Özçıbık Işık Gizem, Turna Akif
Department of Thoracic Surgery, Istanbul University-Cerrahpaşa, Cerrahpaşa Medical Faculty, Istanbul, Türkiye.
Front Surg. 2024 Nov 18;11:1467789. doi: 10.3389/fsurg.2024.1467789. eCollection 2024.
Thymic epithelial tumors originate from the epithelial cells of the thymus and are typically diagnosed during the 5th and 6th decades of life. The incidence is consistent between men and women, averaging 1.7 cases per year. Thymomas, neuroendocrine tumors, and thymic carcinomas are subtypes of thymic epithelial tumors, with thymomas being the most prevalent (75%-80%) and thymic carcinomas following at 15%-20%. Thymoma and thymic carcinoma exhibit distinct disease courses; thymomas grow slowly and are confined to the thymus, while thymic carcinomas demonstrate rapid growth and metastasis. Overall survival rates vary, with a 78% 5-year survival rate for thymoma and a 30% rate for thymic carcinoma. Thymic epithelial tumors may be linked to paraneoplastic autoimmune diseases, including myasthenia gravis, hypogammaglobulinemia, pure red cell aplasia, Cushing's syndrome, systemic lupus erythematosus, and polymyositis. Staging of thymic epithelial tumors can be done according to Masaoka-Koga and/or TNM 8th staging systems. The treatment algorithm is primarily determined by resectability, with surgery (Extended Thymectomy) serving as the foundational treatment for early-stage patients (TNM stage I-IIIA, Masaoka-Koga stage I-III). Adjuvant radiotherapy or chemotherapy may be considered following surgery. In advanced or metastatic cases, chemotherapy is the first-line treatment, followed by surgery and radiotherapy for local control. Myasthenia gravis, an autoimmune disease presents with progressive muscle fatigue and diplopia. Positive antibodies (Anti-AChR, Anti-MuSK, LRP4) and electromyography aid in diagnosis, and approximately 10% of myasthenia gravis patients can also have thymoma. Treatment includes cholinesterase inhibitors and immunotherapy agents, with extended thymectomy serving as an effective surgical option for drug-resistant cases. Minimally invasive approaches (video-assisted thoracoscopic surgery or robot-assisted thoracoscopic surgery) have demonstrated comparable oncological outcomes to sternotomy, highlighting their effectiveness and reliability.
胸腺瘤起源于胸腺的上皮细胞,通常在50至60岁之间被诊断出来。男性和女性的发病率一致,平均每年1.7例。胸腺瘤、神经内分泌肿瘤和胸腺癌是胸腺瘤的亚型,其中胸腺瘤最为常见(75%-80%),胸腺癌次之,占15%-20%。胸腺瘤和胸腺癌表现出不同的病程;胸腺瘤生长缓慢,局限于胸腺,而胸腺癌则生长迅速并发生转移。总体生存率各不相同,胸腺瘤的5年生存率为78%,胸腺癌为30%。胸腺瘤可能与副肿瘤性自身免疫性疾病有关,包括重症肌无力、低丙种球蛋白血症、纯红细胞再生障碍性贫血、库欣综合征、系统性红斑狼疮和多发性肌炎。胸腺瘤的分期可根据Masaoka-Koga和/或TNM第8版分期系统进行。治疗方案主要取决于可切除性,手术(扩大胸腺切除术)是早期患者(TNM分期I-IIIA,Masaoka-Koga分期I-III)的基础治疗方法。术后可考虑辅助放疗或化疗。在晚期或转移性病例中,化疗是一线治疗方法,随后进行手术和放疗以控制局部病变。重症肌无力是一种自身免疫性疾病,表现为进行性肌肉疲劳和复视。阳性抗体(抗乙酰胆碱受体、抗肌肉特异性酪氨酸激酶、低密度脂蛋白受体相关蛋白4)和肌电图有助于诊断,约10%的重症肌无力患者也可能患有胸腺瘤。治疗包括胆碱酯酶抑制剂和免疫治疗药物,扩大胸腺切除术是耐药病例的有效手术选择。微创方法(电视辅助胸腔镜手术或机器人辅助胸腔镜手术)已证明其肿瘤学结果与胸骨切开术相当,突出了其有效性和可靠性。