肺原发涎腺型肿瘤临床病理学观察

Clinicopathological features of lung salivary gland-type tumors

目的 探讨肺原发涎腺型肿瘤(salivary gland-type tumors,SGT)的临床病理特点,与涎腺原发的相应肿瘤进行对比.方法 收集北京大学第一医院2004年1月至2018年9月收治的肺原发SGT病例共23例,对其组织形态特点、免疫表型、基因型和预后进行全面的研究.结果 23例SGT患者中男性13例,女性10例,年龄13~79岁,中位年龄54岁.23例SGT包括腺样囊性癌11例,黏液表皮样癌10例,透明细胞癌1例,肌上皮瘤1例,其组织形态和免疫表型均与涎腺原发者具有高度相似性.11例腺样囊性癌中1例检测到MYB基因易位.10例黏液表皮样癌均检测到MAML2基因易位.1例透明细胞癌检测到EWSR1基因易位.随访显示,3例腺样囊性癌生存时间大于60个月,1例生存时间52个月,3例生存时间12~36个月.7例有随访结果的黏液表皮样癌,均无复发和死亡.1例透明细胞癌随访52个月复发.结论 肺原发SGT和涎腺原发的相应肿瘤,虽然两者在组织形态、免疫表型、基因型和预后诸多方面相似,但亦存在差异.涎腺原发腺样囊性癌大多存在MYB基因易位,而肺原发腺样囊性癌则仅有罕见病例检测到MYB基因易位;肺原发黏液表皮样癌患者预后好于涎腺原发者,肺原发腺样囊性癌患者预后差于涎腺原发者.

Objective To study the clinicopathological characteristics of lung salivary gland-type tumors(SGT),and to compare with the corresponding primary SGT in salivary glands.Methods Twenty-three cases of lung SGT were retrieved from the files of Peking University First Hospital from January 2004 to September 2018.The morphology,immunophenotype,genotype and outcome of these cases were analyzed.Results The 23 patients included 13 males and 10 females,with age range of 13-79 years(median 54 years).There were 11 cases of adenoid cystic carcinoma,10 cases of mucoepidermoid carcinoma(MEC),one case each of clear cell carcinoma and myoepithelioma.The morphology and immunophenotype of lung SGT were very similar to their counterparts in salivary gland.MYB rearrangement was detected in one of 11 adenoid cystic carcinomas.MAML2 rearrangement was detected in all the MECs.EWSR1 rearrangement was detected in the one case of clear cell carcinoma.Of patients with adenoid cystic carcinoma,the survival time was more than 60 months(three cases),52 months(one case),and 12-36 months(three cases).There was no recurrence and death in seven cases of MEC with follow-up results.One case of clear cell carcinoma recurred after 52 months of follow-up.Conclusions Although the SGT of lung and their counterparts in salivary gland are very similar in their morphology,immunophenotype,genotype and prognosis,there are also some differences between each other.MYB rearrangement can be detected in most adenoid cystic carcinomas of salivary gland,but rarely in lung adenoid cystic carcinoma.The prognosis of patients with lung MEC is better than that of patients with salivary gland MEC,while the prognosis of patients with lung adenoid cystic carcinoma is worse than that of patients with salivary gland adenoid cystic carcinoma.