Thymic carcinoma (TC) is a rare, aggressive cancer that originates from thymus’s epithelial cells. It distinguishes itself from other thymic epithelial tumors with its unique pathological structure, clinical behavior, and immune characteristics. Immune checkpoint inhibitors (ICIs) targeting the Programmed cell death protein 1/Programmed cell death protein ligand 1 (PD-1/PD-L1) pathway have shown promise in advanced TC, potentially benefiting from frequent PD-L1 overexpression and abundant CD8+tumor-infiltrating lymphocytes (TILs), despite typically low tumor mutational burden (TMB). While ICI monotherapy can achieve disease control in some patients, its overall efficacy is limited and it is associated with a distinct profile of immune-related adverse events (irAEs) which occur less often than in thymomas. The predictive value of biomarkers—particularly PD-L1 expression—remains uncertain, underscoring the importance of consistent assessment criteria. In this review, we summarize evidence on ICI monotherapy as well as combination approaches that incorporate anti-angiogenic agents, chemotherapy, or dual checkpoint blockade. Emerging therapeutic targets—such as CD70, TIM-3, and B7-H4—are also considered in the context of their potential clinical relevance. Finally, we discuss future directions aimed at improving efficacy, extending response durability, and reducing treatment-related toxicity through biomarker-based patient selection and tailored therapeutic strategies.
胸腺癌是一种罕见且具有侵袭性的恶性肿瘤，起源于胸腺上皮细胞。其独特的病理结构、临床行为及免疫特征使其区别于其他胸腺上皮肿瘤。针对程序性细胞死亡蛋白1/程序性细胞死亡蛋白配体1通路的免疫检查点抑制剂在晚期胸腺癌治疗中展现出潜力，这可能得益于该肿瘤常出现PD-L1过表达及丰富的CD8+肿瘤浸润淋巴细胞，尽管其肿瘤突变负荷通常较低。虽然单药免疫检查点抑制剂可使部分患者实现疾病控制，但其总体疗效有限，且伴随特有的免疫相关不良事件谱，其发生率低于胸腺瘤。生物标志物（尤其是PD-L1表达）的预测价值仍不明确，凸显了统一评估标准的重要性。本文综述了单药免疫检查点抑制剂疗法以及联合抗血管生成药物、化疗或双检查点阻断等综合治疗策略的证据。同时探讨了CD70、TIM-3和B7-H4等新兴治疗靶点潜在的临床意义。最后，我们讨论了未来通过基于生物标志物的患者选择和个体化治疗策略来提高疗效、延长应答持续时间及降低治疗相关毒性的发展方向。
肿瘤(癌症)患者之家