Department of Surgery and Hospital de Gastroenterología "Dr. Carlos Bonorino Udaondo," Ciudad Autónoma de Buenos Aires, Argentina.
Department of Pathology, Hospital de Gastroenterología "Dr. Carlos Bonorino Udaondo," Ciudad Autónoma de Buenos Aires, Argentina.
J Laparoendosc Adv Surg Tech A. 2021 Jul;31(7):729-737. doi: 10.1089/lap.2021.0239. Epub 2021 Jun 7.
Patients with hereditary diffuse gastric cancer (HDGC) and germline mutations in the E-cadherin gene, , have a very high cumulative lifetime risk of developing diffuse gastric cancer. In these patients, it is formally recommended to perform a prophylactic total gastrectomy (PTG). We analyzed the course of patients with HDGC who have undergone PTG in our institution. Pedigree analysis, preoperative screening results, operative course, postoperative data, and complete stomach pathologic examination were performed in all patients. Seven patients with confirmed mutation underwent PTG, five were women, and average age was 27 years (range 17-42). Signet ring cell carcinoma was found in 1 patient in the preoperative surveillance endoscopic biopsies. Laparoscopic PTG was performed in all patients. There were two complications, an intestinal obstruction that required reintervention and an asymptomatic esophagojejunal anastomosis leak that resolved with conservative treatment. In all gastrectomy specimens, intramucosal signet ring cell carcinoma foci limited to the lamina propria were found (range 1-31), 83.5% were in the body-fundus region. The mean follow-up was 28.5 months (range 8-72). The mean weight loss was 9% (range 2-18). Postoperative symptoms associated with Dumping syndrome were the most frequent. All the patients reported of being very satisfied with the procedure and of having a better quality of life than expected before the procedure. Laparoscopic PTG is an excellent resource to prevent the development of advanced diffuse gastric cancer (DGC) in patients with HDGC with mutation. The procedure was well tolerated with a high satisfaction rate and very good functional results. It should be considered in these patients due to the high risk of developing advanced DGC and the lack of effective and reliable surveillance studies.
遗传性弥漫性胃癌（HDGC）患者和 E-钙黏蛋白基因突变的种系突变，有一个非常高的终生发展弥漫性胃癌的累积风险。在这些患者中，正式建议进行预防性全胃切除术（PTG）。我们分析了在我们机构接受 PTG 的 HDGC 患者的病程。对所有患者进行家系分析、术前筛查结果、手术过程、术后数据和完整胃的病理检查。7 名经证实携带突变的患者接受了 PTG，其中 5 名女性，平均年龄为 27 岁（范围 17-42）。在术前监测内镜活检中发现 1 例患者存在印戒细胞癌。所有患者均行腹腔镜 PTG。有 2 例并发症，1 例肠梗阻需要再次干预，1 例无症状食管空肠吻合口漏通过保守治疗解决。在所有胃切除标本中，均发现局限于固有层的黏膜内印戒细胞癌灶（范围 1-31），83.5%位于体-底区。平均随访时间为 28.5 个月（范围 8-72）。平均体重减轻 9%（范围 2-18）。与倾倒综合征相关的术后症状最为常见。所有患者均报告对手术非常满意，并且术后生活质量比术前预期的要好。腹腔镜 PTG 是预防携带突变的 HDGC 患者发生进展性弥漫性胃癌（DGC）的极好方法。该手术耐受性良好，满意度高，功能效果非常好。鉴于进展性 DGC 的高风险和缺乏有效、可靠的监测研究，应考虑在这些患者中采用这种方法。
