Abstract
Integrase interactor 1 (INI1)-deficient lung cancer is extremely rare, often with poor prognosis, and lacks effective treatment. Previous studies have reported the efficacy of immunotherapy and enhancer of the zeste homolog 2 (EZH2) inhibitor tazemetostat in various types of INI1-deficient tumors, such as sarcomas. However, the effectiveness of these treatments in INI1-deficient lung cancer has not yet been verified. We hereby report a case of a patient who was diagnosed with advanced squamous lung cancer with INI1 deficiency and received chemotherapy, immunotherapy, and tazemetostat treatments successively. The patient showed optimal response in the initial chemotherapy combined with anti-programmed cell death protein 1 (PD-1) immunotherapy, made rapid progress in the subsequent stage of maintenance immunotherapy, and showed nonresponse to tazemetostat. To the best of our knowledge, this is the first case of a lung cancer patient with INI1 deficiency who received tazemetostat treatment.