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Thus, 106 patients were evaluable for the survival analysis and 110 patients for descriptive statistics.

One patient had a bifocal disease (left and right arm), where one tumour was treated with surgery alone and the other with surgery and post-operative radiotherapy. Fifty-nine percent of all recurrences appeared during the first 2 years and 82% within 5 years of treatment. Sixty-eight patients were treated with surgery and post-operative radiotherapy (Sx RT), 42 with surgical resection alone (Sx).

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

Additionally, radiotherapy alone may serve as a primary therapy and result in minor or no deficits for those patients whose tumours are un-resectable.

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. Depending on the three major anatomic locations in which they arise, they are classified as: extra-abdominal fibromatosis, abdominal desmoid, occurring typically in women during or following pregnancy; and intra-abdominal fibromatosis, either a pelvic or mesenteric location.

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

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.

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One hundred and ten patients from several European countries qualified for this study.