阑尾杯状细胞腺癌临床病理学观察

目的探讨阑尾杯状细胞腺癌(goblet cell adenocarcinomas,GCA)的临床病理学特征及免疫表型特征,分析其生物学行为。方法收集北京大学第一医院2015—2018年诊断的7例GCA的临床资料,进行形态学观察,按照2019年WHO消化系统肿瘤分类第5版重新分级,并行免疫组织化学EnVision法染色及电镜观察,随访患者并复习相关文献。结果7例患者中男性2例,女性5例;年龄48~72岁,中位年龄58岁。1例为阑尾游离端出现局限性肿物,切面灰白实性,质地中等;其余6例阑尾弥漫增粗;镜下观察均可见低级别GCA,5例同时伴有高级别GCA,3例分级为1级,3例分级为2级及1例分级为3级。6例可见神经侵犯,1例可见脉管癌栓。免疫组织化学染色嗜铬粒素A、突触素、CD56、广谱细胞角蛋白、癌胚抗原、细胞角蛋白20、MLH1、MSH2、MSH6和PMS2均阳性,Ki-67阳性指数5%~50%,p53在高级别GCA的表达强度及阳性细胞数高于低级别GCA。随访14~46个月,6例患者生存,1例患者死亡。结论GCA为阑尾较少见的肿瘤,同时具有神经内分泌及腺上皮的分化,生物学行为从惰性到高度侵袭性均可。

Clinicopathological characteristics of goblet cell adenocarcinoma of the appendix

Objective To investigate the clinicopathological characteristics of goblet cell adenocarcinoma(GCA)of the appendix.Methods Seven cases of GCA were collected at the First Hospital of Peking University,Beijing,China from 2015 to 2018.Hematoxylin and eosin staining and immunohistochemical studies(EnVision method)were carried out on all cases.Three cases were examined using electron microscopy.Results Based on the review of 7 patients with GCA,the mean age of presentation for GCA of the appendix was 58 years with 2 men and 5 women,including 3 cases of grade 1,3 cases of grade 2 and 1 case of grade 3 GCAs.Six GCAs had a prominent pattern of submucosal growth and lacked the formation of a well-defined tumor border.One of the 7 tumors was located in the distal segment of the appendix.Perineural involvement and lymphatic invasion were seen in 6 tumors and 1 tumor,respectively.Immunohistochemically,the tumor cells were diffusely or focally positive for chromogranin A,synaptophysin,CD56,cytokeratin,carcinoembryonic antigen,cytokeratin 20,MLH1,MSH2,MSH6 and PMS2,while the Ki-67 index ranged from 5%to 50%.The expression levels and positive cell numbers of p53 in high-grade GCAs were higher than those in low-grade GCAs.In the 7 patients with available follow-up information(14?46 months),1 patient died and the other 6 were alive at the end of the follow-up.Conclusions GCA of the appendix is a rare neoplasm that shares histological features of both adenocarcinoma and carcinoid tumor.Its biological behavior ranges from indolent to highly aggressive.