Hereditary diffuse gastric cancer (HDGC) is an autosomal-dominant syndrome associated with early onset diffuse gastric cancer. Definitive treatment is prophylactic total gastrectomy (PTG) associated with significant morbidity. Studies published from January 2000 to December 2022 reporting clinical, histopathological or health-related quality of life outcomes in HDGC patients undergoing PTG were identified. The study quality was assessed by the “Newcastle–Ottawa scale”. Of the 257 articles screened, 21 were selected. A total of 353 patients were examined in 15 studies that reported surgical outcomes. The median age was 42 years old. The median major complication and mortality rates were 19.2% and 0.3%, respectively. The most common complications were wound infection at 4.8% followed by anastomotic leak and pulmonary complications at 4.5% each. Following PTG, 88.6% of patients had early lesions amongst 414 patients. The mean/median number of signet ring cell carcinoma foci in the gastrectomy specimens was from 2 to 78. All cases were stage 1 with no lymph node involvement. There was a wide range of psychosocial effects following PTG closely related to the physical symptoms. It is imperative for patients to receive comprehensive preoperative counselling to make an informed decision and be followed up under the care of a multidisciplinary team.
遗传性弥漫性胃癌（HDGC）是一种与早发性弥漫性胃癌相关的常染色体显性遗传综合征。其确定性治疗方式为预防性全胃切除术（PTG），但该手术伴随显著的并发症风险。本研究筛选了2000年1月至2022年12月期间发表的、报道HDGC患者接受PTG后临床、组织病理学或健康相关生活质量结局的研究文献，并采用“纽卡斯尔-渥太华量表”进行质量评估。在初筛的257篇文献中，最终纳入21篇研究进行分析。 在15项报告手术结局的研究中，共纳入353例患者，中位年龄为42岁。主要并发症中位发生率为19.2%，中位死亡率为0.3%。最常见的并发症为伤口感染（4.8%），其次是吻合口漏和肺部并发症（各占4.5%）。在414例接受PTG的患者中，88.6%发现早期病灶。胃切除标本中印戒细胞癌灶的平均/中位数量为2至78个。所有病例均为Ⅰ期且无淋巴结转移。PTG术后患者出现广泛的心理社会影响，这些影响与躯体症状密切相关。因此，必须为患者提供全面的术前咨询以支持其做出知情决策，并在多学科团队的协作下进行长期随访。
肿瘤(癌症)患者之家