Desmoid tumours are rare clonal proliferations that arise from mesencyhmal cells.  These tumours do not metastasise but are locally aggressive and their growth may lead to significant morbidity.  Their clinical course is both variable and unpredictable; tumours may rapidly progress while in other instances may remain stable or regress without intervention.  

Aims: The aim of this review is to examine the current treatment of these rare tumours and to specifically develop an evidence-based algorithm to assist with decision-making at the time of presentation.

Methods: A literature search was conducted of MEDLINE and Cochrane databases for published studies over the last 20 years (1995 to July 2015) using the search terms fibromatosis aggressive, desmoid with drug therapy, radiation therapy, prevention and control, radiotherapy, surgery and therapy.  Articles were categorized as surgery, radiation, surgery + radiation, systemic therapy and front line observation.  Articles were included if they reported a retrospective or prospective comparative or observational study with an analysed sample size of 10 patients or more with confirmed diagnosis of desmoid tumour and described one of the following clinical outcomes: relapse-free or progression-free survival, local control rate, response rate.

Results:   416 articles were reviewed, following screening for eligibility 43 were included in final evaluation.  Systemic agents with demonstrated response include cytotoxic chemotherapy, non-steroidal anti-inflammatory agents, hormonal manipulation and molecularly-targeted agents. Disease control rates were highly variable. A recent trend was noted towards upfront observation.

Conclusions:  Decision making in this rare tumour is complicated by the range of treatment options available.Careful counseling at a referral center is essential to the management of desmoid tumours.  We propose a treatment algorithm to assist clinicians in selecting an upfront management plan, we believe that this should be individualized, and specifically incorporate the option of careful observation with subsequent intervention in the case of progression.