胸腺上皮肿瘤患者合并自身免疫性疾病的趋势

Aneesha Anand, Nikhitha Lavu, Kenneth A. Kesler, Patrick J. Loehrer
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摘要

胸腺上皮肿瘤(TET)是一种罕见的恶性肿瘤,起源于前纵隔的胸腺。TET包括胸腺癌和胸腺瘤。约 30-40% 的胸腺瘤伴有自身免疫性副肿瘤疾病,其中最常见的是重症肌无力。此外,还有其他多种副肿瘤综合征的报道。目前,人们对合并自身免疫性疾病的胸腺瘤患者的人口学或组织学趋势知之甚少。在这项单一机构的回顾性病历审查中,我们评估了IU西蒙癌症中心(IUSCC)胸腺瘤相关副肿瘤综合征的分布情况,以确定人口统计学和组织学特征方面的趋势。我们创建了2000-2023年期间IUSCC患者的数据库,并确定了170名经活检证实患有恶性TET和相关自身免疫性疾病的患者。数据被导出到 excel 和 R 中进行分析。分析的因素包括:诊断时的年龄、出生时的性别、体重指数、WHO(世界卫生组织)分类和正冈分期。总生存率还与无副肿瘤综合征的匹配对照组进行了比较。共发现37种不同的副肿瘤综合征与IUSCC患者的胸腺瘤有关。最常见的是重症肌无力(110 例),其次是甲状腺功能减退症(21 例,其中 5 例确诊为桥本氏甲状腺炎)、良好综合征(19 例)和纯红细胞增生症(15 例)。重要发现包括36.4%的副肿瘤合并症患者伴有1种以上的副肿瘤综合征,51.8%的患者为IV期疾病,40.4%的患者为WHO B2型肿瘤病理,其次为B3型肿瘤(25%)。没有发现明显的人口统计学关联。有副肿瘤综合征的 TET 患者的 10 年生存率与无副肿瘤综合征的患者无明显差异(P= 0.721)。这些结果表明,胸腺瘤分期和分级与副肿瘤性疾病的发生之间可能存在关联。有必要使用更大的数据集进行进一步分析。血清和血液检测分析也可阐明胸腺瘤患者发生副肿瘤性疾病的原因。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Trends in Thymic Epithelial Tumor Patients with Comorbid Autoimmune Disease
Thymic epithelial tumors (TETs) are rare malignancies originating from the thymus in the anterior mediastinum. TETs include thymic carcinoma and thymoma. Approximately 30-40% of thymomas have associated autoimmune paraneoplastic disorders, the most common being myasthenia gravis. A broad range of other paraneoplastic syndromes have also been reported. Currently, little is known about demographic or histological trends in thymoma patients with comorbid autoimmune disease. In this single institution retrospective chart review, we assessed the distribution of thymoma-associated paraneoplastic syndromes at the IU Simon Cancer Center (IUSCC) to identify trends within demographic and histological features. We created a database of IUSCC patients seen from 2000-2023 and identified 170 subjects with biopsy-proven malignant TET and associated autoimmune disease. Data was exported to excel and R for analysis. Factors analyzed included: age at diagnosis, sex assigned at birth, BMI, WHO (World Health Organization) classification, and Masaoka staging. Overall survival was also compared to matched controls without paraneoplastic syndrome. A total of 37 different paraneoplastic syndromes were identified in association with thymoma in IUSCC patients. The most prevalent was Myasthenia Gravis (110 patients), followed by Hypothyroidism (21 patients, 5 confirmed as Hashimoto’s thyroiditis), Good Syndrome (19 patients), and Pure Red Cell Aplasia (15 patients). Significant findings included: 36.4% of patients with paraneoplastic comorbidity had >1 paraneoplastic syndrome, 51.8% presented with Stage IV disease, and 40.4% had WHO Type B2 tumor pathology, with Type B3 being second most common (25%). No significant demographic associations were identified. 10-year survival of TET patients with paraneoplastic syndromes was not significantly different from those without (p= 0.721). These results indicate potential associations between thymoma staging and grading and development of paraneoplastic disease. Further analysis with a larger data set is warranted. Serum and blood test analysis may also elucidate reasons behind the development of paraneoplastic disease in thymoma patients. 
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