In this E-Learning module, the author comprehensively elaborates on clinical presentation, biology and treatment landscape of desmoid tumours (DT), that are rare, locally aggressive, and invasive soft-tissue tumours challenging to manage due to their variable clinical presentation, unpredictable disease course, and lack of approved therapies.
In the first part of the module, the author elaborates on the epidemiology of desmoid tumours with 5 cases diagnosed per one million of the population per year. In terms of incidence, the author underlines that 5-10% of desmoid tumours are associated with Gardner syndrome, while 90-95% are sporadic fibromatosis/desmoid tumours. Furthermore, the author presents the sites of desmoid tumours occurrence and explains the clinical presentation.
The author goes on to present a treatment landscape for desmoid tumours, indications for treatment and treatment individualisation to optimise tumour control and improve symptoms. Treatment options are explained from the aspects of efficacy and safety, and supported by findings from the clinical studies. The author also provides a summary of ongoing trials for the treatment of patients with desmoid tumours.
In the next part of the module, the author illustrates the recommendations from the global evidence-based desmoid consensus, in terms of positioning a place for local therapies, available systemic therapies, indications for surgery, radiotherapy, as well as the approach in paediatric patients, measuring quality-of life and benefit and presenting treatment algorithms, thus providing very useful guidance for clinical practise.
