RESUMO
BACKGROUND: The desmoid tumor is a histologically benign neoplasm originating in muscular-aponeurotic structures and possesses a high tendency of local recurrence. The aggressive nature of the tumor obliges radical surgery to be performed principally in extra-abdominal tumors. METHODS: Twelve patients were treated from 1983 to 1993. The treatments performed prior to and after histologic diagnosis, the time of evolution when radical treatment was carried out upon diagnosis and the evolution after treatment were evaluated. RESULTS: Of the patients with desmoid tumor 8 were males with a mean age of 29.9 +/- 12.5 years. In eleven cases the presentation was as a tumor and was of pain in only one case. All the patients were surgically treated with a total of 27 interventions being carried out. Three patients also received pharmacologic treatment with tamoxifen, in 2 cases associated to sulindac. In two radiotherapy was used but not as a first line treatment. Following a mean follow up of 38 +/- 17 months no mortality has been registered but 5 patients are not disease free. CONCLUSIONS: The therapeutic attitude to the desmoid tumor should be radical surgery in both the primary tumor and the recurrences with medical treatment being performed in specific situations.
