|ECR 2019 / C-0910||
|Moving towards a more conservative approach of desmoid tumours: role of MR in the assessment of their aggressiveness and in their management|
Aims and objectives
Desmoid tumours (DT) are rare mesenchymal tumours with an incidence of 5-6/1,000,000/year.
They usually affect young women between 15 and 60 years-old, with a peak age of about 30-40 years-old. Most cases are sporadic although approximately 5-10 % arises in the context of familial adenomatous polyposis (FAP) and Gardner’s syndrome, suggesting a link with mutations of the APC gene on chromosome 5q22.
They may classified in three types according to their location: intraabdominal, abdominal wall and extraabdominal. These last ones are located deep in the interfascial planes of musculature of the trunk and the extremities.
Histologically, they are benign tumours characterised by a monoclonal fibroblastic proliferation with marked production of intercellular collagen but they often present an unpredictable clinical course with an infiltrative growth pattern and a tendency to local recurrence, despite they lack any metastatic potential.
Since there is not an unified evidence-based approach for their management, a multidisciplinary and individualised approach is needed. However, a consensus treatment algorithm was proposed by the Soft Tissue and Bone Sarcoma group of experts of the European Organisation for Research and Treatment of Cancer (EORTC) and Sarcoma PAtients EuroNet (SPAEN) where they stand for a more conservative approach with an initial watch & wait strategy. In this scenario, imaging techniques, especially MR, have a very important role.
− To highlight the importance of MR in the management of DT (diagnosis, response to treatment and detection of recurrences)
− To evaluate usefulness of functional sequences of MR, such as diffusion and dynamic contrast-enhanced sequences.
− To revise the role of MR in the new algorithms proposed.
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