肺转移性产黏液内分泌汗腺癌的临床病理学观察

Clinicopathological features of metastatic endocrine mucin⁃producing sweat gland carcinoma in lung

目的探讨产黏液内分泌汗腺癌(EMPSGC)的临床病理特征、诊断及鉴别诊断。方法回顾性分析1例肺转移性EMPSGC患者的临床病理特征及免疫表型,并复习相关文献。结果患者男性,53岁,10年前患有左侧腋窝皮肤汗腺腺样囊性癌病史。患者3个月前,无明显诱因的出现咳嗽、咳痰症状,支气管镜活检标本病理组织学示:正常的支气管黏膜下方见肿瘤组织,肿瘤细胞呈实性、筛孔状结构,异型性不明显;瘤细胞内及细胞外见少量黏液;未见坏死。免疫组化示瘤细胞CK(P)、EMA、GATA3、AR、ER、PR、Mucin⁃2弥漫强阳性,INSM1中等阳性,Mammaglobin和GCDFP⁃15部分阳性,CK7、CK20、TTF⁃1、NapsinA、P63、Syn、CgA、S⁃100、CD117、Dog1和Pan⁃TRK均阴性,Ki⁃67增殖指数约10%。结论EMPSGC是一种少见的表达神经内分泌标记的低度恶性的汗腺肿瘤,可局部复发,极少数病例可发生远处转移。目前,仍需要更多的病例研究来探讨其复发风险因素及远处转移发生率。

Objective To investigate the clinicopathological features,diagnosis and differential diagnosis of endocrine mucin⁃producing sweat gland carcinoma(EMPSGC).Methods The clinicopathological features and immunophenotype of a patient with lung metastatic EMPSGC were analyzed retrospectively,and the related literature was reviewed.Results The patient was a 53⁃year⁃old male with a history of left axillary sweat gland adenoid cystic carcinoma for 10 years.Three months ago,the patient began to present symptoms of cough and sputum without obvious causes.The histopathology of the bronchoscopic biopsy specimen showed that tumor tissue was found below the normal bronchial mucosa,and the tumor cells were solid and sieve shaped,with no obvious atypia.A small amount of mucous was found inside and outside the tumor cells.There was no necrosis.Immunohistochemistry showed strongly diffuse positive for CK(P),EMA,GATA3,AR,ER,PR,Mucin⁃2,moderately positive for INSM1,partial positive for mammaglobin and GCDFP⁃15.CK7,CK20,TTF⁃1,NapsinA,P63,Syn,CgA,S⁃100,CD117,Dog1 and Pan⁃TRK were negative,and the proliferation index of Ki⁃67 was about 10%.Conclusion EMPSGC is a rare low⁃grade malignant sweat gland tumor expressing neuroendocrine markers.It may recur locally,and distant metastasis in rare cases.At present,more case studies are needed to investigate the risk factors for recurrence and the incidence of distant metastasis.