Objective To review the presentation, diagnosis, staging and treatment of thymoma. Data sources Data were obtained from papers on thymoma published in English within the last 30 years. No formal systematic review was ...Objective To review the presentation, diagnosis, staging and treatment of thymoma. Data sources Data were obtained from papers on thymoma published in English within the last 30 years. No formal systematic review was conducted, but an effort was made to be comprehensive. Study selection Studies were selected if they contained data relevant to the topic addressed in the particular section. In particular, standards adopted by the International Thymic Malignancies Interest Group through a formal process of achieving worldwide consensus are featured. Because of the limited length of this article, we have frequently referenced recent reviews that contain a comprehensive amalgamation of literature rather than the actual source papers. Results Thymomas are rare malignant tumors. They account for about half （47%） of anterior mediastinal tumors. About one third of these are associated with myasthenia gravis. Computed tomography with intravenous contrast is the standard diagnostic modality. Thymomas appear as round or oval masses in early stages but irregular shapes with calcifications occurring in later stages. They can invade surrounding structures including mediastinal fat, pleura, major blood vessels and nerves. Fine needle aspiration, core needle biopsy or open biopsy is used to obtain tissue diagnosis. Masaoka-Koga classification is currently used to stage thymomas. All thymomas should be considered for resection due to their malignant potential. A complete resection is a major prognostic factor and every effort should be made to achieve this even if this means resection and reconstruction of a major thoracic structure. Median sternotomy is the standard approach for thymoma resection. A number of minimally invasive techniques are used in selective centers. While stage I and II tumors undergo primary surgery, preoperative chemotherapy appears to increase the chances of complete resection for stage III and IVa tumors. Postoperative radiation could be considered for patients with residual disease. Excellent 5 and 10展开更多
Background Thymectomy is considered the most effective treatment in patients with myasthenia gravis. This study aimed to explore the predictors of postoperative myasthenic crisis in patients with myasthenia gravis aft...Background Thymectomy is considered the most effective treatment in patients with myasthenia gravis. This study aimed to explore the predictors of postoperative myasthenic crisis in patients with myasthenia gravis after thymectomy.Methods Clinical records of 243 patients with myasthenia gravis who underwent thymectomy were reviewed retrospectively. The following factors were analyzed in relation to the occurrence of myasthenic crisis after thymectomy：gender, age, duration of symptoms, Osserman stage, history of myasthenic crisis, concomitant diseases, preoperative pyridostigmine dose, preoperative steroid therapy, operation approach, operation time, presence of thymoma, major postoperative complications.Results Forty-four patients experienced postoperative myasthenic crisis during the first month after thymectomy.Univariate analysis revealed that Osserman stage （RR=0.0976, P=0.000）, history of myasthenic crisis （RR=0.2309,P=0.012）, preoperative pyridostigmine dose （RR=0.4349, P=0.016）, thymoma （RR=0.0606, P=0.000）, and major postoperative complications （RR=0.1094, P=0.000） were significantly related to postoperative myasthenic crisis.Multivariate Logistic regression analysis showed that Osserman stage （Ⅱb＋Ⅲ＋Ⅳ） （RR=0.0953, P=0.000）, thymoma （RR=0.0294, P=0.000）, and major postoperative complications （RR=0.0424, P=0.000） independently predict postoperative myasthenic crisis.Conclusion Osserman stage （Ⅱb＋Ⅲb＋Ⅳ）, thymoma and major postoperative complications are independent predictors of postoperative myasthenic crisis in patients with myasthenia gravis who underwent thymectomy.展开更多
背景与目的胸腺瘤常伴发重症肌无力(myasthenia gravis,MG),但是这些患者行胸腺切除的预后与MG的关系尚不明确。本研究旨在探讨影响胸腺瘤合并MG患者预后的因素。方法回顾性分析中国胸腺瘤协作组(Chinese Alliance for Research of Thym...背景与目的胸腺瘤常伴发重症肌无力(myasthenia gravis,MG),但是这些患者行胸腺切除的预后与MG的关系尚不明确。本研究旨在探讨影响胸腺瘤合并MG患者预后的因素。方法回顾性分析中国胸腺瘤协作组(Chinese Alliance for Research of Thymoma,ChART)数据库1992年-2012年875例随访20年资料完整的胸腺瘤病例,分析世界卫生组织(World Health Organization,WHO)组织学分型、Masaoka分期、术后辅助治疗与MG及预后的关系。结果胸腺瘤WHO组织学分型与MG有相关性,差异有统计学意义(χ~2=24.908,P<0.001)。MG发生率为22.7%,其中B2型(58/178,32.58%)>B3型(65/239,27.20%)>B1型(27/132,20.45%)>AB型(43/267,16.10%)>A型(6/59,10.17%),Masaoka分期与MG无相关性(χ~2=1.365,P=0.714)。生存分析表明WHO分型、Masaoka分期与预后有关(P<0.05),而是否合并MG(χ~2=0.113,P=0.736)、是否行胸腺扩大切除(χ~2=1.548,P=0.213)、术后辅助放疗(χ~2=0.380,P=0.538)与预后无相关,术后辅助化疗与差的预后相关(χ~2=14.417,P<0.001)。是否行胸腺扩大切除与MG的疗效有相关性(χ~2=24.695,P<0.001)。结论胸腺瘤患者是否合并MG和是否行胸腺扩大切除与预后无相关性,胸腺扩大切除可改善MG患者的疗效。展开更多
The thymus is a central lymphatic organ that is responsible for many immunological functions,including the production of mature,functional T cells and the induction of self-tolerance.Benign or malignant tumors may ori...The thymus is a central lymphatic organ that is responsible for many immunological functions,including the production of mature,functional T cells and the induction of self-tolerance.Benign or malignant tumors may originate from the thymus gland,with thymoma being the most common and accounting for 50% of anterior mediastinal tumors.Malignancies linked to thymoma include the loss of self-tolerance and the presence of autoimmunity.In this review,we compiled the current scientific evidence detailing the various interactions between thymoma and autoimmune diseases,including myasthenia gravis,systemic lupus erythematosus,inappropriate antidiuretic hormone secretion,pure red cell aplasia,pernicious anemia,pemphigus and autoimmune thyroid diseases.In recent years,several mechanisms have been proposed to explain these interactions.Most are based on the assumption that the‘sick’thymus,like the‘normal’thymus,can generate mature T cells;however,the T cells generated by the sick thymus are impaired and thus may exert cellular autoreactivity.Here,we present several theories that may shed light on the loss of self-tolerance associated with this epithelial tumor of the thymus.展开更多
Objective Diffuse panbronchiolitis, a distinct clinical entity of unknown etiology, has been reported originally and primarily in Japanese and rarely in non-Japanese populations. Macrolide therapy is effective for thi...Objective Diffuse panbronchiolitis, a distinct clinical entity of unknown etiology, has been reported originally and primarily in Japanese and rarely in non-Japanese populations. Macrolide therapy is effective for this once dismal disease. Diffuse panbronchiolitis complicated with thymoma is uncommon; only 2 cases have been reported to date. The aims of this study were to describe the clinical profiles, assess the response to macrolide therapy, and to discuss the possible pathogenesis of diffuse panbronchiolitis in this setting.Methods The clinical profiles, macrolide therapy response of diffuse panbronchiolitis complicated with encapsulated thymoma in 2 historically confirmed cases were described and discussed with the 2 cases reported in the literature: one complicated with encapsulated thymoma, another with invasive thymoma.Results Of the 2 cases, both had negative PPD skin testing and abnormal serum levels of various immunoglobulins, 1 had positive anti-nuclear antibody, but none had elevated cold hemagglutinin liters, and both had an excellent response to macrolide therapy. Of the 2 cases reported in the literature, both had negative PPD or tuberculin skin testing, 1 had severe hypogammaglobulinemia, 1 had elevated IgA, 1 had positive anti-DNA, 1 had elevated cold hemagglutinin titers, but both died of respiratory failure in spite of macrolide therapy in 1 case.Conclusions Prognosis for diffuse panbronchiolitis complicated with thymoma may depend on the nature of the thymoma and on the disease course. Macrolide therapy is also effective if administered early in the disease course and if the thymoma is cured. Immunological factors may play an important role in the pathogenesis of diffuse panbronchiolitis in this setting.展开更多
文摘Objective To review the presentation, diagnosis, staging and treatment of thymoma. Data sources Data were obtained from papers on thymoma published in English within the last 30 years. No formal systematic review was conducted, but an effort was made to be comprehensive. Study selection Studies were selected if they contained data relevant to the topic addressed in the particular section. In particular, standards adopted by the International Thymic Malignancies Interest Group through a formal process of achieving worldwide consensus are featured. Because of the limited length of this article, we have frequently referenced recent reviews that contain a comprehensive amalgamation of literature rather than the actual source papers. Results Thymomas are rare malignant tumors. They account for about half （47%） of anterior mediastinal tumors. About one third of these are associated with myasthenia gravis. Computed tomography with intravenous contrast is the standard diagnostic modality. Thymomas appear as round or oval masses in early stages but irregular shapes with calcifications occurring in later stages. They can invade surrounding structures including mediastinal fat, pleura, major blood vessels and nerves. Fine needle aspiration, core needle biopsy or open biopsy is used to obtain tissue diagnosis. Masaoka-Koga classification is currently used to stage thymomas. All thymomas should be considered for resection due to their malignant potential. A complete resection is a major prognostic factor and every effort should be made to achieve this even if this means resection and reconstruction of a major thoracic structure. Median sternotomy is the standard approach for thymoma resection. A number of minimally invasive techniques are used in selective centers. While stage I and II tumors undergo primary surgery, preoperative chemotherapy appears to increase the chances of complete resection for stage III and IVa tumors. Postoperative radiation could be considered for patients with residual disease. Excellent 5 and 10
文摘Background Thymectomy is considered the most effective treatment in patients with myasthenia gravis. This study aimed to explore the predictors of postoperative myasthenic crisis in patients with myasthenia gravis after thymectomy.Methods Clinical records of 243 patients with myasthenia gravis who underwent thymectomy were reviewed retrospectively. The following factors were analyzed in relation to the occurrence of myasthenic crisis after thymectomy：gender, age, duration of symptoms, Osserman stage, history of myasthenic crisis, concomitant diseases, preoperative pyridostigmine dose, preoperative steroid therapy, operation approach, operation time, presence of thymoma, major postoperative complications.Results Forty-four patients experienced postoperative myasthenic crisis during the first month after thymectomy.Univariate analysis revealed that Osserman stage （RR=0.0976, P=0.000）, history of myasthenic crisis （RR=0.2309,P=0.012）, preoperative pyridostigmine dose （RR=0.4349, P=0.016）, thymoma （RR=0.0606, P=0.000）, and major postoperative complications （RR=0.1094, P=0.000） were significantly related to postoperative myasthenic crisis.Multivariate Logistic regression analysis showed that Osserman stage （Ⅱb＋Ⅲ＋Ⅳ） （RR=0.0953, P=0.000）, thymoma （RR=0.0294, P=0.000）, and major postoperative complications （RR=0.0424, P=0.000） independently predict postoperative myasthenic crisis.Conclusion Osserman stage （Ⅱb＋Ⅲb＋Ⅳ）, thymoma and major postoperative complications are independent predictors of postoperative myasthenic crisis in patients with myasthenia gravis who underwent thymectomy.
文摘背景与目的胸腺瘤常伴发重症肌无力(myasthenia gravis,MG),但是这些患者行胸腺切除的预后与MG的关系尚不明确。本研究旨在探讨影响胸腺瘤合并MG患者预后的因素。方法回顾性分析中国胸腺瘤协作组(Chinese Alliance for Research of Thymoma,ChART)数据库1992年-2012年875例随访20年资料完整的胸腺瘤病例,分析世界卫生组织(World Health Organization,WHO)组织学分型、Masaoka分期、术后辅助治疗与MG及预后的关系。结果胸腺瘤WHO组织学分型与MG有相关性,差异有统计学意义(χ~2=24.908,P<0.001)。MG发生率为22.7%,其中B2型(58/178,32.58%)>B3型(65/239,27.20%)>B1型(27/132,20.45%)>AB型(43/267,16.10%)>A型(6/59,10.17%),Masaoka分期与MG无相关性(χ~2=1.365,P=0.714)。生存分析表明WHO分型、Masaoka分期与预后有关(P<0.05),而是否合并MG(χ~2=0.113,P=0.736)、是否行胸腺扩大切除(χ~2=1.548,P=0.213)、术后辅助放疗(χ~2=0.380,P=0.538)与预后无相关,术后辅助化疗与差的预后相关(χ~2=14.417,P<0.001)。是否行胸腺扩大切除与MG的疗效有相关性(χ~2=24.695,P<0.001)。结论胸腺瘤患者是否合并MG和是否行胸腺扩大切除与预后无相关性,胸腺扩大切除可改善MG患者的疗效。
文摘The thymus is a central lymphatic organ that is responsible for many immunological functions,including the production of mature,functional T cells and the induction of self-tolerance.Benign or malignant tumors may originate from the thymus gland,with thymoma being the most common and accounting for 50% of anterior mediastinal tumors.Malignancies linked to thymoma include the loss of self-tolerance and the presence of autoimmunity.In this review,we compiled the current scientific evidence detailing the various interactions between thymoma and autoimmune diseases,including myasthenia gravis,systemic lupus erythematosus,inappropriate antidiuretic hormone secretion,pure red cell aplasia,pernicious anemia,pemphigus and autoimmune thyroid diseases.In recent years,several mechanisms have been proposed to explain these interactions.Most are based on the assumption that the‘sick’thymus,like the‘normal’thymus,can generate mature T cells;however,the T cells generated by the sick thymus are impaired and thus may exert cellular autoreactivity.Here,we present several theories that may shed light on the loss of self-tolerance associated with this epithelial tumor of the thymus.
文摘Objective Diffuse panbronchiolitis, a distinct clinical entity of unknown etiology, has been reported originally and primarily in Japanese and rarely in non-Japanese populations. Macrolide therapy is effective for this once dismal disease. Diffuse panbronchiolitis complicated with thymoma is uncommon; only 2 cases have been reported to date. The aims of this study were to describe the clinical profiles, assess the response to macrolide therapy, and to discuss the possible pathogenesis of diffuse panbronchiolitis in this setting.Methods The clinical profiles, macrolide therapy response of diffuse panbronchiolitis complicated with encapsulated thymoma in 2 historically confirmed cases were described and discussed with the 2 cases reported in the literature: one complicated with encapsulated thymoma, another with invasive thymoma.Results Of the 2 cases, both had negative PPD skin testing and abnormal serum levels of various immunoglobulins, 1 had positive anti-nuclear antibody, but none had elevated cold hemagglutinin liters, and both had an excellent response to macrolide therapy. Of the 2 cases reported in the literature, both had negative PPD or tuberculin skin testing, 1 had severe hypogammaglobulinemia, 1 had elevated IgA, 1 had positive anti-DNA, 1 had elevated cold hemagglutinin titers, but both died of respiratory failure in spite of macrolide therapy in 1 case.Conclusions Prognosis for diffuse panbronchiolitis complicated with thymoma may depend on the nature of the thymoma and on the disease course. Macrolide therapy is also effective if administered early in the disease course and if the thymoma is cured. Immunological factors may play an important role in the pathogenesis of diffuse panbronchiolitis in this setting.
