Abstract
Purpose
Data on long-term outcomes of familial adenomatous polyposis (FAP) are unclear in Japan because a nationwide registry system is lacking. We assessed overall survival, incidence of neoplasms, fecal incontinence, and postoperative follow-up status of patients with FAP treated surgically in our hospital.
Methods
In total, 154 patients with FAP who underwent radical surgery from 1981 to 2017 in our department were available for the questionnaire. Sixty-five patients, 36 of whom were followed at our hospital, were assessed using clinical records and the questionnaire.
Results
The median follow-up time was 187 months (interquartile range, 93.5–296 months). The median age at surgery was 36 years (range, 12–69 years). The 5-, 10-, 15-, and 20-year overall survival rate was 100%, 98%, 95%, and 89%, respectively. All five deaths were caused by diseases other than colorectal cancer. FAP-related neoplasms comprised 23 colorectal cancers, five duodenal cancers, three gastric cancers, five thyroid cancers, two ileal pouch cancers, and nine desmoid tumors. The incidence of desmoid tumors was significantly associated with the operation date. The duration from radical surgery to neoplasm onset significantly differed by neoplasm type. Forty-five of 54 patients (excluding those who died or underwent ileostomy) developed fecal incontinence (median Wexner score of 8). Surgical procedures involving hand-sewn sutures with rectal mucosal stripping were significantly associated with fecal incontinence and the Wexner score. Fifty-eight of the 60 surviving patients underwent follow-up examinations.
Conclusion
Overall survival was favorable. Fecal incontinence depended on the surgical procedures. Most patients continued to receive follow-up examinations.
Trial registration
No. 3112 by Institutional Review Board of Hyogo College of Medicine
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Change history
10 November 2020
In Table 4 of the original publication, an error occurred where two values were switched inadvertedly. The Wexner score for duration after surgery (year) for ���20 and over��� should be 5.32 and for ���less than 20��� should be 8.56.
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Acknowledgments
We thank all of the patients who participated in this study. We also thank Ms. Okada and Ms. Yamashita for helping with the data collection. Finally, we thank Edanz Group (www.edanzediting.com/ac) for editing a draft of this manuscript.
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All the authors contributed to the study conception and design. Data acquisition: Babaya, Yamano, Matsubara T, Takenaka, Song, Kimura, Yasuhara-Hamanaka, Kataoka, Beppu, Uchino, Ikeda, Ikeuchi, Matsubara N, Tamura, and Tomita. Drafting of manuscript: Yamano; Critical revision of manuscript: Babaya, Yamano, Matsubara T, Takenaka, Song, Kimura, Yasuhara-Hamanaka, Kataoka, Beppu, Uchino, Ikeda, Ikeuchi, Matsubara N, Tamura, and Tomita.
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Babaya, A., Yamano, T., Matsubara, T. et al. Long-term clinical outcomes and follow-up status in Japanese patients with familial adenomatous polyposis after radical surgery: a descriptive, retrospective cohort study from a single institute. Int J Colorectal Dis 35, 675–684 (2020). https://doi.org/10.1007/s00384-020-03524-y
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DOI: https://doi.org/10.1007/s00384-020-03524-y