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
© 2023. The Author(s) under exclusive licence to Japan Society of Clinical Oncology..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:29 |
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| Enthalten in: |
International journal of clinical oncology - 29(2024), 2 vom: 15. Feb., Seite 169-178 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Miyakura, Yasuyuki [VerfasserIn] |
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| Links: |
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| Themen: |
APC pathogenic variant |
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| Anmerkungen: |
Date Completed 05.02.2024 Date Revised 05.02.2024 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1007/s10147-023-02442-7 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM366331132 |
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| 100 | 1 | |a Miyakura, Yasuyuki |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Clinical features and distribution of the APC variant in duodenal and ampullary polyps in patients with familial adenomatous polyposis |b a multicenter retrospective cohort study in Japan |
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| 500 | |a Date Completed 05.02.2024 | ||
| 500 | |a Date Revised 05.02.2024 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2023. The Author(s) under exclusive licence to Japan Society of Clinical Oncology. | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a CONCLUSIONS: Periodic endoscopic surveillance of the papilla of Vater and small intestine should be planned for patients with FAP with duodenal polyps | ||
| 650 | 4 | |a Multicenter Study | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a APC pathogenic variant | |
| 650 | 4 | |a Ampullary polyp | |
| 650 | 4 | |a Duodenal polyp | |
| 650 | 4 | |a Familial adenomatous polyposis | |
| 650 | 4 | |a Spigelman stage | |
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| 700 | 1 | |a Yamaguchi, Tatsuro |e verfasserin |4 aut | |
| 700 | 1 | |a Lefor, Alan Kawarai |e verfasserin |4 aut | |
| 700 | 1 | |a Tamaki, Sawako |e verfasserin |4 aut | |
| 700 | 1 | |a Takao, Akinari |e verfasserin |4 aut | |
| 700 | 1 | |a Takao, Misato |e verfasserin |4 aut | |
| 700 | 1 | |a Mori, Yoshiko |e verfasserin |4 aut | |
| 700 | 1 | |a Chikatani, Kenichi |e verfasserin |4 aut | |
| 700 | 1 | |a Ishida, Hideyuki |e verfasserin |4 aut | |
| 700 | 1 | |a Kono, Mitsuhiro |e verfasserin |4 aut | |
| 700 | 1 | |a Takeuchi, Yoji |e verfasserin |4 aut | |
| 700 | 1 | |a Ishikawa, Hideki |e verfasserin |4 aut | |
| 700 | 1 | |a Nagasaki, Toshiya |e verfasserin |4 aut | |
| 700 | 1 | |a Sasaki, Kazuhito |e verfasserin |4 aut | |
| 700 | 1 | |a Matsubara, Takaaki |e verfasserin |4 aut | |
| 700 | 1 | |a Hirata, Keiji |e verfasserin |4 aut | |
| 700 | 1 | |a Taniguchi, Fumitaka |e verfasserin |4 aut | |
| 700 | 1 | |a Tanakaya, Kohji |e verfasserin |4 aut | |
| 700 | 1 | |a Tomita, Naohiro |e verfasserin |4 aut | |
| 700 | 1 | |a Ajioka, Yoichi |e verfasserin |4 aut | |
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