controversy remain, and further studies and global collaboration are needed to obtain prospective and randomized data, in order to develop an appropriate shared stepwise approach.

Cite this article: Bone Joint J 2023;105-B(7):729–734.

Purpose of the study: Extra-abdominal desmoids tumours are benign tumours generally managed by more or less wide surgical resection. This surgery can be mutilating and carries a risk of recurrence to the order of 50% according to several authors. Material and methods: We followed a series of 26 patients from 1989 to 2009 with non-operated extra-abdominal desmoids tumours. Our objective was to study the clinical, radiographic and pathological course of these tumours and identify prognostic factors. For 11 patients, no medical or surgical treatment was delivered beyond the diagnostic biopsy; for 15, the tumour recurred after surgery and no other adjuvant treatment was given. Results: Twenty-four tumours stabilised at 13.2 months on average for primary tumours and 20.9 for recurrent tumours. Thus en general, the tumour progression was less than 3 years. In all cases, once the tumour stopped progressing, it did not progress again. One primary tumour regressed spontaneously and one recurrence continued to progress at last follow-up of 23 months. Surgery was not required for any of the patients, excepting biopsy procedures to confirm diagnosis. MRI was the exploration of choice for follow-up. Discussion: This series, which is the largest devoted to followed extra-abdominal desmoid fibromas, confirms recent data in the literature. A wait-and-see attitude should always be discussed with this type of tumour

Desmoid tumours are not common but have a distinct resemblance to fibrosarcomata. Their clinical appearance and progress should be recognised since failure to distinguish them from sarcomata could result in extensive and unnecessarily mutilating operations. Three cases of extra-abdominal desmoid tumours are reported. Two of the patients had tumours arising from multicentric foci in the same limb. The disappearance of the tumours in two patients after the menopause, and the variation in the tumours during the menstrual cycle in the third patient, add weight to the theories about endocrine control

1. Two patients with multicentric extra-abdominal desmoid tumours are reported. Multicentric foci have not been described previously. 2. Both cases conform to the usual clinical, macroscopic and microscopic criteria of desmoid tumours in all other regards. 3. Their occurrence in a single limb bud suggests a congenital propensity and leads to the advocacy of radical surgery in such cases