Desmoid tumors in patients with familial adenomatous polyposis.
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 in the bowel mesentery. Surgical resection of desmoids in patients with FAP has been controversial.


METHODS
A retrospective review of patients with FAP and desmoid tumors treated from 1950 to 1991 was performed. Patients were evaluated for gender, age, site of desmoid tumors, treatment, recurrence, and survival.


RESULTS
Twenty-one of 24 patients underwent 60 surgical procedures related to the desmoid tumors. Seven of nine patients who underwent potentially curative surgery had recurrences; three were reresected. Major morbidity after palliative or curative surgery was 47%. Five patients were alive with no evidence of disease 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 of 31 months after diagnosis.


CONCLUSIONS
Desmoid tumors are common in patients with FAP. Unresectability and recurrence are more common than cure. Palliative and curative resections have a high morbidity. Surgery should be reserved for those patients with symptomatic mesenteric desmoids.
Rodriguez-Bigas
M A
MA
Department of Surgical Oncology, Roswell Park Cancer Institute, Buffalo, New York 14263-0001.
Mahoney
M C
MC
Karakousis
C P
CP
Petrelli
N J
NJ
D000008:Abdominal Neoplasms / Q000473:pathology* / Q000601:surgery*; D011125:Adenomatous Polyposis Coli / Q000473:pathology*; D000293:Adolescent; D000328:Adult; D005260:Female; D018221:Fibromatosis, Abdominal / Q000473:pathology* / Q000601:surgery*; D018222:Desmoid Tumors / Q000473:pathology* / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007414:Intestinal Neoplasms / Q000473:pathology* / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000601:surgery; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009852:Omentum / Q000473:pathology / Q000601:surgery; D010166:Palliative Care; D010534:Peritoneal Neoplasms / Q000473:pathology / Q000601:surgery; D011183:Postoperative Complications; D012086:Reoperation; D012189:Retrospective Studies; D013274:Stomach Neoplasms / Q000473:pathology / Q000601:surgery; D015996:Survival Rate