Abstract
The mesenchymal-epithelial transition exon 14 (METex14) skipping mutation is a driver of non-small cell lung cancer (NSCLC). MET tyrosine kinase inhibitors (TKIs), such as tepotinib, are effective against METex14 skipping-positive NSCLC but carry a risk of acute exacerbation of interstitial lung disease (AE-ILD) in patients with idiopathic pulmonary fibrosis (IPF). Here, we present a case of a 54-year-old man with IPF and METex14 skipping-positive lung adenocarcinoma. Following first-line platinum-based chemotherapy, disease control was not achieved for either lung cancer or IPF. Therefore, the patient underwent second-line treatment with tepotinib and the antifibrotic agent nintedanib. This combination effectively controlled both lung cancer and IPF, resulting in a partial response without AE-ILD. Our case suggests that nintedanib treatment may offer a safe and effective option for progressive IPF during MET-TKI therapy, presenting a novel therapeutic approach for patients with lung cancer complicated by IPF.