Thymoma complicated with polymyositis and myasthenia gravis is a rare case, which can be clearly diagnosed and given symptomatic treatment according to its own diagnostic criteria, imaging and laboratory examinations. This paper reports the clinical data of a thymoma patient with polymyositis and myasthenia gravis admitted to the Seventh Affiliated Hospital of Sun Yat-Sen University, and discusses the possible pathogenesis and treatment methods.
Objective To collect evidence of treatment for the patients with ocular myasthenia gravis. Methods We searched The Cochrane Library (Issue 1, 2010), PubMed (1966 to April, 2010), CNKI (1979 to April, 2010) and VIP (1989 to April 2010) to identify systematic reviews, randomized controlled trials, controlled clinical trials and prospective avaicohort studies about efficacy and safety of treatment for ocular myasthenia gravis. Results We identified 2 guidelines, 5 systematic reviews and 4 observational studies on inhibitor of acetylcholinesterase, immunosuppressive agents, intravenous immunoglobulin, corticosteroids, and thymectomy for ocular myasthenia gravis. The first choice for ocular myasthenia gravis was inhibitor of acetylcholinesterase. When the symptom could not be remitted, the corticosteroids should be considered. Immunosuppressive agents might be added if the effect of corticosteroids was not good. Plasma exchange was not recommended. Thymectomy should be performed if the patients suffered from thymoma. Conclusion The best clinical evidence can be available by the evidence-based method.
Objective To evaluate the risk factors of the patients with myasthenia gravis (MG) after resection of thymoma. Methods We retrospectively analyzed the clinical data of 126 thymoma patients without preoperative MG who underwent a thymectomy in our hospital from June 2002 through May 2015. There were 51 males and 75 females at age of 51.71±14.06 years. The risk factors for MG after resection of thymoma were evaluated. Results MG occurred in nine patients after resection of thymoma (7.1%). Incomplete resection (P=0.024), A and AB type of WHO classification (P=0.048), concomitant autoimmune diseases (P=0.024), postoperative pulmonary infection (P=0.036) were the risk factors for the MG after resection of thymoma. Postoperative radiotherapy and chemotherapy (P=0.011) reduced the risk for the patients with incomplete resection or invasive thymoma. Conclusion Incomplete resection, A and AB type of WHO classification, concomitant autoimmune diseases, postoperative pulmonary infection are considered as the risk factors for MG after resection of thymoma, and postoperative radiotherapy and chemotherapy should be performed for the patients with incomplete resection or invasive thymoma.
ObjectiveTo investigate the long-term efficacy and the influencing factors of thymectomy for thymoma patients associated with myasthenia gravis. MethodsWe retrospectively analyzed the clinical and follow-up data of 126 thymoma patients associated with myasthenia gravis underwent extended thymectomy from June 2002 to December 2015 in our hospital. There were 26 males and 37 females at the mean age of 54.51±12.62 years. We built up survival analysis model to analyze the effect of those following factors on postoperative result:sex, the age when operated, the preoperative course of disease, the condition of associating with other diseases, history of critical illness, steroid administration time before operation, Osserman classification, Masaoka staging, WHO pathological type, surgical approach, tumor size and so on. Result The average follow-up time was 35(5-96) months. During follow-up period, 12 patients (19%) achieved complete remission, 39 patients (62%) achieved partial remission, 7 patients (11%) kept stable, 5 patients (8%) deteriorated and the total effective rate was 81%. The result of log-rank analysis showed that the preoperative course of disease (P=0.027), history of critical illness on myasthenia gravis (P=0.035) and Osserman classification (P=0.018) were related to incomplete remission, whlie the result of Cox regression analysis showed that the preoperative course of disease (P=0.001) and Osserman classification (P=0.012) were the independent risk factors for incomplete remission. ConclusionExtended thymectomy is an effective treatment for thymoma patients associated with myasthenia gravis, but the symptom of those patients whose preoperative course of disease are more than 12 months or Osserman classification is at ⅡB, Ⅲ and Ⅳ type of Osserman classification have poor effect after operation.
ObjectiveTo compare the perioperative outcomes of subxiphoid robot-assisted extended thymectomy (SRAET) and video-assisted thoracoscopic extended thymectomy (VATET) for myasthenia gravis complicated with thymoma.MethodsRetrospective analysis of 61 patients with myasthenia gravis combined with thymoma who were admitted to the Department of Thoracic Surgery, West China Hospital, Sichuan University from January 2017 to June 2019 was performed. All patients underwent extended thymectomy, and the patients were divided into a SRAET group and a VATET group. There were 26 patients in the SRAET group, including 11 males and 15 females, with an average age of 42.20±13.20 years. There were 35 patients in the VATET group, including 14 males and 21 females, with an average age of 45.00±13.00 years. The perioperative outcomes of the two groups including gender, age, operation time, intraoperative blood loss, conversion rate, postoperative drainage, tube removal time, drainage volume, visual analogue scale, hospital stay and postoperative complications were compared.ResultsThere was no conversion to thoracotomy, death or myasthenia crisis in both groups. The operation time (111.42±28.60 min vs. 103.71±26.20 min, P=0.845), intraoperative blood loss (32.31±23.84 mL vs. 63.57±132.22 mL, P=0.239), visual analogue scale at postoperative 24 h (2.46±0.76 vs. 2.40±0.74, P=0.751) and postoperative 48 h (2.12±0.77 vs. 2.26±0.56, P=0.407), complication rate (3.8% vs. 2.9%, P=0.675), drainage volume (206.85±130.09 mL vs. 276.86±173.46 mL, P=0.089) and hospital stay (5.81±2.52 d vs. 5.29±2.17 d, P=0.642) were not significantly different between the two groups. The visual analogue scale of the SRAET group at postoperative 72 h (1.12±0.65 vs. 1.86±0.91, P=0.001) was significantly lower than that of the VATET group.ConclusionSRAET is a safe and feasible method with less postoperative short-term pain, which is an alternative surgical treatment for myasthenia gravis complicated with thymoma.
Objective To compare the clinical efficacy of subxiphoid video-assisted thoracoscopic surgery (XVATS) and conventional intercostal VATS (CVATS) extended thymectomy for myasthenia gravis (MG). MethodsThe clinical data of MG patients who underwent extended thymectomy in the Department of Thoracic Surgery of Xuzhou Central Hospital from October 2016 to October 2021 and finished the follow-up were retrospectively reviewed. They were divided into an XVATS group and a CVATS group according to the procedure. The perioperative variables and clinical efficacy of the two groups were compared. ResultsA total of 84 patients were collected, including 43 males and 41 females, with a mean age of 52.3 years. There were 41 patients in the XVATS group and 43 patients in the CVATS group. There was no mortality, cardiopulmonary thrombosis, prolonged air leak, or mediastinal infection. Additionally, the CVATS group recorded 5 (11.6%) patients of conversion to open thoracotomy, 1 (2.3%) patient of postoperative MG crisis, 1 (2.3%) patient of bleeding in thorax, and 1 (2.3%) patient of chylothorax. The operation time (127.4±50.4 min vs. 122.9±38.6 min), intraoperative bleeding [46.9 (25.7, 79.2) mL vs. 45.7 (21.9, 92.1) mL], incidence of complications [0 vs. 7.0% (3/43)], chest tube duration (4.3±1.9 d vs. 4.8±2.8 d), follow-up time (19.1±8.5 months vs. 22.5±13.7 months), the proportion of residual mediastinal fat tissue [12.2% (5/41) vs. 4.7% (2/43)], and total MG remission rate [29.3% (12/41) vs. 51.2% (22/43)] were not statistically different between the two groups (P>0.05). However, the two groups showed significantly different incidence of conversion to open thoracotomy [0 vs. 11.6% (5/43), P=0.024], postoperative hospital stay time (8.2±3.3 d vs. 11.4±5.8 d, P=0.003) and total drainage volume [396.7 (173.8, 542.5) mL vs. 218.8 (102.1, 430.0) mL, P=0.038]. ConclusionXVATS extended thymectomy is technically safe and feasible; however, more evidence is warranted before the recommendation of this approach for the treatment of MG.
ObjectiveTo assess the correlation of WHO pathological classification and Masaoka stage of thymomas with its prognosis.MethodsA total of 468 patients with thymomas who received surgeries during 2009-2019 in Huashan Hospital, Fudan University, were collected. There were 234 males and 234 females with an average age of 21-83 (49.6±18.7) years. A total of 132 patients underwent video-assisted thoracic surgery (VATS) and 336 patients underwent thymectomy with median sternal incision. The follow-up time was 5.7±2.8 years. The clinical data of the patients were analyzed.ResultsThe amount of intraoperative bleeding was 178.3±133.5 mL in the median sternal incision group, and 164.8±184.1 mL in the VATS group (P=0.537). The operative time was 3.3±0.7 h in the median sternal incision group and 3.4±1.2 h in the VATS group (P=0.376). Postoperative active bleeding, phrenic nerve injury and chylothorax complications occurred in 8 patients, 9 patients and 1 patient in the VATS group, respectively, and 37 patients, 31 patients and 7 patients in the median sternal incision group, respectively. There was no statistical difference between the two groups (P=0.102, 0.402, 0.320). The 5-year cumulative progression free survival (PFS) rates of patients with WHO type A, AB, B1, B2, B3 and C thymomas were 100.0%, 100.0%, 95.7%, 81.4%, 67.5% and 50.0%, respectively (P<0.001). The 5-year PFS rates of patients with Masaoka stageⅠ-Ⅳ thymomas were 96.1%, 89.2%, 68.6% and 19.3%, respectively (P<0.001). The 5-year PFS rate was 87.3% in patients with myasthenia gravis (MG) and 78.2% in patients without MG (P<0.001). The 5-year PFS rates of patients with different surgeries were 82.4% and 83.8%, respectively (P=0.904). ConclusionWHO pathological classification and Masaoka stage have significant clinical prognosis suggestive effect. Thymoma patients combined with MG have better prognosis, which suggests early diagnosis and treatment of thymoma are important.
Thymectomy is the main treatment for thymoma and other thymic diseases. But the incidence of non-therapeutic thymectomy is high due to the difficulty in the differential diagnosis of anterior mediastinum lesions. Formerly, it was believed that the thymus gradually degraded and lost function with aging, and the preservation of the thymus was not valued. Recent studies have found that the removal of the thymus at all ages has adverse effects on overall health and leads to a significant increase in the risk of autoimmune diseases, malignancy, and all-cause mortality. Therefore, unnecessary thymectomy should be avoided. This article reviews the influence of thymectomy, including the changes of immunological indexes and clinical prognosis, and further discusses the current situation and avoidance methods of non-therapeutic thymectomy.
ObjectiveTo investigate the expression of α7 nicotinic acetylcholine receptor (α7 nAChR) in thymocytes of patients with myasthenia gravis (MG) and its effect on cytokine secretion and T cell proliferation. MethodsPatients with MG who underwent expanded thoracoscopic thymectomy in the Comprehensive Diagnosis and Treatment Center of the Henan Provincial People’s Hospital from June 2021 to June 2022 were selected and allocated to a MG group. Patients who underwent partial thymectomy to expose the surgical field during the cardiac disease surgery from June 2021 to September 2022 in the Department of Adult Cardiac Surgery of Fuwai Huazhong Cardiovascular Hospital were selected as the control group. Thymic single cell suspensions were prepared from MG and control groups, and the expression of α7 nAChR in thymocytes of the two groups was detected by real-time polymerase chain reaction and Western blotting. Then CD3/CD28 monoclonal antibody coupled with magnetic beads was used to induce T cell activation, and the levels of cytokines interferon-gamma (IFN-γ), tumor necrosis factor-α (TNF-α), interleukin-4 (IL-4), IL-6, IL-10, IL-17, and IL-21 in thymocytes of the two groups were detected by enzyme-linked immunosorbent assay (ELISA). The activated T cells of the MG group were divided into a blank control group, an α7 nAChR antagonist group, and an α7 nAChR agonist group according to different treatment methods. After 72 hours of culture, IFN-γ, TNF-α, IL-4, IL-6, IL-10, IL-17, and IL-21 expression levels in the culture supernatant were measured by ELISA. Afterwards, CD4-PE and CD8-APC antibodies were added, and the proliferation of T cell subsets was detected by flow cytometry. ResultsA total of 10 MG patients were collected, including 3 males and 7 females with an average age of 19.25±6.28 years; and 15 control patients were collected, including 6 males and 9 females with an average age of 26.18±6.77 years. Compared with the control group, the mRNA and protein levels of α7 nAChR in the thymocytes of MG group were decreased, and the expression levels of IFN-γ, TNF-α, IL-4, IL-6 and IL-21 in the supernatant were increased (P<0.05), but there was no statistical difference in the expression of IL-10 and IL-17 (P>0.05). The cell-culture experiment showed that compared with the blank control group, the levels of IFN-γ, TNF-α, IL-6 and IL-21 secreted by T cells in the α7 nAChR antagonist group were increased (P<0.05), while they were decreased in the α7 nAChR agonist group (P<0.05). There was no statistical difference in the secretion levels of IL-4, IL-10 or IL-17 among the three groups (P>0.05). CD4+ T and CD8+ T cells in the α7 nAChR agonist group were significantly less than those in the blank control group and α7 nAChR antagonist group (P<0.001), while they were significantly more in the α7 nAChR antagonist group than those in the blank control group (P<0.001).ConclusionThe expression of α7 nAChR in thymocytes of MG patients is decreased, and α7 nAChR may be involved in the inflammatory response in thymocytes and thus in thymic function.
Objective To compare the different surgical treatment methods of thymoma combined with myasthenia gravis (MG), and to discuss the clinical effectiveness of thoracoscopic combined mediastinoscopic extended thymectomy. Methods We retrospectively analyzed the clinical data of 58 patients of thymoma combined with myasthenia gravis in Northern Jiangsu People's Hospital between 2011 and 2016 year. According to the operation method, the patients were divided into three groups including a group A for thoracoscopic thymectomy (n=32), a group B for thoracoscopic combined mediastinoscopic thymectomy (n=15), and a group C for transsternal thymectomy (n=11). The clinical effects were observed and compared. Results In the group A and the group B, the bleeding volume, postoperative hospital stay and other complications were significantly lower than those in the group C with statistical differences (P<0.05). The incidence of myasthenic crisis in the group B (6.7%) was less than that in the group C (36.4 %), but the difference was not statistically different (P=0.058). The operation time of the three groups was 122.0 ± 39.4 min, 130.3 ± 42.5 min, and 142.3 ± 40.8 min respectively with no statistical difference between the two groups (P>0.05). The rate of dissection grade in the group B (grade 1, 12 patients, 80%) was significantly greater than that in the group A (grade 1, 14 patients, 43.8%,P<0.05). The effective rate of the group A, the group B, the group C was 84.4%, 93.3% and 90.9%, respectively with no statistical difference between groups (P>0.05). Conclusion The thoracoscopy combined mediastinoscopic thymectomy not only has the advantages of less trauma, quicker recovery and fewer complications, but also can more thoroughly clean the thymus and adipose tissue, which can achieve the same therapeutic effect as the transsternal thymectomy.