Extremity and trunk desmoid tumors - A multifactorial analysis of outcome

BACKGROUND. The natural history of desmoid tumors remains an enigma. Previo us reports attempting to identify their biology have included recurrent and primary tumors as well as tumors from both intra- and extra-abdominal site s. 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. METHODS. Between July 1982 and June 1997, 189 patients with extremity and s uperficial trunk desmoid tumors were treated and followed prospectively. Of these, 105 presented with primary disease and formed the basis of this stu dy. RESULTS. The median follow-up for the entire group of patients was 49 month s; it was 46 months for patients who did not develop a local recurrence. Du ring 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, includ ing age, gender, depth of tumor, size of tumor, or tumor site, were signifi cant 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 ma rgin status. CONCLUSIONS, Attempts to achieve negative resection margins may result in u nnecessary morbidity and may not prevent local recurrence. Operations that preserve function and structure should be the primary goal, because the pre sence of residual disease cannot be clearly shown to impact adversely on 5- year disease free or overall survival. Cancer 1999;86:2045-52. (C) 1999 Ame rican Cancer Society.