Abstract：
UNASSIGNED: APC-associated polyposis is a rare hereditary disorder characterized by the development of multiple adenomas in the digestive tract. Individuals with APC-associated polyposis need to be managed by specialized multidisciplinary teams in dedicated centers.
UNASSIGNED: The study aimed to review the literature on Familial adenomatous polyposis (FAP) to provide an update on diagnostic and surgical management while focusing on strategies to minimize the risk of desmoid-type fibromatosis, cancer in anorectal remnant, and postoperative complications. FAP individuals require a comprehensive approach that includes diagnosis, surveillance, preventive surgery, and addressing specific extracolonic concerns such as duodenal and desmoid tumors. Management should be personalized considering all factors: genotype, phenotype, and personal needs. Total colectomy and ileo-rectal anastomosis have been shown to yield superior QoL results when compared to Restorative Procto colectomy and ileopouch-anal anastomosis with acceptable oncological risk of developing cancer in the rectal stump if patients rigorously adhere to lifelong endoscopic surveillance. Additionally, a low-inflammatory diet may prevent adenomas and cancer by modulating systemic and tissue inflammatory indices.
UNASSIGNED: FAP management requires a multidisciplinary and personalized approach. Integrating genetic advances, innovative surveillance techniques, and emerging therapeutic modalities will contribute to improving outcomes and quality of life for FAP individuals.
UNASSIGNED: The study aimed to review the literature on Familial adenomatous polyposis (FAP) to provide an update on diagnostic and surgical management while focusing on strategies to minimize the risk of desmoid-type fibromatosis, cancer in anorectal remnant, and postoperative complications. FAP individuals require a comprehensive approach that includes diagnosis, surveillance, preventive surgery, and addressing specific extracolonic concerns such as duodenal and desmoid tumors. Management should be personalized considering all factors: genotype, phenotype, and personal needs. Total colectomy and ileo-rectal anastomosis have been shown to yield superior QoL results when compared to Restorative Procto colectomy and ileopouch-anal anastomosis with acceptable oncological risk of developing cancer in the rectal stump if patients rigorously adhere to lifelong endoscopic surveillance. Additionally, a low-inflammatory diet may prevent adenomas and cancer by modulating systemic and tissue inflammatory indices.
UNASSIGNED: FAP management requires a multidisciplinary and personalized approach. Integrating genetic advances, innovative surveillance techniques, and emerging therapeutic modalities will contribute to improving outcomes and quality of life for FAP individuals.
摘要：
■APC相关息肉病是一种罕见的遗传性疾病，其特征是消化道中多发性腺瘤的发展。患有APC相关息肉病的个人需要由专门中心的专业多学科团队进行管理。
■该研究旨在回顾有关家族性腺瘤性息肉病（FAP）的文献，以提供有关诊断和手术管理的最新信息，同时重点关注将纤维瘤病的风险降至最低的策略。肛门直肠残余癌，术后并发症。FAP个人需要一个全面的方法，包括诊断，监视,预防性手术,并解决特定的结肠外问题，如十二指肠和硬纤维瘤。管理应该是个性化的，考虑到所有因素：基因型，表型,和个人需求。与恢复性结肠切除术和回肠-肛门吻合术相比，全结肠切除术和回肠-直肠吻合术具有更好的QoL结果，如果患者严格遵守终身内窥镜监测，则在直肠残端发生癌症的肿瘤风险可接受。此外,低炎症饮食可通过调节全身和组织炎症指数来预防腺瘤和癌症.
■FAP管理需要多学科和个性化的方法。整合基因进步，创新的监控技术,和新兴的治疗模式将有助于改善FAP患者的预后和生活质量。
■该研究旨在回顾有关家族性腺瘤性息肉病（FAP）的文献，以提供有关诊断和手术管理的最新信息，同时重点关注将纤维瘤病的风险降至最低的策略。肛门直肠残余癌，术后并发症。FAP个人需要一个全面的方法，包括诊断，监视,预防性手术,并解决特定的结肠外问题，如十二指肠和硬纤维瘤。管理应该是个性化的，考虑到所有因素：基因型，表型,和个人需求。与恢复性结肠切除术和回肠-肛门吻合术相比，全结肠切除术和回肠-直肠吻合术具有更好的QoL结果，如果患者严格遵守终身内窥镜监测，则在直肠残端发生癌症的肿瘤风险可接受。此外,低炎症饮食可通过调节全身和组织炎症指数来预防腺瘤和癌症.
■FAP管理需要多学科和个性化的方法。整合基因进步，创新的监控技术,和新兴的治疗模式将有助于改善FAP患者的预后和生活质量。
