Extremity and trunk desmoid tumors: a multifactorial analysis of outcome

The natural history of desmoid tumors remains an enigma. Previous reports attempting to identify their biology have included recurrent and primary tumors as well as tumors from both intra- and extra-abdominal sites. The purpose of this study was to analyze patients with primary extremity and trunk desmoid tumors treated and followed at a single institution and to determine factors influencing disease free survival.

Between July 1982 and June 1997, 189 patients with extremity and superficial trunk desmoid tumors were treated and followed prospectively. Of these, 105 presented with primary disease and formed the basis of this study.

The median follow-up for the entire group of patients was 49 months; it was 46 months for patients who did not develop a local recurrence. During this time, 24 patients (23%) had a local recurrence. No patients died of disease. The 2-year and 5-year local recurrence free survival rates were 80% and 75%, respectively. None of the prognostic factors analyzed, including age, gender, depth of tumor, size of tumor, or tumor site, were significant for predicting local recurrence. Moreover, positive resection margins were not predictive of recurrence. The selective use of adjuvant radiation therapy did not influence the rate of local recurrence regardless of the margin status.

Attempts to achieve negative resection margins may result in unnecessary morbidity and may not prevent local recurrence. Operations that preserve function and structure should be the primary goal, because the presence of residual disease cannot be clearly shown to impact adversely on 5-year disease free or overall survival.