Piórek Aleksandra, Płużański Adam, Kowalski Dariusz M, Krzakowski Maciej
Department of Lung Cancer and Thoracic Tumors, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland.
Cancers (Basel). 2025 Jun 13;17(12):1975. doi: 10.3390/cancers17121975.
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. 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. 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. 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.
胸腺类癌是起源于前纵隔的罕见神经内分泌肿瘤,由于临床表现不具特异性,往往在晚期才得以诊断。与其他高分化神经内分泌肿瘤相比,由于数据匮乏、发病率低以及生物学行为侵袭性强,其治疗仍然具有挑战性。我们对当前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、治疗选择、预后因素以及新兴的实验性疗法。重点在于将近期的分子和治疗进展融入临床实践。手术切除仍然是局限性疾病治疗的基石,而对于晚期病例,依维莫司、生长抑素类似物、铂类化疗和肽受体放射性核素治疗(PRRT)等全身治疗方法可供选择。包括NETest、64Cu-DOTATATE PET和18F-FDOPA PET在内的新型诊断方法在早期检测和疾病监测方面具有前景。分子学见解,特别是涉及MEN1、ATRX和DAXX突变的见解,为个体化靶向治疗铺平了道路。免疫治疗和放射免疫治疗代表了新兴的、尽管仍处于实验阶段的方法。预后在很大程度上取决于肿瘤分期、分化程度、可切除性和功能活性,高复发率需要长期监测。胸腺类癌带来了重大的诊断和治疗挑战。分子分析、新型成像技术和全身治疗的进展为改善治疗结果带来了希望。鉴于该疾病的罕见性,通过登记处和多中心研究持续合作对于完善基于证据的管理策略至关重要。