摘要
目的研究我国南方家族性胃癌家系的发病特点和预后，及其家系中发生相关肿瘤的特点，探讨我国家族性胃癌的诊断和筛选方法。方法通过家系调查，收集8个符合国际遗传性胃癌协作组制定的遗传性胃癌（ICG—HGC）家系和4个可疑ICG—HGC家系，绘制其家系图谱，分析其发病的病理特点及预后，同时了解其家族成员发生相关肿瘤的特点。结果ICG—HGC家系和4个可疑ICG—HGC家系中共有肿瘤患者45例，发病年龄29～65岁，共有肿瘤病灶58个（包括1例多源癌），有胃癌病灶41个，其中胃病灶30个，以胃窦、体部为主，病理类型为中低分化腺癌；胃外肿瘤17个，其中结直肠肿瘤6个。11例先证者中根治术后4例生存时间大于3年，最长超过10年。结论中国南方家族性胃癌表现为发病年龄轻，病理分化差，进展期胃癌常发生在远端胃，常伴发肠道肿瘤。遴选ICG-HGC家系时应注意与遗传性非息肉病性结直肠癌家系区分。
Objective To explore clinical features, prognosis and study related cancer types in patients with familial gastric carcinoma. Methods Nine families of ICG-HGC and 3 families of suspected- ICG-HGC according with International Collaborative Group on Hereditary Gastric Cancer standard were collected and their pedigree trees were drawn. Clincial features and prognosis of ICG-HGC and suspected- ICG-HGC families were analyzed, and the related cancer types of ICG-HGC and suspected-ICG-HGC kindreds were investigated. Results The morbidity of ICG-HGC and suspected-ICG-HGC was 0. 99% ; The age of patients among the propositi of 12 kindreds were 29 - 65 years old with the mean age of 56 years old. Among 11 kindreds, there were 45 cases of cancers including 2 cases of multiple cancers. There were 30 foci of gastric cancer, most of which were located in lower and middle third of the stomach, with pathologic type of adenocarcinoma in poor to moderate differentiation. There were totally 15 extrastomach tumors including 7 colorectal cancers. Four cases of 11 kindreds had survived for 3 years including 1 case surviving for more than 10 years. Conclusion The familial gastric cancers in southern China have special characteristics such as poorly pathologic differentiation, advanced cancer stage, early age of onset, distal end of the stomach in location and high incidence of related tumors in colorectum.
出处
《中华普通外科杂志》
CSCD
北大核心
2008年第4期265-268,共4页
Chinese Journal of General Surgery
基金
国家自然基金（30571832）
广东省自然基金项目（7001669）
中山大学附属第一医院与生命科学院合作项目（162013）
关键词
胃肿瘤
病理学
临床
系谱
家族性
Stomach neoplasms
Pathology,clinical
Pedigree
Familial
