We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
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.
- Authors
Miyakura, Yasuyuki; Yamaguchi, Tatsuro; Lefor, Alan Kawarai; Tamaki, Sawako; Takao, Akinari; Takao, Misato; Mori, Yoshiko; Chikatani, Kenichi; Ishida, Hideyuki; Kono, Mitsuhiro; Takeuchi, Yoji; Ishikawa, Hideki; Nagasaki, Toshiya; Sasaki, Kazuhito; Matsubara, Takaaki; Hirata, Keiji; Taniguchi, Fumitaka; Tanakaya, Kohji; Tomita, Naohiro; Ajioka, Yoichi
- 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.
- Subjects
JAPAN; ADENOMATOUS polyposis coli; POLYPS; DESMOID tumors; BILIARY tract cancer; ADENOMATOUS polyps; COHORT analysis; SYMPTOMS
- Publication
International Journal of Clinical Oncology, 2024, Vol 29, Issue 2, p169
- ISSN
1341-9625
- Publication type
Article
- DOI
10.1007/s10147-023-02442-7