Clinical features and distribution of the APC variant in duodenal and ampullary polyps in patients with familial adenomatous polyposis: a multicenter retrospective cohort study in Japan.
Int J Clin Oncol
; 29(2): 169-178, 2024 Feb.
Article
in En
| MEDLINE
| ID: mdl-38142452
ABSTRACT
BACKGROUND:
Management of duodenal or ampullary adenomas in patients with familial adenomatous polyposis (FAP) is a major challenge for clinicians. Insufficient data are available to evaluate the clinical manifestations and distribution of adenomatous polyposis coli (APC) variants in these patients.METHODS:
We enrolled 451 patients with data regarding duodenal or ampullary polyps from 632 patients with FAP retrospectively registered in a nationwide Japanese multicenter study. Clinicopathological features and distribution of APC variants were compared between patients with and without duodenal or ampullary polyps.RESULTS:
Duodenal and ampullary polyps were found in 59% and 18% of patients with FAP, respectively. The incidence of duodenal cancer was 4.7% in patients with duodenal polyps, and that of ampullary cancer was 18% in patients with ampullary polyps. Duodenal polyps were significantly associated with the presence of ampullary polyps and jejunal/ileal polyps. Duodenal polyps progressed in 35% of patients with a median follow-up of 776 days, mostly in those with early Spigelman stage lesions. Ampullary polyps progressed in 50% of patients with a follow-up of 1484 days. However, only one patient developed a malignancy. The proportion of patients with duodenal polyps was significantly higher among those with intermediate- or profuse-type APC variants than attenuated-type APC variants. The presence of duodenal polyps was significantly associated with ampullary and jejunal/ileal polyps in patients with intermediate- or profuse-type APC variants.CONCLUSIONS:
Periodic endoscopic surveillance of the papilla of Vater and small intestine should be planned for patients with FAP with duodenal polyps.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Ampulla of Vater
/
Common Bile Duct Neoplasms
/
Adenomatous Polyposis Coli
/
Duodenal Neoplasms
Limits:
Humans
Country/Region as subject:
Asia
Language:
En
Journal:
Int J Clin Oncol
Journal subject:
NEOPLASIAS
Year:
2024
Document type:
Article
Affiliation country:
Country of publication: