Clinical Characteristics, MAML2 Gene Rearrangement and Prognosis
of Pulmonary Mucoepidermoid Carcinoma.
10.3779/j.issn.1009-3419.2025.101.10
- Author:
Jianrong BAI
1
;
Meng YAN
1
;
Lingchuan GUO
1
;
Zhe LEI
1
;
Weishuo LIU
1
;
Zigui ZOU
1
;
Jiao LI
1
;
Yushuang ZHENG
1
Author Information
1. Department of Pathology, The First Affiliated Hospital of Soochow University, Suzhou 215006, China.
- Publication Type:Journal Article
- Keywords:
Grade;
MAML2 gene rearrangement;
Prognosis;
Pulmonary mucoepidermoid carcinoma
- MeSH:
Humans;
Male;
Female;
Middle Aged;
Carcinoma, Mucoepidermoid/mortality*;
Lung Neoplasms/mortality*;
Trans-Activators/genetics*;
Prognosis;
Adult;
Gene Rearrangement;
Aged;
Retrospective Studies;
Transcription Factors/genetics*;
DNA-Binding Proteins/genetics*
- From:
Chinese Journal of Lung Cancer
2025;28(6):441-449
- CountryChina
- Language:Chinese
-
Abstract:
BACKGROUND:Primary pulmonary mucoepidermoid carcinoma (PMEC) is an exceedingly rare malignancy originating from bronchial submucosal glands, accounting for <0.2% of lung cancers. Histologically characterized 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 characteristics 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 patients (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 clinicopathological 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.
