Abstract
PurposeA multi-centre study to assess the value of combined surgical resection and radiotherapy for the treatment of desmoid tumours.Patients and methodsOne hundred and ten patients from several European countries qualified for this study. Pathology slides of all patients were reviewed by an independent pathologist. Sixty-eight patients received post-operative radiotherapy and 42 surgery only. Median follow-up was 6 years (1 to 44). The progression-free survival time (PFS) and prognostic factors were analysed.ResultsThe combined treatment with radiotherapy showed a significantly longer progression-free survival than surgical resection alone (p smaller than 0.001). Extremities could be preserved in all patients treated with combined surgery and radiotherapy for tumours located in the limb, whereas amputation was necessary for 23% of patients treated with surgery alone. A comparison of PFS for tumour locations proved the abdominal wall to be a positive prognostic factor and a localization in the extremities to be a negative prognostic factor. Additional irradiation, a fraction size larger than or equal to 2 Gy and a total dose larger than 50 Gy to the tumour were found to be positive prognostic factors with a significantly lower risk for a recurrence in the univariate analysis. This analysis revealed radiotherapy at recurrence as a significantly worse prognostic factor compared with adjuvant radiotherapy. The addition of radiotherapy to the treatment concept was a positive prognostic factor in the multivariate analysis.ConclusionPostoperative radiotherapy significantly improved the PFS compared to surgery alone. Therefore it should always be considered after a non-radical tumour resection and should be given preferably in an adjuvant setting. It is effective in limb preservation and for preserving the function of joints in situations where surgery alone would result in deficits, which is especially important in young patients.
Highlights
Desmoid tumours are uncommon benign soft tissue neoplasms
While most cases are sporadic, some are associated with familial adenomatous polyposis (FAP, Gardner's Syndrome) and these are most often intraabdominal [5]
This study aims to contribute to an assessment of the therapeutic value of radiotherapy in the multimodal treatment of desmoid tumours
Summary
Desmoid tumours are uncommon benign soft tissue neoplasms. Their incidence is reported to be 2–4/1.000.000 inhabitants in Finland [1,2] or 3% of all soft tissue tumours [3]. Aggressive fibromatoses or desmoid tumours are fibroblastic lesions with aggressive, infiltrative and destructive growth, which frequently recur if not widely resected [4]. There are cases of familial desmoid tumours at multiple sites, often involving one extremity, in patients without FAP. In both FAP and familial nonFAP tumours, mutations of the adenomatous polyposis coli (APC) gene on the long arm of chromosome 5 have been incriminated. In all settings and locations these fibroblastic proliferations are similar: variably cellular, often hypocellular ill-defined fascicles of fibroblasts and myofibroblasts lacking nuclear pleomorphism and showing little mitotic activity [7]
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