Current clinical practice for familial adenomatous polyposis in Japan: A nationwide multicenter study

Matsubara, Takaaki; Beppu, Naohito; Ikeda, Masataka; Ishida, Hideyuki; Takeuchi, Yoji; Nagasaki, Toshiya; Takao, Akinari; Sasaki, Kazuhito; Akagi, Kiwamu; Sudo, Tomoya; Ueno, Hideki; Yamaguchi, Tatsuro; Tomita, Naohiro; Ajioka, Yoichi

doi:10.1002/ags3.12577

Cited by 5 publications

(2 citation statements)

References 25 publications

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“…Familial adenomatous polyposis (FAP) is a genetic disease that typically develops multiple colon polyps until the patients reach their 20s and eventually harbor colorectal cancer at 100% penetrance until their 60s [ 1 , 2 ]. Chemoprevention or intensive endoscopic resection may have antineoplastic effects on the recurrence of colonic adenomas [ 3 – 5 ]; however, prophylactic surgery of the colon and rectum is the only curative treatment for FAP.…”

Section: Introductionmentioning

confidence: 99%

Long-term prognosis after stapled and hand-sewn ileal pouch–anal anastomoses for familial adenomatous polyposis: a multicenter retrospective study

Tatsuta,

Sakata,

Iwaizumi

et al. 2024

Int J Colorectal Dis

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Purpose The long-term prognosis of stapled and hand-sewn ileal pouch–anal anastomoses in familial adenomatous polyposis patients in Japan remains unknown. This study aimed to compare the overall survival in familial adenomatous polyposis patients who underwent stapled or hand-sewn ileal pouch–anal anastomosis. Methods This multicenter retrospective study was conducted at 12 institutions in Shizuoka Prefecture, Japan. The clinical outcomes of 53 eligible familial adenomatous polyposis patients who underwent stapled (n = 24) and hand-sewn (n = 29) ileal pouch–anal anastomosis were compared. Results The median follow-up duration was 171.5 months. The incidence of adenoma in the remnant rectum or anal transitional zone and metachronous rectal cancer was significantly more common in stapled ileal pouch–anal anastomosis (adenoma: stapled, 45.8%, vs. hand-sewn, 10.3%, p = 0.005; metachronous rectal cancer: 29.2%, vs. none, p = 0.002). The number of deaths was remarkably higher in stapled ileal pouch–anal anastomosis (p = 0.002). Metachronous rectal cancer was the most common cause of death. Overall survival was worse in stapled ileal pouch–anal anastomosis than in hand-sewn ileal pouch–anal anastomosis (120 months, 90.7% vs. 96.6%; 240 months, 63.7% vs. 96.6%; p = 0.044). Cox regression analysis revealed the independent effects of preoperative advanced colorectal cancer and stapled ileal pouch–anal anastomosis on overall survival. Conclusion Stapled ileal pouch–anal anastomosis negatively affected the overall survival of familial adenomatous polyposis patients. Therefore, hand-sewn ileal pouch–anal anastomosis is recommended for better prognosis in these patients.

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Section: Introductionmentioning

confidence: 99%

Long-term prognosis after stapled and hand-sewn ileal pouch–anal anastomoses for familial adenomatous polyposis: a multicenter retrospective study

Tatsuta,

Sakata,

Iwaizumi

et al. 2024

Int J Colorectal Dis

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“…The leading cause of FAP-related deaths is CRC, although the number seems to be decreasing with widespread knowledge of the disease and the performance of prophylactic (procto)colectomy [3]. Today, prophylactic (procto)colectomy is the goldstandard treatment; however, recent trends have focused on individualization of treatment, including endoscopic treatment and/or prolonged prophylactic (procto)colectomy [4][5][6][7]. However, there is little reliable data justifying this.…”

Section: Introductionmentioning

confidence: 99%

Usefulness of genotyping APC gene for individualizing management of patients with familial adenomatous polyposis

Mori,

Ishida,

Chika

et al. 2023

Int J Clin Oncol

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BackgroundColorectal polyp burden is crucial for the management of patients with familial adenomatous polyposis (FAP). However, accurate evaluation of polyp burden is di cult to standardize. This study aimed to examine the possible utility of genotype-oriented management of colorectal neoplasms in patients with FAP. MethodsClinicopathological data from genetically proven patients with FAP was analyzed using the database of a nationwide retrospective Japanese multicenter study. The cumulative incidence of CRC was evaluated between different genotype groups. Genotype-1 were de ned as germline variants on attenuated FAP associated regions (codons 1-177, alternative splice site of exon 10 (codon 312), 1581-2843) and Genotype-2 as the other variants. Weibull and Joinpoint analyses were performed to determine the annual percentage changes in CRC risk. ResultsOverall, 69 men and 102 women were included. Forty-eight patients underwent colorectal resection for the rst CRC, and ve patients underwent resection for rst cancer in the remnant anorectal segment after prophylactic surgery. The 70-year cumulative incidence of CRC in all patients was 59.3%. Patients with Genotype-1 (n = 23) demonstrated a lower risk of CRC stage II-IV than those with Genotype-2 (n = 148, P = 0.04). The risk of stage II-IV CRC was estimated to increase markedly at the age of 49 years in the Genotype-1 patients and 34 years in the Genotype-2 patients, respectively. ConclusionsDifferent interventional strategies based on genotypes may be proposed for the clinical management of patients with FAP. This policy needs to be validated in further prospective studies focusing on long-term endoscopic intervention and optimal age at prophylactic (procto)colectomy.

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Risk of gastric adenoma and adenocarcinoma in patients with familial adenomatous polyposis in Japan: a nationwide multicenter study

Sasaki,

Kawai,

Nozawa

et al. 2024

J Gastroenterol

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Background Patients with familial adenomatous polyposis (FAP) have an increased risk of developing gastric neoplasms. However, the clinical course of FAP with these gastric lesions has not yet been fully clarified. The present study aimed to clarify the changes in the incidence risk of developing gastric adenoma or gastric cancer during the lifespan of patients with FAP. Methods Four hundred forty-three patients with data regarding gastric adenoma and gastric cancer retrospectively registered in a nationwide Japanese multicenter study were enrolled. The cumulative incidences and hazard rates (HRs) of gastric neoplasms were evaluated. Results The cumulative incidence rates in 50-year-old patients with FAP were 22.8% for gastric adenoma and 7.6% for gastric cancer, respectively. No significant association was found between gastric neoplasms and the colonic phenotype. The peak age for the HR of gastric adenoma was 65 years, with the highest HR (0.043). Regarding the incidence of gastric cancer, the HR increased moderately up to the age of 40 years, but the increase accelerated from the age of 50 years (HR = 0.0067). Conclusion Careful surveillance of the upper gastrointestinal tract in elderly patients with FAP, such as shortening the interval of follow-up according to age, may be helpful for early diagnosis of gastric cancer.

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Current clinical practice for familial adenomatous polyposis in Japan: A nationwide multicenter study

Abstract: This paper is the Ph.D. thesis for Takaaki Matsubara, and this study was carried out and written under the guidance of his supervisor Naohito Beppu. As a result, we would like to request that you permit us to submit this manuscript with double first authors including Matsubara and Beppu.

Cited by 5 publications

References 25 publications

Long-term prognosis after stapled and hand-sewn ileal pouch–anal anastomoses for familial adenomatous polyposis: a multicenter retrospective study

Long-term prognosis after stapled and hand-sewn ileal pouch–anal anastomoses for familial adenomatous polyposis: a multicenter retrospective study

Usefulness of genotyping APC gene for individualizing management of patients with familial adenomatous polyposis

Risk of gastric adenoma and adenocarcinoma in patients with familial adenomatous polyposis in Japan: a nationwide multicenter study

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