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Published in: Radiation Oncology 1/2007

Open Access 01-12-2007 | Research

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

Authors: Brigitta G Baumert, 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

Published in: Radiation Oncology | Issue 1/2007

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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.
Appendix
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Metadata
Title
The impact of radiotherapy in the treatment of desmoid tumours. An international survey of 110 patients. A study of the Rare Cancer Network
Authors
Brigitta G Baumert
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
Publication date
01-12-2007
Publisher
BioMed Central
Published in
Radiation Oncology / Issue 1/2007
Electronic ISSN: 1748-717X
DOI
https://doi.org/10.1186/1748-717X-2-12

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