Analysis of the Clinicopathological Characteristics, Genetic Phenotypes, and Prognostics of Primary Pulmonary and Bronchial Adenoid Cystic Carcinoma

ABSTRACT Background Primary pulmonary and bronchial adenoid cystic carcinoma (PACC) is a rare, low‐grade malignant tumor of the lung. However, the relationship between its clinical features, surgical prognosis, and genetic phenotype has not been fully described. Methods PACC patient information was...

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Main Authors: Zhengyang Hu, Xing Jin, Jian Wang, Qihai Sui, Yanjun Yi, Dejun Zeng, Zhencong Chen, Qun Wang, Jiacheng Yin, Lin Wang, Zongwu Lin
Format: Article
Language:English
Published: Wiley 2025-01-01
Series:Thoracic Cancer
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Online Access:https://doi.org/10.1111/1759-7714.15526
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author Zhengyang Hu
Xing Jin
Jian Wang
Qihai Sui
Yanjun Yi
Dejun Zeng
Zhencong Chen
Qun Wang
Jiacheng Yin
Lin Wang
Zongwu Lin
author_facet Zhengyang Hu
Xing Jin
Jian Wang
Qihai Sui
Yanjun Yi
Dejun Zeng
Zhencong Chen
Qun Wang
Jiacheng Yin
Lin Wang
Zongwu Lin
author_sort Zhengyang Hu
collection DOAJ
description ABSTRACT Background Primary pulmonary and bronchial adenoid cystic carcinoma (PACC) is a rare, low‐grade malignant tumor of the lung. However, the relationship between its clinical features, surgical prognosis, and genetic phenotype has not been fully described. Methods PACC patient information was collected from the SEER, TCGA, and Zhongshan Hospital, Fudan University (FDZSH) databases. Overall survival (OS) was evaluated using the Kaplan–Meier method. Univariate and multivariate analyses through Cox proportional hazards regression identified risk factors that predicted OS. The limma and matfools packages from R were used to compare the differential genes and mutations between PACC and LUAD, respectively. Results Two hundred and ninety‐two patients, 14 patients, and 12 patients with PACC were identified from the SEER, TCGA, and FDZSH databases, respectively. The 3‐, 5‐, and 10‐year OS of the PACC patients were 91.7%, 88.6%, and 85.0%, respectively, compared to 95.8%, 93.9%, and 93.3% for patients who underwent surgery. Race, pathological grade, M stage, regional node examination, and regional node positive were independent prognostic factors for the OS of patients who underwent surgery. The gene map of PACC and lung adenocarcinoma (LUAD) shows significant differences. Common mutations found in lung cancer were almost undetectable in PACC patients, whereas mutations in the NOTCH pathway were more common. TMB levels and PD‐1/PD‐L1 expressions were also lower in PACC patients. Conclusion Our study analyzed the main factors that influence the prognosis of PACC patients undergoing surgery and discovered the unique genetic phenotype of PACC.
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spelling doaj-art-da43631375f541ce8b4368b20110401c2025-01-30T22:40:34ZengWileyThoracic Cancer1759-77061759-77142025-01-01162n/an/a10.1111/1759-7714.15526Analysis of the Clinicopathological Characteristics, Genetic Phenotypes, and Prognostics of Primary Pulmonary and Bronchial Adenoid Cystic CarcinomaZhengyang Hu0Xing Jin1Jian Wang2Qihai Sui3Yanjun Yi4Dejun Zeng5Zhencong Chen6Qun Wang7Jiacheng Yin8Lin Wang9Zongwu Lin10Department of Thoracic Surgery, Zhongshan Hospital Fudan University Shanghai ChinaDepartment of Thoracic Surgery, Zhongshan Hospital Fudan University Shanghai ChinaDepartment of Thoracic Surgery Shanghai Xuhui Central Hospital Shanghai ChinaDepartment of Thoracic Surgery, Zhongshan Hospital Fudan University Shanghai ChinaDepartment of Thoracic Surgery, Zhongshan Hospital Fudan University Shanghai ChinaDepartment of Thoracic Surgery, Zhongshan Hospital Fudan University Shanghai ChinaDepartment of Thoracic Surgery, Zhongshan Hospital Fudan University Shanghai ChinaDepartment of Thoracic Surgery, Zhongshan Hospital Fudan University Shanghai ChinaDepartment of Thoracic Surgery, Zhongshan Hospital Fudan University Shanghai ChinaDepartment of Thoracic Surgery, Zhongshan Hospital Fudan University Shanghai ChinaDepartment of Thoracic Surgery, Zhongshan Hospital Fudan University Shanghai ChinaABSTRACT Background Primary pulmonary and bronchial adenoid cystic carcinoma (PACC) is a rare, low‐grade malignant tumor of the lung. However, the relationship between its clinical features, surgical prognosis, and genetic phenotype has not been fully described. Methods PACC patient information was collected from the SEER, TCGA, and Zhongshan Hospital, Fudan University (FDZSH) databases. Overall survival (OS) was evaluated using the Kaplan–Meier method. Univariate and multivariate analyses through Cox proportional hazards regression identified risk factors that predicted OS. The limma and matfools packages from R were used to compare the differential genes and mutations between PACC and LUAD, respectively. Results Two hundred and ninety‐two patients, 14 patients, and 12 patients with PACC were identified from the SEER, TCGA, and FDZSH databases, respectively. The 3‐, 5‐, and 10‐year OS of the PACC patients were 91.7%, 88.6%, and 85.0%, respectively, compared to 95.8%, 93.9%, and 93.3% for patients who underwent surgery. Race, pathological grade, M stage, regional node examination, and regional node positive were independent prognostic factors for the OS of patients who underwent surgery. The gene map of PACC and lung adenocarcinoma (LUAD) shows significant differences. Common mutations found in lung cancer were almost undetectable in PACC patients, whereas mutations in the NOTCH pathway were more common. TMB levels and PD‐1/PD‐L1 expressions were also lower in PACC patients. Conclusion Our study analyzed the main factors that influence the prognosis of PACC patients undergoing surgery and discovered the unique genetic phenotype of PACC.https://doi.org/10.1111/1759-7714.15526clinicopathological characteristicsgenetic phenotypesprimary pulmonary and bronchial adenoid cystic carcinomaprognosis
spellingShingle Zhengyang Hu
Xing Jin
Jian Wang
Qihai Sui
Yanjun Yi
Dejun Zeng
Zhencong Chen
Qun Wang
Jiacheng Yin
Lin Wang
Zongwu Lin
Analysis of the Clinicopathological Characteristics, Genetic Phenotypes, and Prognostics of Primary Pulmonary and Bronchial Adenoid Cystic Carcinoma
Thoracic Cancer
clinicopathological characteristics
genetic phenotypes
primary pulmonary and bronchial adenoid cystic carcinoma
prognosis
title Analysis of the Clinicopathological Characteristics, Genetic Phenotypes, and Prognostics of Primary Pulmonary and Bronchial Adenoid Cystic Carcinoma
title_full Analysis of the Clinicopathological Characteristics, Genetic Phenotypes, and Prognostics of Primary Pulmonary and Bronchial Adenoid Cystic Carcinoma
title_fullStr Analysis of the Clinicopathological Characteristics, Genetic Phenotypes, and Prognostics of Primary Pulmonary and Bronchial Adenoid Cystic Carcinoma
title_full_unstemmed Analysis of the Clinicopathological Characteristics, Genetic Phenotypes, and Prognostics of Primary Pulmonary and Bronchial Adenoid Cystic Carcinoma
title_short Analysis of the Clinicopathological Characteristics, Genetic Phenotypes, and Prognostics of Primary Pulmonary and Bronchial Adenoid Cystic Carcinoma
title_sort analysis of the clinicopathological characteristics genetic phenotypes and prognostics of primary pulmonary and bronchial adenoid cystic carcinoma
topic clinicopathological characteristics
genetic phenotypes
primary pulmonary and bronchial adenoid cystic carcinoma
prognosis
url https://doi.org/10.1111/1759-7714.15526
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