SMARCA4-deficient undifferentiated thoracic tumor is extremely invasive. This tumor with poor prognosis is easily confused with SMARCA4-deficent non-small cell lung cancer or sarcoma. Standard and efficient treatment has not been established. In this review, we summarized the etiology, pathogenesis and diagnosis, reviewed current and proposed innovative strategies for treatment and improving prognosis. Immunotherapy, targeting tumor microenvironment and epigenetic regulator have improved the prognosis of cancer patients. We summarized clinicopathological features and immunotherapy strategies and analyzed the progression-free survival (PFS) and overall survival (OS) of patients with SMARCA4-UT who received immune checkpoint inhibitors (ICIs). In addition, we proposed the feasibility of epigenetic regulation in the treatment of SMARCA4-UT. To our knowledge, this is the first review that aims to explore innovative strategies for targeting tumor microenvironment and epigenetic regulation and identify potential benefit population for immunotherapy to improve the prognosis.

SMARCA4 缺陷的未分化胸部肿瘤具有极高的侵袭性。这种预后不良的肿瘤很容易与SMARCA4缺陷的非小细胞肺癌或肉瘤混淆。尚未建立标准和有效的治疗方法。在这篇综述中，我们总结了其病因、发病机制和诊断，回顾了当前的情况并提出了治疗和改善预后的创新策略。针对肿瘤微环境和表观遗传调节剂的免疫治疗改善了癌症患者的预后。我们总结了接受免疫检查点抑制剂（ICIs）治疗的 SMARCA4-UT 患者的临床病理特征和免疫治疗策略，并分析了无进展生存期（PFS）和总生存期（OS）。此外，我们提出了表观遗传调控在SMARCA4-UT治疗中的可行性。据我们所知，这是第一篇旨在探索针对肿瘤微环境和表观遗传调控的创新策略，并确定免疫治疗的潜在受益人群以改善预后的综述。