Background. Sporadic desmoid tumors occur mainly in the abdominal wall
and in extraabdominal sites. Desmoid tumors in patients with familial
adenomatous polyposis (FAP) usually occur in the abdominal wall and i
n the bowel mesentery. Surgical resection of desmoids in patients with
FAP has been controversial. Methods. A retrospective review of patien
ts with FAP and desmoid tumors treated from 1950 to 1991 was performed
. Patients were evaluated for gender, age, site of desmoid tumors, tre
atment, recurrence, and survival. Results. Twenty-one of 24 patients u
nderwent 60 surgical procedures related to the desmoid tumors. Seven o
f nine patients who underwent potentially curative surgery had recurre
nces; three were reresected. Major morbidity after palliative or curat
ive surgery was 47%. Five patients were alive with no evidence of dise
ase at a median of 198 months, 10 patients were alive with disease at
a median of 102 months, and 5 patients died with disease at a median o
f 31 months after diagnosis. Conclusions. Desmoid tumors are common in
patients with FAP. Unresectability and recurrence are more common tha
n cure. Palliative and curative resections have a high morbidity. Surg
ery should be reserved for those patients with symptomatic mesenteric
desmoids.