Risk factors for development of desmoid tumours in familial adenomatous polyposis.
BACKGROUND
Desmoid tumours (DTs) are the primary cause of death of patients with familial adenomatous polyposis (FAP) following restorative proctocolectomy. The aim of this study was to identify risk factors for DT in a French population.


METHODS
Clinical data for 442 patients with FAP from 1983 to 2004 were reviewed retrospectively.


RESULTS
A total of 124 DTs were documented in 50 patients (25 female). DT sites were mesenteric (73 tumours), abdominal wall (44) and extra-abdominal (seven). Female patients developed DT earlier than males. Although DTs appeared after colectomy in 34 patients, the type of surgery did not influence the risk of DT. An identified point mutation in the adenomatous polyposis coli (APC) gene after codon 1444 was a significant risk factor (hazard ratio 3.3 (95 per cent confidence interval 1.5 to 7.3)). Belonging to a family affected by DT did not increase the individual's risk in this population.


CONCLUSIONS
No risk factor for life-threatening mesenteric DT could meaningfully modify the management of patients with FAP.
Lefevre
J H
JH
Department of Digestive Surgery, Hospital Saint-Antoine, Assistance Publique-Hôpitaux de Paris (AP-HP), Pierre and Marie Curie University, Paris, France.
Parc
Y
Y
Kernéis
S
S
Goasguen
N
N
Benis
M
M
Parc
R
R
Tiret
E
E
D000008:Abdominal Neoplasms / Q000209:etiology* / Q000235:genetics; D011125:Adenomatous Polyposis Coli / Q000150:complications* / Q000235:genetics / Q000601:surgery; D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D002648:Child; D005260:Female; D018221:Fibromatosis, Abdominal / Q000209:etiology* / Q000235:genetics; D018222:Desmoid Tumors / Q000209:etiology* / Q000235:genetics; D017491:Genes, APC; D006801:Humans; D008297:Male; D008643:Mesentery; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D010375:Pedigree; D016737:Proctocolectomy, Restorative; D012189:Retrospective Studies; D012307:Risk Factors