日本家族性腺瘤性息肉病患者根治性手术后的长期临床结局和随访情况：来自单中心的描述性、回顾性队列研究。
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.
机构信息
Division of Lower Gastrointestinal Surgery, Department of Surgery, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo, 663-8501, Japan.
Division of Surgery, Department of Inflammatory Bowel Disease, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo, 663-8501, Japan.
出版信息
Int J Colorectal Dis. 2020 Apr;35(4):675-684. doi: 10.1007/s00384-020-03524-y. Epub 2020 Feb 7.
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.
目的
由于缺乏全国性的登记系统，日本有关家族性腺瘤性息肉病（FAP）长期预后的数据尚不清楚。我们评估了我院外科治疗 FAP 患者的总体生存率、肿瘤发生率、粪便失禁和术后随访情况。
方法
在我们科室，1981 年至 2017 年间共有 154 例 FAP 患者接受了根治性手术，其中 65 例患者（36 例在我院接受随访）可通过临床记录和问卷调查进行评估。
结果
中位随访时间为 187 个月（四分位距 93.5-296 个月）。手术时的中位年龄为 36 岁（12-69 岁）。5 年、10 年、15 年和 20 年的总生存率分别为 100%、98%、95%和 89%。所有 5 例死亡均由结直肠癌以外的疾病引起。FAP 相关肿瘤包括 23 例结直肠癌、5 例十二指肠癌、3 例胃癌、5 例甲状腺癌、2 例回肠套叠癌和 9 例纤维瘤。纤维瘤的发病率与手术日期显著相关。从根治性手术到肿瘤发病的时间间隔因肿瘤类型而异。54 例患者中（不包括死亡或行肠造口术的患者）有 45 例发生粪便失禁（Wexner 评分中位数为 8）。直肠黏膜剥离的手工缝合手术与粪便失禁和 Wexner 评分显著相关。60 例存活患者中的 58 例接受了随访检查。
结论
总体生存率良好。粪便失禁取决于手术方式。大多数患者继续接受随访检查。
临床试验注册号
Hyogo College of Medicine 机构审查委员会 No. 3112。
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