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. 2007 Mar 7:2:12.
doi: 10.1186/1748-717X-2-12.

The impact of radiotherapy in the treatment of desmoid tumours. An international survey of 110 patients. A study of the Rare Cancer Network

Brigitta G Baumert  1 , Martin O Spahr, Arthur Von Hochstetter, Sylvie Beauvois, Christine Landmann, Katrin Fridrich, Salvador Villà, Michael J Kirschner, Guy Storme, Peter Thum, Hans K Streuli, Norbert Lombriser, Robert Maurer, Gerhard Ries, Ernst-Arnold Bleher, Alfred Willi, Juerg Allemann, Ulrich Buehler, Hugo Blessing, Urs M Luetolf, J Bernard Davis, Burkhardt Seifert, Manfred Infanger
Affiliations

The impact of radiotherapy in the treatment of desmoid tumours. An international survey of 110 patients. A study of the Rare Cancer Network

Brigitta G Baumert et al. Radiat Oncol. .

Abstract

Purpose: A multi-centre study to assess the value of combined surgical resection and radiotherapy for the treatment of desmoid tumours.

Patients and methods: One 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.

Results: The 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.

Conclusion: Postoperative 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.

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Figures

Figure 1
Figure 1
Overall progression-free survival at the last reported follow-up (Kaplan-Meier curves).
Figure 2
Figure 2
Progression-free survival taking multiple recurrent events per patient into account (Cox proportional hazard regression with shared frailty).
See this image and copyright information in PMC

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