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.

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Relapse rates at 5 years after radiotherapy are reported as 33% [].

This might support the hypothesis that with a combined treatment only modest surgical interventions may be needed, thus avoiding disfigurement.

While most cases are sporadic, some are associated with familial adenomatous polyposis (FAP, Gardner's Syndrome) and these are most often intra-abdominal [].

There are also cases of familial desmoid tumours at multiple sites, often involving one extremity, in patients without FAP.

The addition of radiotherapy to the treatment concept was a positive prognostic factor in the multivariate analysis.

Postoperative radiotherapy significantly improved the PFS compared to surgery alone.

Sixty-eight patients received post-operative radiotherapy and 42 surgery only. The progression-free survival time (PFS) and prognostic factors were analysed.

The combined treatment with radiotherapy showed a significantly longer progression-free survival than surgical resection alone (p smaller than 0.001).

malignant fibromyosarcoma, Dupuytren, juvenile fibromatosis) or insufficient information available for pathologic review.