| 中文关键词: 遗传性弥漫性胃癌 CDH1基因 病理特征 临床管理 预后 |
| 英文关键词:hereditary diffuse gastric cancer CDH1 gene pathological features clinical management prognosis |
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| 中文摘要: |
| 摘 要：遗传性弥漫性胃癌（hereditary diffuse gastric cancer，HDGC）是一种常染色体显性遗传性肿瘤综合征，主要是由E?鄄钙黏蛋白编码基因CDH1胚系突变引起，家系中常伴随其他疾病的发生，小叶性乳腺癌是除胃癌以外最常见的肿瘤，癌细胞通常浸润在完整的黏膜上皮之下，不会产生黏膜溃疡或隆起，早期在内镜下难以发现病灶，病理常见印戒细胞癌，预后差，建议行预防性全胃切除术。全文文对HDGC的诊断标准、CDH1胚系突变、病理特征、临床管理、预后等展开综述。 |
| 英文摘要: |
| Abstract： Hereditary diffuse gastric cancer(HDGC) is an autosomal dominant hereditary tumor syndrome， which is mainly caused by germline mutation of CDH1 gene， which could encode E-cadherin. Lobular breast cancer is the most common tumor except gastric cancer. Cancer cells usually infiltrate under intact mucosal epithelium and do not produce mucosal ulcers or protuberances， so it is difficult to detect lesions under endoscopy in the early stage. Signet ring cell carcinoma is common in pathology and the prognosis is poor. Prophylactic total gastrectomy is recommended. This article reviews the diagnostic criteria， CDH1 germline mutation， pathological features， clinical treatment and prognosis of hereditary diffuse gastric cancer. |
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