阑尾杯状细胞腺癌临床病理学观察

Appendiceal goblet cell adenocarcinoma:a clinicopathologic analysis

目的探讨阑尾杯状细胞腺癌(GCA)的临床病理学特点。方法收集8例手术切除GCA的患者病例,总结其临床资料、组织学形态、免疫组化表型、特殊染色及随访情况,并按照WHO消化系统肿瘤第5版(2019)重新进行组织学分级,结合文献对该疾病进行讨论。结果8例GCA中,女性4例,男性4例,年龄范围47~74岁。大体检查3例回盲部可见肿物,1例阑尾增粗,4例阑尾无显著改变;镜下肿瘤在阑尾壁内呈浸润性生长,间质反应轻微,低级别成分由杯状细胞样的黏液细胞、数量不等的内分泌细胞以及潘氏细胞组成类似肠隐窝、小管及簇状结构,高级别成分则呈单个、条索状及片状结构,可见印戒细胞样细胞;低级别及高级别均见细胞外黏液;组织学分级6例G1,1例G2,1例G3;5例见神经侵犯;1例广泛淋巴结转移并大网膜内转移。免疫组化结果:8例均表达CEA,局灶或弥漫表达Syn、CgA、CD56;5例弥漫或部分表达CK20,3例CK20阴性,所有病例CK7均阴性;错配修复蛋白MLH1、PSM2、MSH2及MSH6均呈阳性表达,6例行AB-PAS特殊染色均显示胞内黏液阳性。结论GCA是一种罕见的特殊类型阑尾恶性肿瘤,具有独特的组织学特征,正确诊断该疾病对临床治疗及判断预后有重要意义。

Objective To investigate the clinicopathologic characteristics of the Appendiceal goblet cell adenocarcinoma(GCA).Methods Eight cases of GCA diagnosed in our hospital from 2013 to 2021 were reviewed for clinical related information,histopathology,immunohistochemical markers(CgA,Syn,CD56,CEA,CK7,CK20,MLH1,PSM2,MSH2,MSH6 and Ki-67;AB-PAS)and prognosis,performed histological classification according to the latest WHO classification.Results The 8 cases of GCA included 4 females and 4 males with age range 47~74 yeas.Tumor cells invaded into the appendix wall,without eliciting a stromal reaction.Low-grade histology features exhibited tubular or clustered patterns comprising a mixture of goblet-like mucus cells,cuboidal cells and Paneth-like cells,whereas high-grade exhibited single cells,single file,sheet-like growth of tumor cells;Either low grade or high grade presented extracellular mucus;5 of 8 cases disclosed perineural invasive.By immunohistochemistry,GCA showed focal and patchy expression of neuroendocrine markers,synaptophysin(Syn),chromogranin(CgA)and neural cell adhesion molecule(NCAM/CD56).In addition,all cases tumor cells showed diffuse positivity for CEA,5 cases showed partly or diffuse CK20 positivity.CK7 were negative for all cases,all cases were absence of microsatellite instability with retained nuclear expression of MLH1,PMS2,MSH2 and MSH6.Conclusion GCA of the appendix is a rare and distinctive neoplasm,and correct diagnosis is important to guide clinical treatments and judge prognosis.