Sinha A, Karstensen J G, Liska D
St Mark's Centre for Familial Intestinal Cancers, St Mark's, The National Bowel Hospital, London, UK.
Danish Polyposis Register, Gastro Unit, Copenhagen University Hospital - Amager and Hvidovre, Hvidovre, Denmark.
Fam Cancer. 2025 Sep 5;24(4):69. doi: 10.1007/s10689-025-00492-6.
Familial adenomatous polyposis (FAP) is an inherited condition that predisposes individuals to colorectal cancer without preventive treatment. Surgical management typically involves restorative proctocolectomy with an ileal pouch anal anastomosis or colectomy with ileorectal anastomosis. Complete removal of the large intestine and rectum with a permanent stoma may also be required in selected cases. This narrative review highlights decision-making in FAP regarding the timing, extent, and modality of large bowel surgery. Key considerations include the extent of polyps, cancer risk in the remaining rectum, and associated extra-colonic manifestations like desmoid disease. The timing of surgery and the extent of bowel removal are critical factors requiring a personalized approach that considers patient preferences and clinical factors. Regardless of the chosen strategy, continued surveillance is essential to monitor disease progression.
家族性腺瘤性息肉病（FAP）是一种遗传性疾病，若不进行预防性治疗，个体易患结直肠癌。手术治疗通常包括回肠储袋肛管吻合术的结直肠全切除术或回肠直肠吻合术的结肠切除术。在某些特定情况下，可能还需要进行大肠和直肠的完全切除并造永久性造口。本叙述性综述重点介绍了FAP在大肠手术时机、范围和方式方面的决策。关键考虑因素包括息肉范围、剩余直肠的癌症风险以及诸如硬纤维瘤病等相关的结肠外表现。手术时机和肠切除范围是关键因素，需要采用个性化的方法，考虑患者的偏好和临床因素。无论选择何种策略，持续监测对于监测疾病进展至关重要。
