Peutz-Jeghers综合征10例消化道肿瘤临床病理分析  

Peutz-Jeghers syndrome with gastrointestinal tumors:a clinicopathologic analysis of ten cases

作　　者：林文生 张芳[1] 李腾 任力 LIN Wen-sheng;ZHANG Fang;LI Teng;REN Li(Department of Pathology,Air Force General Hospital,Beifing 100142,China)

机构地区：[1]空军总医院病理科,北京100142

出　　处：《诊断病理学杂志》2018年第7期497-502,共6页Chinese Journal of Diagnostic Pathology

基　　金：2016年军队青年基金课题(课题号16QNP025)

摘　　要：目的探讨Peutz-Jeghers综合征(PJS)消化道肿瘤的临床病理特征、发病机制,提高对该肿瘤易感的认识。方法回顾性分析10例PJS消化道肿瘤病例的临床特点、病理和免疫组化染色特征,并文献复习。结果本文收集10例罹患消化道肿瘤的PJS,其中6例为错构瘤息肉恶变为黏液腺癌,2例为十二指肠黏液腺癌;其余2例,1例为十二指肠高-中分化腺癌,1例为横结肠中-低分化腺癌。结论本报道为国内关于PJS罹患消化道肿瘤的最大病例数报道。PJS罹患消化道肿瘤的主要病理类型为黏液腺癌。Objective To investigate the pathological features and pathogenesis of Peutz-Jeghers syndrome（ PJS） with gastrointestinal carcinoma,and to enhance the understanding of its mechanism. Methods Clinical characteristics,histological features and immunohistochemical staining were analyzed in 10 cases of gastrointestinal carcinoma in PJS,with review of relevant literature. Results We selected 10 cases of PJS with gastrointestinal carcinoma. Among them,there were 6 cases of polyps that were transformed into mucinous adenocarcinoma, 2 cases of duodenal mucinous adenocarcinoma,one well-morderate duodenal adenocarcinoma and one morderate-poorly differentiated transverse colon adenocarcinoma. Conclusion As far as we know,this is the largest study of PJS with gastroinstinal carcinoma（ 10 cases）. The main hisological type is mucinous adenocarcinoma in this cohort.

关 键 词：PEUTZ-JEGHERS综合征 消化道肿瘤 黏液腺癌 

分 类 号：R735[医药卫生—肿瘤]