中国肺癌杂志2025，Vol.28Issue(6)：441-449,9.DOI:10.3779/j.issn.1009-3419.2025.101.10
原发性肺黏液表皮样癌临床特征、MAML2基因重排和预后分析
Clinical Characteristics,MAML2 Gene Rearrangement and Prognosis of Pulmonary Mucoepidermoid Carcinoma
摘要
Abstract
Background and objective Primary pulmonary mucoepidermoid carcinoma(PMEC)is an exceeding-ly rare malignancy originating from bronchial submucosal glands,accounting for＜0.2％of lung cancers.Histologically charac-terized by a triphasic composition of mucinous,epidermoid,and intermediate cells,PMEC is classified into low-grade(favorable prognosis)and high-grade(aggressive behavior)subtypes.This study aimed to investigate the clinicopathological character-istics and prognostic indicators of PMEC.Methods Clinicopathological,radiological,molecular,and survival data from 26 PMEC patients were retrospectively analyzed,including immunohistochemical profiles and MAML2 rearrangement status,supplemented by literature review.Results The cohort comprised 14 males and 12 females(mean age:55.6 years).Eight pa-tients(30.8％)were smokers,and 19(73.1％)presented with symptoms.Central tumors predominated(n=19,73.1％)versus peripheral lesions(n=7,26.9％).Computed tomography(CT)imaging consistently revealed hypo-to-isodense masses/nodules.Pathologically,19 cases were low-grade and 7 high-grade.Immunohistochemically,the tumor cells were positive for CK7,P40,P63 and CK5/6,and the Ki-67 index ranged from 2％to 70％.MAML2 rearrangement was detected in 52.4％(11/21)of tested cases.Clinical staging distribution:stage Ⅰ(n=14),stage Ⅱ(n=8),stage Ⅲ(n=3),stage Ⅳ(n=1).Treatment modalities:radical surgery alone(n=13),surgery with adjuvant chemotherapy(n=11),chemoradiotherapy(n=1),and conservative management(n=1).With a median follow-up of 57 months,6 patients(23.1％)died.Prognostic analysis demonstrated:(1)Significantly inferior survival in high-grade versus low-grade groups(P＜0.05);(2)Lymph node metastasis,advanced stage,Ki-67＞20％,and high-grade histology significantly correlated with reduced overall survival(P＜0.05);(3)Lymph node metastasis constituted an independent poor prognostic factor(HR=12.73,95％CI:1.22-132.96).Conclusions PMEC exhibits distinct clinicopatho-logical features,with MAML2 rearrangement present in approximately half of cases.Lymph node metastasis,advanced stage,high Ki-67 proliferation index,and high-grade histology are key determinants of poor prognosis,with lymph node metastasis serving as an independent risk factor.关键词
肺黏液表皮样癌/分级/预后/MAML2基因重排Key words
Pulmonary mucoepidermoid carcinoma/Grade/Prognosis/MAML2 gene rearrangement引用本文复制引用
白建荣,阎萌,郭凌川,雷哲,刘卫硕,邹子归,李娇,郑玉双..原发性肺黏液表皮样癌临床特征、MAML2基因重排和预后分析[J].中国肺癌杂志,2025,28(6):441-449,9.基金项目
本研究受江苏省自然科学基金面上项目(No.BK20241793)资助 This study was supported by the grant from the Natural Science Foundation of Jiangsu Province(No.BK20241793)(to Lingchuan GUO). （No.BK20241793）
