胸腺原发性神经内分泌肿瘤临床病理特征

Primary thymic neuroendocrine tumours:a clinicopathological analysis

目的探讨胸腺神经内分泌肿瘤(thymic neuroendocrine tumour,TNET)的临床病理学特征、诊断及鉴别诊断。方法收集16例TNET的临床资料,采用免疫组化EnVision两步法检测CK、CD56、Syn、CgA、Ki-67等的表达,分析其与临床病理特征的关系,并复习相关文献。结果患者年龄35~75岁,中位年龄56岁。临床症状和影像学表现无特异性,影像学显示为前中纵隔占位,为孤立类圆形性结节,边界不定,肿瘤最大径1.5~9.8 cm。病理诊断:1例经典型类癌,10例中级别非典型类癌,1例大细胞神经内分泌癌,4例小细胞癌。免疫表型:CK(16/16),神经内分泌标志物CD56(14/16)、Syn(13/16)和CgA(10/16)均阳性,Ki-67增殖指数为1%~95%。随访时间6~120个月,1例经典型类癌患者仍健在,3例中级别非典型类癌患者发生远处转移,5例高级别神经内分泌癌患者均发生转移而死亡。结论TNET是一类罕见的胸腺恶性肿瘤,具有异质性。TNET确诊依赖于组织病理学、免疫表型及分子表型;TNET精准病理诊断和临床病理分期具有重要意义。

Purpose To investigate the clinicopathological features,diagnosis and differential diagnosis of primary thymic neuroendocrine tumour(TNET).Methods The clinical data of 16 cases of TNET were collected,and the expression of CK,CD56,Syn,CgA,Ki-67,etc.were detected by immunohistochemistry EnVision two step method.The relationship between the expression of the clinicopathological characteristics was analyzed,and the related literatures were reviewed.Results The patient age ranged from 35-75 years old(average age:56 years old).There were no specific clinical symptoms.Images findings were solitary round nodules with unclear edges in the anterior mediastinum.The tumor size was arranged from 1.5-9.8 cm.Pathologically,one case was diagnosed as typical carcinoid(TC),ten cases were diagnosed as atypical carcinoid(AC),one case was diagnosed as large cell neuroendocrine carcinoma(LCNEC),and four cases were diagnosed as small cell carcinoma(SCC).Immumohistochemical staining showed that CK(16/16)were positive,neuroendocrinal markers for CD56(14/16),Syn(13/16)and CgA(10/16)were positive.The Ki-67 index of 16 cases was about 1%-95%.All patients were followed up for 6-120 month,and one patient with TC was survival,three patients with AC were metastasis and five patients with high grade NEC were metastasis and died.Conclusion TNET is a rare heterogenous thymic tumours.The accurate diagnosis of TNET depends on histopathology,immumohistochemistry and molecular phenotype.Accurate pathological diagnosis and clinical pathological staging of TNET are of great significance。