BACKGROUND: The desmoid tumor is a histologically benign neoplasm orig
inating in muscular-aponeurotic structures and possesses a high tenden
cy of local recurrence. The aggressive nature of the tumor obliges rad
ical surgery to be performed principally in extra-abdominal tumors. ME
THODs: Twelve patients were treated from 1983 to 1993. The treatments
performed prior to and after histologic diagnosis, the time of evoluti
on when radical treatment was carried out upon diagnosis and the evolu
tion after treatment were evaluated. RESULTS: Of the patients with des
moid tumor 8 were males with a mean age of 29.9 +/- 12.5 years. In ele
ven 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 inte
rventions being carried out. Three patients also received pharmacologi
c treatment with tamoxifen, in 2 cases associated to sulindac. In two
radiotherapy was used but not as a first line treatment. Following a m
ean follow up of 38 +/- 17 months no mortality has been registered but
5 patients are not disease free. CONCLUSIONS: The therapeutic attitud
e to the desmoid tumor should be radical surgery in both the primary t
umor and the recurrences with medical treatment being performed in spe
cific situations.