Desmoid disease as a part of familial adenomatous polyposis coli.
In the Finnish Polyposis Registry with 71 known families and 168 verified adenomatosis coli patients, 19 (11%) cases were complicated by desmoid tumors. Six polyposis patients (3.6%) had a desmoid before colectomy and the cumulative risk of postoperative desmoid tumors was 16% at ten years after colectomy. The median interval between the operation and the diagnosis of desmoid was 2.3 years. Eleven women and eight men were affected; the mean age was 28.5 years. Development of postoperative desmoid tumors was associated with early age at the time of colectomy, 26 years versus 38 years in those without subsequent desmoids (p less than 0.01). In two families more than one polyposis patient were affected by desmoids. All patients with desmoid tumors had other accompanying extracolonic lesions. No deaths from desmoid disease have occurred, even though only four of eight mesenteric tumors and five of nine abdominal wall tumors (53%) were excised without evidence of recurrence. Operative treatment is required in most cases, but resection may involve great hazard especially in mesenteric desmoids.
Järvinen
H J
HJ
Second Department of Surgery, University Central Hospital, Helsinki, Finland.
D011125:Adenomatous Polyposis Coli / Q000235:genetics* / Q000601:surgery; D000293:Adolescent; D000328:Adult; D002648:Child; D003082:Colectomy; D005260:Female; D005350:Fibroma / Q000235:genetics* / Q000601:surgery; D005387:Finland; D006801:Humans; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000235:genetics* / Q000601:surgery; D011183:Postoperative Complications; D011379:Prognosis; D012307:Risk Factors