Long-term clinical outcomes and follow-up status in Japanese patients with familial adenomatous polyposis after radical
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ORIGINAL ARTICLE
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 Akihito Babaya 1 & Tomoki Yamano 1 & Takaaki Matsubara 1 & Yuya Takenaka 1 & Jihyung Song 1 & Kei Kimura 1 & Michiko Yasuhara 1 & Kozo Kataoka 1 & Naohito Beppu 1 & Motoi Uchino 2 & Masataka Ikeda 1 & Hiroki Ikeuchi 2 & Nagahide Matsubara 3 & Kazuo Tamura 4 & Naohiro Tomita 1 Accepted: 30 January 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
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 followup 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 Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00384-020-03524-y) contains supplementary material, which is available to authorized users. * Tomoki Yamano [email protected] 1
Division of Lower Gastrointestinal Surgery, Department of Surgery, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, Japan
2
Division of Surgery, Department of Inflammatory Bowel Disease, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, Japan
3
Department of Surgery, Amagasaki Chuo Hospital, 1-21-1 Shioe, Amagasaki, Hyogo 660-0808, Japan
4
Departm
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