胆囊原发性混合性腺神经内分泌癌9例临床病理分析  

Mixed adenoneuroendocrine carcinoma of gallbladder:a clinicopathological analysis of nine cases

作　　者：王鹏雁 姜英[1] 贾丛伟[1] 常晓燕[1] WANG Peng-yan;JIANG Ying;JIA Cong-wei;CHANG Xiao-yan(Department of Pathology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Science,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院病理科,北京100730

出　　处：《诊断病理学杂志》2018年第9期620-624,共5页Chinese Journal of Diagnostic Pathology

基　　金：中央级公益性科研院所基金[胰腺癌及其癌前病变(导管内肿瘤及导管上皮内瘤变)及神经内分泌肿瘤的综合研究](2015PT320002)

摘　　要：目的总结胆囊原发性混合性腺神经内分泌癌(GB-MANECs)的临床病理学特征。方法回顾性分析9例胆囊混合性腺神经内分泌癌患者的临床特点,观察病理组织学形态及行免疫组化检测。结果 9例GB-MANECs患者中,男性4例,女性5例,男女比为1∶1.25;年龄37~75岁,平均年龄60岁。临床症状无特异性。肿瘤大体表现,6例可见胆囊壁增厚,3例可见胆囊腔内溃疡型肿物。镜下,1例组织形态和免疫组化结果均符合杯状细胞类癌;8例肿瘤由腺癌和神经内分泌肿瘤两种成分组成,且每种成分均>30%,其中两种成分碰撞生长1例,混合穿插在一起生长7例,腺癌高分化5例,中分化3例。8例神经内分泌肿瘤呈弥漫片状、巢状、梁状及细条索状生长,7例为G3(大细胞性4例,小细胞性3例),1例为G2。免疫组化:腺癌CEA、CK(+);神经内分泌癌Syn、CgA联合阳性率100%,CK弱(+),Ki-67阳性指数10%~85%。9例均行胆囊癌根治术,术后随访3~31个月,其中7例死亡,平均生存时间为14.4个月;杯状细胞类癌患者生存31个月,仍无复发存活。结论胆囊原发性混合性腺神经内分泌癌少见,其临床表现缺乏特异性,确诊依赖于病理形态学观察及免疫组化检测;病理形态和淋巴结转移情况提示神经内分泌成分具有更高的局部侵袭性和远处转移能力,杯状细胞类癌预后明显好于其他生长方式。Objective To evaluate the clinicopathological characteristics of gallbladder mixed adenoneuroendocrine carcinoma（ GB-MANEC）. Methods Nine cases of GB-MANECs were studied by light microscopy and immunohistochemistry with review of relevant literatures. Results Among the 9 patients,4 were males and 5 were females. The age ranged from 37 to 75 years with an average age of 60 years. There were no specific clinical symptoms.Grossly,these tumors showed intramural ulcerative nodules（ 3 cases） and diffuse thickening and sclerosis（ 6 cases） of the gallbladder wall. Microscopically one case was diagnosed as goblet cell carcinoid,while the other eight tumors were composed of adenocarcinoma and neuroendocrine neoplasms,each more than 30%. There were three growing patterns with adenocarcinoma and neuroendocrine neoplasm： arranged in collision（ 1 case）,mixed together（ 7 cases）,and amphicrine（ 1 case）. The neuroendocrine neoplasm arranged in a large nest,trabecular and thin cord,and graded in G3（ 7 cases,large cell/small cell = 4/3） and G2（ 1 case）. Immunohistochemical study showed that adenocarcinoma expressed CEA and pan CK,and neuroendocrine neoplasm expressed Cg A,Syn and pan CK. Ki-67 index was about 10-85%. All cases were treated with radical cholecystectomy. Follow-up period was 3 to 31 months,with average survival time of 14. 4 months. Two patients survived up to the present with no recurrence,and the rest 7 patients died. Conclusions GBMANECs are rare. Clinical symptoms are non-specific. Final diagnosis relies on both histological features and immunohistochemical examinations. Neuroendocrine neoplasms are more aggressive to local invasion and metastasis than adenocarcinomas. Goblet cell carcinoid has much better prognosis than other growing patterns of the tumor.

关 键 词：胆囊 混合性腺神经内分泌癌 病理诊断 

分 类 号：R735.8[医药卫生—肿瘤]