家族性腺瘤性息肉病的当前治疗方法。

IF 2.9 3区 医学 Q2 ONCOLOGY Expert Review of Anticancer Therapy Pub Date : 2024-06-01 Epub Date: 2024-05-26 DOI:10.1080/14737140.2024.2344649
Sara Lauricella, Emanuele Rausa, Ilaria Pellegrini, Maria Teresa Ricci, Stefano Signoroni, Elena Palassini, Federica Cavalcoli, Patrizia Pasanisi, Chiara Colombo, Marco Vitellaro
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引用次数: 0

摘要

简介APC相关性息肉病是一种罕见的遗传性疾病,其特征是消化道出现多发性腺瘤。APC相关性息肉病患者需要在专门的中心由专业的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,提供诊断和手术治疗的最新信息,同时重点关注如何最大限度地降低发生类脂样纤维瘤病、肛门直肠残余癌和术后并发症的风险。FAP 患者需要全面的治疗方法,包括诊断、监测、预防性手术以及解决十二指肠和蜕膜肿瘤等结肠外的特殊问题。应综合考虑基因型、表型和个人需求等所有因素,进行个性化治疗。全结肠切除术和回肠直肠吻合术与恢复性直肠切除术和回肠肛门吻合术相比,如果患者严格遵守终生内镜监测,直肠残端发生癌症的肿瘤风险是可以接受的。此外,低炎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 的治疗需要多学科和个性化的方法。专家观点:FAP 的治疗需要多学科和个性化的方法,整合基因进展、创新监测技术和新兴治疗模式将有助于改善 FAP 患者的治疗效果和生活质量。
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Current management of familial adenomatous polyposis.

Introduction: 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.

Areas covered: 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.

Expert opinion: 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.

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来源期刊
CiteScore
5.10
自引率
3.00%
发文量
100
审稿时长
4-8 weeks
期刊介绍: Expert Review of Anticancer Therapy (ISSN 1473-7140) provides expert appraisal and commentary on the major trends in cancer care and highlights the performance of new therapeutic and diagnostic approaches. Coverage includes tumor management, novel medicines, anticancer agents and chemotherapy, biological therapy, cancer vaccines, therapeutic indications, biomarkers and diagnostics, and treatment guidelines. All articles are subject to rigorous peer-review, and the journal makes an essential contribution to decision-making in cancer care. Comprehensive coverage in each review is complemented by the unique Expert Review format and includes the following sections: Expert Opinion - a personal view of the data presented in the article, a discussion on the developments that are likely to be important in the future, and the avenues of research likely to become exciting as further studies yield more detailed results Article Highlights – an executive summary of the author’s most critical points.
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