首页|11例原发性肺黏液表皮样癌的临床病理分析

11例原发性肺黏液表皮样癌的临床病理分析

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目的 探讨原发性肺黏液表皮样癌(PMEC)的临床病理及分子特征.方法 回顾性分析 11 例 2005 年 1 月至2021 年 12 月经手术切除并确诊为PMEC患者的临床病理资料,并复习相关文献.结果 11 例患者中,男性 7 例,女性4 例;年龄 19~58 岁,中位年龄33 岁;肿瘤均发生于段及段以上支气管,临床以支气管刺激症状为主.胸部CT显示10 例为边界尚清晰的类圆形结节,1 例显示为不规则肿块,5 例伴有浅分叶.11 例肿块增强扫描后均显示轻-中度的不均匀强化,肿块内有钙化者 5 例.1 例组织学分级为高级别PMEC,10 例为低级别PMEC.免疫组织化学染色结果显示,瘤细胞CK7、p63 及p40 阳性,TTF1、NapsinA、CK20 和 SMA 阴性,增殖指数 Ki-67 约 4%~28%.特殊染色示黏液细胞 PAS 阳性.6 例患者行MAML2 的FISH检测,4 例患者显示MAML2 基因重排阳性.11 例患者均行手术治疗,其中 3 例术后辅助化疗.8 例患者随访8~144 个月,1 例高级别PMEC复发.结论 PMEC是一种罕见肿瘤,其诊断需结合病理组织学特点和免疫组化,FISH检测到MAML2 基因易位,主要依据术后组织病理,手术是治疗的主要方法,其预后与病理分级及临床分期相关.
Clinical and pathological analysis of primary pulmonary mucoepidermoid carcinoma
Objective To investigate the clinicopathological and molecular features of pulmonary mucoepidermoid carcinoma(PMEC).Methods The clinicopathological data of 11 cases pathologically proven PMEC from January 2005 to December 2021 were analyzed retrospectively.The clinicopathological features were discussed and related literatures were reviewed.Results Among the 11 patients,there were 7 males and 4 females,with a median age of 33 years(range 19-58 years).All the tumors occurred in the segmental and above-segmental bronchi,and the main clinical symptoms were bronchial irritation.CT in chest showed well-defined round nodules in 10 cases,irregular mass in 1 case and shallow lobulation in 5 cases.After contrast-enhanced scanning,the tumors showed mild to moderate inhomogeneous enhancement and calcification in 5 cases.One case was histologically classified as high-grade PMEC and the others were low-grade PMEC.Immunohistochemistry showed that the tumor cells were positive for CK7,p63 and p40,but negative for TTF1,NapsinA,CK20 and SMA.The Ki-67 index was about 4%-28%.Special staining showed that mucous cells were PAS positive.MAML2 was detected in 6 patients,and MAML2 gene rearrangement was positive in 4 patients.All the 11 patients were treated by operation and 3 patients were treated with adjuvant chemotherapy after operation.During the follow-up from 8 months to 144 months,1 case of high-grade PMEC recurred.Conclusion PMEC is an extremely rare pulmonary malignant tumor,and the diagnosis mainly depends on postoperative histopathology.CRTC1/MAML2 translocation is detected by FISH.Surgery is the main method of treatment,and its prognosis is related to pathological grade and clinical stage.

Lung cancerMucoepidermoid carcinomaPathologyClinical featuresTreatmentPrognosis

聂小、刘益飞、毛海峰、王伟、张青、刘颖

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214400 江苏江阴 江阴市人民医院病理科

226001 南通大学附属医院病理科

肺癌 黏液表皮样癌 病理 临床特征 治疗 预后

2024

临床肿瘤学杂志
解放军第八一医院

临床肿瘤学杂志

CSTPCD
影响因子:1.583
ISSN:1009-0460
年,卷(期):2024.29(2)
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