An In-Depth Review of Desmoid Tumours

Dear Colleagues,

Desmoid tumour or desmoid-type fibromatosis is defined as clonal fibroblastic soft tissue neoplasms, characterized by aggressive, infiltrative growth and a tendency toward local recurrence but an inability to metastasize. The incidence rate is 2–4 cases per million population per year, making it a rare tumour type. However, given its presentation at a younger age (median: fourth decade of life), lack of metastatic potential and low mortality rate (<1%), most patients with desmoid tumours are followed long-term, making its prevalence rate significantly higher. Therefore, the affected patient population makes up a sizable proportion of those seen in soft tissue tumour cancer clinics and are often discussed repeatedly at multidisciplinary case conferences.

Desmoid tumours occur more frequently in females and are heterogeneous in terms of presentation, anatomic location, associated conditions and clinical behaviour. A major change in initial management has been a shift away from surgical resection to active surveillance strategies. This approach is successful in 50–60% of patients, permitting the majority to avoid potential surgical complications and functional loss. Success is usually defined as a lack of tumour growth and severe symptoms under active surveillance.

Despite this paradigm shift in initial management, unfortunately, 30–40% of tumours progress on active surveillance and/or have significant symptoms; combined, 40–50% fail this strategy. The next steps in management are variable depending on the tumour location, patient factors and differing tumour groups’ expertise. At present, the majority of management strategies and the order of these is based primarily on expert consensus guidelines and retrospective series. There is a paucity of high-level prospective or randomized evidence; the rarity of desmoid tumours and their heterogeneous behaviour present major challenges in study design. Unfortunately, although it is a growing area of study, there is even less information available on functional outcomes or quality of life for patients afflicted by this disease.

In this Special Issue, we invite original studies and review articles on the molecular pathogenesis and heterogeneous presentation of patients with desmoid tumours, potential predictors of success—such as imaging characteristics—with active surveillance, and therapeutic options (surgery, radiation, hormonal, chemotherapy and minimally invasive or percutaneous approaches) for those who fail active surveillance strategies. This Special Issue also encourages papers exploring more difficult patient populations, including when desmoid tumours present in pregnancy or evaluating aspects related to functional outcomes or the quality of life of patients with desmoid fibromatosis. 

Dr. Lloyd Mack
Guest Editor

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