Clinicopathological, Genomic Characteristics and Prognostic Analysis in 18 Cases of SMARCA4-deficient or SMARCA4-mutated Pulmonary Tumors.

Background and objective The diagnosis of SMARCA4-altered pulmonary tumors primarily relies on the detection of protein loss by immunohistochemistry (IHC). However, the concordance between IHC and genetic testing results, as well as the clinical and biological differences caused by various mutation...

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Publicado en:Chinese Journal of Lung Cancer Vol. 29; no. 3; pp. 180 - 190
Autores principales: LIU, Chang, YANG, Jing, MENG, Fanlu, ZHANG, Linlin, WANG, Xin, YU, Tao, ZHONG, Diansheng
Formato: diagnostic images pictorial research tables/charts Journal Article
Publicado: Chinese Journal of Lung Cancer Mar2026
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Mar2026
      vid: 29
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      pub: Chinese Journal of Lung Cancer
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        193720502
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        10.3779/j.issn.1009-3419.2026.106.09
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        atl: Clinicopathological, Genomic Characteristics and Prognostic Analysis in 18 Cases of SMARCA4-deficient or SMARCA4-mutated Pulmonary Tumors.
      aug:
        au:
          LIU, Chang
          YANG, Jing
          MENG, Fanlu
          ZHANG, Linlin
          WANG, Xin
          YU, Tao
          ZHONG, Diansheng
        affil: Department of Oncology; 2Department of Pathology, Tianjin Medical University General Hospital, Tianjin 300052, China
      sug:
        subj:
          Mutation
          Lung Neoplasms Physiopathology
          Lung Neoplasms Familial and Genetic
          Lung Neoplasms Prognosis
          Human
          Male
          Female
          Middle Age
          Aged
          Immunohistochemistry
          Genetic Screening
          Retrospective Design
          Record Review
          Genetic Variation
          Carcinoma, Non-Small-Cell Lung Physiopathology
          Genotype
          Phenotype
          Correlational Studies
          Proteins Metabolism
          Tumor Burden
          Programmed Cell Death Ligand 1 Physiology
          Gene Expression Profiling
          Epidermal Growth Factor Receptors
          Lung Neoplasms Drug Therapy
          Tyrosine Kinase Inhibitors Therapeutic Use
          Neoplasm Staging
          Antineoplastic Agents, Combined Therapeutic Use
          Overall Survival
          Survival Analysis
          Immune Checkpoint Inhibitors Therapeutic Use
          Chemotherapy, Cancer
          Drug Efficacy
          Funding Source
          Middle Aged: 45-64 years
          Aged: 65+ years
          Male
          Female
      ab: Background and objective The diagnosis of SMARCA4-altered pulmonary tumors primarily relies on the detection of protein loss by immunohistochemistry (IHC). However, the concordance between IHC and genetic testing results, as well as the clinical and biological differences caused by various mutation types, require further investigation. This study aimed to explore the clinicopathological, genomic characteristics, as well as the prognosis, of these tumors. Methods A total of 18 consecutive cases of pulmonary tumor patients with SMARCA4 deficiency/mutation confirmed by IHC or genetic testing were retrospectively enrolled for clinical, genomic, and prognostic analysis. Based on the variant type, patients were categorized into Class 1 (protein loss or loss-of-function gene mutations, n=10) and Class 2 (missense mutation or other variants of unknown significance without protein loss, n=8) for intergroup comparison. Results Seventeen cases were diagnosed with nonsmall cell lung cancer (NSCLC) and one was diagnosed with thoracic SMARCA4-deficient undifferentiated tumor (SD-UT). Genotype-phenotype correlation analysis revealed that truncating mutations consistently led to protein loss, whereas missense mutations mostly did not. Two cases with protein loss but negative genetic testing results were identified. Class 1 alterations showed trends towards higher tumor mutational burden (TMB) (median 9.3 vs 4.7 Muts/Mb) and lower programmed cell death ligand 1 (PD-L1) expression (60.0% vs 25.0% with <1% expression) compared to Class 2, but the differences were not statistically significant (P>0.05). Two patients with co-occurring epidermal growth factor receptor (EGFR) L858R mutations showed poor initial response to third-generation EGFR-tyrosine kinase inhibitor monotherapy, but the efficacy was observed after combination with other therapies. Among stage IV patients, the median overall survival (OS) was 10.3 months for Class 1 and 19.9 months for Class 2 (P=0.967). Fourteen patients (77.8%) received immune checkpoint inhibitors (ICIs) combined with chemotherapy. Among them, 8 patients (57.1%) achieved an OS exceeding 12 months, and 7 patients (50.0%) exceeded 24 months. Conclusion SMARCA4 gene mutations are not entirely consistent with IHC protein loss, necessitating combined interpretation. When SMARCA4 alterations co-exist with EGFR mutations, targeted monotherapy efficacy may be limited, and combination strategies should be considered. Classifying SMARCA4 alterations into Class 1 and Class 2 may have prognostic implications and could help predict the tumor immune microenvironment status (TMB and PD-L1). ICIs combined with chemotherapy is currently one of the main treatment options for patients with advanced SMARCA4-altered pulmonary tumors and showed potential efficacy in this study.
      pubtype: Academic Journal
      doctype:
        diagnostic images
        pictorial
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: Chinese
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