Setia Namrata, Clark Jeffrey W, Duda Dan G, Hong Theodore S, Kwak Eunice L, Mullen John T, Lauwers Gregory Y
Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA.
Department of Hematology/Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA.
Oncologist. 2015 Dec;20(12):1365-77. doi: 10.1634/theoncologist.2015-0205. Epub 2015 Sep 30.
Although the majority of gastric carcinomas are sporadic, approximately 10% show familial aggregation, and a hereditary cause is determined in 1%-3% cases. Of these, hereditary diffuse gastric cancer is the most recognized predisposition syndrome. Although rare, the less commonly known syndromes also confer a markedly increased risk for development of gastric cancer. Identification and characterization of these syndromes require a multidisciplinary effort involving oncologists, surgeons, genetic counselors, biologists, and pathologists. This article reviews the molecular genetics, clinical and pathologic features, surveillance guidelines, and preventive measures of common and less common hereditary gastric cancer predisposition syndromes.
虽然大多数胃癌是散发性的，但约10%呈现家族聚集性，并且在1%-3%的病例中可确定有遗传原因。其中，遗传性弥漫性胃癌是最广为人知的易患综合征。虽然罕见，但较不为人知的综合征也会显著增加患胃癌的风险。识别和表征这些综合征需要肿瘤学家、外科医生、遗传咨询师、生物学家和病理学家的多学科努力。本文综述了常见和较不常见的遗传性胃癌易患综合征的分子遗传学、临床和病理特征、监测指南及预防措施。
