Curative and Prophylactic Surgery of Young-onset Colorectal Cancer in Inherited Syndromes: A 15-Year Monocentric Retrospective Experience
Curative and Prophylactic Surgery of Young-onset Colorectal Cancer in Inherited Syndromes: A 15-Year Monocentric Retrospective Experience
复制标题
遗传综合征中年轻发病结直肠癌的治疗和预防性手术：15 年单中心回顾性经验
作者：
E. Virgilio;G. Balducci;P. Mercantini;M. Ferri;A. Palumbo;E. Pilozzi;M. Cavallini
背景/目的：尽管在过去的二十年中，关于Lynch综合征（LS）和家族性腺瘤性息肉病（FAP）的基因蛋白质组学和临床病理学知识显著增加，尽管手术是治疗这两种疾病的主要方法，但截至2019年，在缺乏明确指南的情况下，手术选择的时间和程序仍然存在争议。材料和方法：回顾性分析2003年1月1日至2018年12月31日在我院接受手术治疗的结直肠癌（CRC）患者的数据。特别关注LS和FAP≤45岁的患者（年轻发病的CRC）；对这类患者进行手术治疗的利弊比较。结果：共有1878例原发性crc患者接受了大手术；年轻发病的恶性肿瘤占所有crc的3.8%。从11例LS患者和2例FAP患者中手术切除了13例年轻发病的遗传性crc。节段性结肠切除术和恢复性直结肠切除术分别是年轻LS和FAP患者最常进行的手术。结论：根据我们的回顾性结果，我们强调需要进行随机对照试验，比较年轻患者LS和fap相关CRC的手术选择。明确每种手术选择的优势和风险对于改善此类患者的预后和建立一致的建议至关重要。
Background/Aim: Although genoproteomic and clinicopathological knowledge on Lynch syndrome (LS) and familial adenomatous polyposis (FAP) has notably increased during the past two decades and even though surgery represents the mainstay of treatment for both conditions, as of 2019, the surgical choice in terms of timing and procedure still appears controversial in the absence of definitive guidelines. Materials and Methods: Data were retrospectively analyzed of patients with colorectal cancer (CRC) surgically treated at our Institution between 1st January 2003 and 31st December 2018. Particular attention was given to patients with LS and FAP ≤45 years of age (young-onset CRC); for this category of patients, the surgical procedures performed were compared in terms of benefits and disadvantages. Results: A total of 1,878 primary CRCs were submitted to major surgery; young-onset malignancies accounted for 3.8% of all CRCs. Thirteen young-onset inherited CRCs were surgically removed from 11 patients with LS and two with FAP. Segmental colectomy and restorative proctocolectomy were the procedures most frequently performed in young patients with LS and FAP, respectively. Conclusion: In the light of our retrospective results, we highlight the need for randomized controlled trials comparing the surgical options for LS- and FAP-related CRC developing in young patients. Defining the advantages and risks of each surgical option is of the utmost importance in order to improve prognosis of such patients and establish unanimous recommendations.