消化系统杯状细胞腺癌22例临床病理学观察

Goblet cell adenocarcinoma in the digestive system:a clinicopathological analysis of 22 cases

目的探讨消化系统杯状细胞腺癌(goblet cell adenocarcinoma,GCA)临床特征、组织学特征、免疫表型、诊断及鉴别诊断。方法2010年1月至2021年1月消化系统GCA 22例,收集临床病理资料、形态学特征、免疫表型,复习相关文献,同时将25例神经内分泌肿瘤(NEN)和24例腺癌作为阳性对照。结果男性16例,女性6例;年龄36~79岁,平均年龄56岁。22例肿瘤中发生部位位于阑尾17例,阑尾外5例。后者包括胃3例、十二指肠1例和肛管1例。17例阑尾GCA均为纯GCA,5例阑尾外GCA中1例胃GCA为纯GCA、2例胃GCA伴NEN或腺癌,十二指肠GCA伴NEN和腺癌,肛管GCA伴NEN。低级别GCA由数量不等的杯状细胞、潘氏细胞及神经内分泌细胞构成肠隐窝管状或簇状结构,分布于消化系统管壁中。管状和簇状结构缺乏黏附性。杯状细胞呈柱状,位于基底、胞质透明、细胞核小、异型性不明显、核分裂象不常见;细胞外黏液和变异型细胞核的印戒细胞在部分病例可见。常可见神经纤维束侵犯及脉管内瘤栓。高级别GCA则缺乏小管状和簇状结构,结构较复杂。肿瘤细胞混合性地表达神经内分泌和腺上皮标志物。与突触素和嗜铬粒素A的表达模式类似,CD200和INSM1在GCA中呈点状或斑片状阳性表达。结论GCA是一种消化系统不常见的肿瘤,呈现神经内分泌-腺上皮双向分化特征。准确地诊断及分期与患者的预后相关。

Objective To investigate the clinical features,morphological characteristics,immunophenotype,and differential diagnosis of goblet cell adenocarcinoma(GCA)in the digestive system.Methods The clinicopathological data,morphological characteristics,immunophenotypes of 22 cases of GCA in the digestive system diagnosed from January 2010 to January 2021 were collected.Meanwhile,25 cases of neuroendocrine neoplasm(NEN)and 24 cases of adenocarcinoma were used as controls.Relevant literature was also reviewed.Results There were 16 males and 6 females,aged from 36 to 79 years with an average of 56 years.The anatomical sites of the 22 GCA were mostly appendix(17 cases)and occasionally extra-appendix(5 cases),including 3 cases in stomach,1 case in duodenum and 1 case in anal.All 17 cases of appendiceal GCA were pure GCA.Among the 5 cases of extra-appendiceal GCA,One case of gastric GCA was pure,two cases of gastric GCA with NEN or adenocarcinoma,duodenal GCA with NEN and adenocarcinoma,anal GCA with NEN.Low-grade GCAs were composed of goblet,Paneth and neuroendocrine cells,which were arranged in intestinal crypt tubular or cluster structures and distributed in the wall of digestive system.The tubular and cluster structures lacked adhesion.Goblet cells were columnar,located in the base,with clear cytoplasm,small nuclei,inconspicuous atypia,and uncommon mitoses.Extracellular mucus and signet-ring cells with nuclear variations could be seen in some cases.Nerve fiber bundle invasion and tumor thrombus in vessels were often present.High-grade GCAs lacked tubular and cluster structures,and their histological structures were more complex.Tumor cells expressed mixed neuroendocrine and glandular epithelial markers.Similar to the expression patterns of synaptophysin and chromogranin A,CD200 and INSM1 were also dot-like or patch-positive in GCA.Conclusions GCA is an infrequent tumor of the digestive system and shows the bi-directional differentiation characteristics of neuroendocrine and glandular epithelium.Accurate diagnosis and staging are related to its prognosis.