Background:Thymic carcinoids are rare neuroendocrine tumors arising in the anterior mediastinum, often diagnosed at an advanced stage due to nonspecific clinical manifestations. Their management remains challenging because of the paucity of data, rarity of occurrence, and aggressive biological behavior compared to other well-differentiated neuroendocrine neoplasms.Methods:We conducted a comprehensive review of the current literature focusing on the classification, clinical presentation, diagnostics, treatment options, prognostic factors, and emerging experimental therapies for thymic carcinoids. Emphasis was placed on integrating recent molecular and therapeutic advances into clinical practice.Results:Surgical resection remains the cornerstone of treatment for localized disease, while systemic therapies such as everolimus, somatostatin analogs, platinum-based chemotherapy, and peptide receptor radionuclide therapy (PRRT) are options for advanced cases. Novel diagnostic modalities, including NETest, 64Cu-DOTATATE PET, and 18F-FDOPA PET, offer promise in early detection and disease monitoring. Molecular insights, particularly involving MEN1, ATRX, and DAXX mutations, pave the way for individualized targeted therapies. Immunotherapy and radioimmunotherapy represent emerging, albeit still experimental, approaches. Prognosis largely depends on tumor stage, differentiation, resectability, and functional activity, with a high recurrence rate necessitating prolonged surveillance.Conclusions:Thymic carcinoids pose significant diagnostic and therapeutic challenges. Advances in molecular profiling, novel imaging techniques, and systemic therapies offer hope for improved outcomes. Given the disease rarity, continued collaboration through registries and multicenter studies is essential to refine evidence-based management strategies.
背景：胸腺类癌是一种罕见的神经内分泌肿瘤，起源于前纵隔，由于其临床表现缺乏特异性，常于晚期才被确诊。相较于其他高分化神经内分泌肿瘤，胸腺类癌因数据匮乏、发病率低且生物学行为更具侵袭性，其临床管理仍面临挑战。 方法：我们对现有文献进行了全面综述，重点关注胸腺类癌的分类、临床表现、诊断方法、治疗方案、预后因素以及新兴实验性疗法。重点在于将最新的分子学进展与治疗进展整合到临床实践中。 结果：手术切除仍是局限性病变治疗的基石，而对于晚期病例，可选择依维莫司、生长抑素类似物、铂类化疗及肽受体放射性核素治疗等全身性疗法。新型诊断模式，如NETest、64Cu-DOTATATE PET和18F-FDOPA PET，为早期检测和疾病监测提供了希望。分子层面的深入认识，特别是涉及MEN1、ATRX和DAXX基因突变的研究，为个体化靶向治疗铺平了道路。免疫疗法和放射免疫疗法代表了新兴但尚处实验阶段的研究方向。预后主要取决于肿瘤分期、分化程度、可切除性及功能活性，其高复发率要求进行长期监测。 结论：胸腺类癌在诊断和治疗上均构成重大挑战。分子谱分析、新型影像技术和全身性疗法的进步为改善患者预后带来了希望。鉴于该疾病的罕见性，通过登记系统和多中心研究持续开展合作，对于完善基于证据的管理策略至关重要。
肿瘤(癌症)患者之家