Thymic epithelial tumors represent the most common neoplasms of the anterior mediastinum, while atypical type A thymoma is a rare subtype of thymoma. On the morphological basis of type A thymoma, this tumor exhibits some atypical histological features, such as abundant cells, increased mitotic counts, tumor necrosis, and increased Ki67 index. At present, the clinical and pathological data of this tumor is still available. Since it was formally named, 16 cases have been reported around the world. In order to improve the understanding of the disease, this article reviews the related literature and tries to elaborate the atypical type A thymoma from the aspects of pathological features, clinical manifestations, epidemiology and differential diagnosis.
Objective To compare the differences in clinicopathological features, molecular phenotypes, and prognosis between atypical type A thymoma (AAT) and classic type A thymoma (TAT), and to clarify the aggressive nature of AAT. Methods The data of AAT patients (AAT group) and classic TAT patients (TAT group) who underwent surgical resection for thymoma at West China Hospital of Sichuan University between January 2016 and November 2024 were retrospectively collected. Comparisons on the clinical data, histopathology, immunohistochemistry (CD20, Ki-67), GTF2I mutation status, and survival outcomes were performed between the two groups. Results A total of 53 patients were enrolled, including 22 in the AAT group and 31 in the TAT group. There was no significant difference in age, sex, or initial presenting symptoms between the two groups (P>0.05). Compared with the TAT group, the AAT group had larger tumors [(5.6±2.7) vs. (4.1±2.0) cm, P=0.043], a lower proportion of Masaoka stage Ⅰ (31.6% vs. 61.3%, P=0.041), and worse survival outcomes [progression-free survival: hazard ratio (HR)=2.87, 95% confidence interval (CI) (1.42, 5.81), P=0.004; overall survival: HR=1.96, 95%CI (1.02, 3.78), P=0.013]. Pathologically, the AAT group showed more mitotic figures (mean 6/2 mm2), and tumor necrosis was observed in 45.5% of cases. There was no statistically significant difference in the CD20 expression rate (20.0% vs. 41.9%), Ki-67 index [(11.0±6.0)% vs. (8.0±6.9)%], or GTF2I mutation rate (86.7% vs. 92.3%) between the two groups (P>0.05). Conclusions AAT is a subtype of TAT with distinct aggressive pathological features, including higher mitotic activity, a tendency for necrosis, and a greater propensity for recurrence and metastasis. Pathological diagnosis should integrate morphology and molecular testing to guide more aggressive treatment and follow-up strategies.
摘要:目的:研究胸腺瘤与前纵隔(血管前间隙)淋巴瘤的MSCT表现,提高对二者的诊断与鉴别诊断能力。方法:回顾性分析经手术病理证实的30例胸腺瘤与18例血管前间隙淋巴瘤MSCT表现,着重观察肿瘤的密度、形态及其与周围结构的关系。结果:30例胸腺瘤中,24例良性胸腺瘤与邻近大血管分界清晰,肿块表现 “D”字或反“D”字状,平扫CT值16~59 Hu,增强CT值20~110 Hu;6例侵袭性胸腺瘤边界不清,呈分叶状、不规则形,密度不均,平扫CT值23~42 Hu,增强CT值23~60 Hu。18例淋巴瘤中,单发于前上纵隔者6例,其余12例呈多结节、肿块状,侵入血管间隙生长,致大血管受压,增强扫描呈轻度强化,常伴有其它部位淋巴结增大。结论:MSCT能清晰显示胸腺瘤与前纵隔淋巴瘤的影像学表现特征,并能有效提高对二者的鉴别诊断。Abstract: Objective: To diagnosis and differentiate thymoma and malignant lymphoma in the anterior mediastinum on the basis of multislice CT (MSCT) imaging features. Methods:We retrospectively reviewed 30 cases with thymoma and 18 cases with malignant lymphoma proven by surgery and pathology.More attention was put on the density, morphology and relation with the surrounding structures of the tumors. Results: The CT manifestations of 30 cases of thymoma were shown as: For 24 cases of benign thymoma, the boundaries were clear, the shapes were “D” signs or contra“D” signs, CT attenuation value were 1659Hu and 20110Hu on unenhanced and contrastenhanced scanning. For 6 cases of malignant thymoma, the boundaries were unclear, the shapes were lobulated or irregular, the density was heterogeneous, CT attenuation value were 2342Hu and 2360Hu on unenhanced and contrastenhanced scanning. For 18 cases of malignant lymphoma, 6 cases were located at anterior mediastinum, 12 cases were nodes or multiple mass, enveloped the neighboring vessel structures, mildly enhanced on contrastenhanced scanning, and associated with enlargement of lymph nodes in other place. Conclusion: MSCT can display the imaging features of thymoma and anterior mediastinal lymphoma, and effectively differentiate thymoma and mediastinal lymphoma.
Objective To assess the long-term results and relevant influencing factors of extended thymectomy and medicine-alone treatment of non-thymomatous myasthenia gravis (MG) patients. Methods We retrospectively analyzed the clinical data of 174 patients with non-thymomatous MG diagnosed and treated in our department from December 2009 to April 2017, including 81 males and 93 females, aged 13-88 (47.1±17.8) years. According to the different treatment methods, the patients were divided into two groups: an operation group (91 patients receiving extended thymectomy) and a medicine-alone group (83 patients receiving medical therapy alone). The efficacy was evaluated according to the Myasthenia Gravis Foundation of America (MGFA). Survival curves of the patients were plotted using the Kaplan-Meier method to evaluate the remission rate and survival rate. Cox regression analysis was used to assess the influencing factors of the outcomes. Results The patients were followed up for 3 to 94 (39.1±26.9) months. As a result, 29 patients (31.9%) achieved complete remission in the surgery group and 13 patients (15.7%) were completely relieved in the medicine-alone group (P=0.014). Further analysis showed that treatment pattern (P=0.018) and MG type (P=0.021) were the main factors related to the efficacy. Conclusion For patients with non-thymomatous MG, extended thymectomy is superior to the medicine-alone in terms of complete remission rate and the postoperative immunosuppression ratio.
Anterior mediastinal inflammatory myofibroblastoma is a rare tumor with insidious onset and easy misdiagnosis. In this report, we presented a case of anterior mediastinal inflammatory myofibroblastoma with thymoma. The mediastinal tumor was found by physical examination, and the prognosis was good after surgical treatment. For this disease, operation is an effective method for definite diagnosis and treatment, and complete excision can achieve good outcomes.
ObjectiveTo investigate the safety and efficacy of totally no tube three-port thoracoscopic surgery (TNTT) for thymic tumor via lateral thoracic approach. MethodsThe clinical data of patients with thymoma admitted to the Department of Thoracic Surgery of the General Hospital of Northern Theater Command from November 2021 to May 2022 were retrospectively analyzed. The patients were divided into a TNTT group and a single utility port video-assisted thoracic surgery (SVATS) group according to different surgical methods. The clinical data were compared between the two groups. ResultsA total of 111 patients were collected. There were 44 patients in the TNTT group, including 20 males and 24 females, with an average age of 60.11±8.64 years, and 67 patients in the SVATS group, including 30 males and 37 females, with an average age of 62.40±7.92 years. There was no significant difference between the two groups in the baseline data (P>0.05). The postoperative hospital stay and intraoperative blood loss were shorter or less in the TNTT group (P<0.05), and the visual analogue scale score 48 hours after the operation was smaller in the SVATS group (P<0.05). ConclusionTNTT has a good surgical safety, and can shorten postoperative hospital stay, reduce intraoperative blood loss, and has significant advantages in enhanced recovery after surgery, but SVATS can reduce postoperative pain in patients.
Objective To compare the clinical efficacy and safety of da Vinci robot with totally no tube (TNT) versus subxiphoid video-assisted thymectomy surgery (SVATS) in the treatment of thymic tumors. Methods From 2019 to 2021, a retrospective analysis was conducted on patients with thymic tumor resection in the Department of Thoracic Surgery, General Hospital of Northern Theater Command. All patients underwent total thymectomy and mediastinal fat removal, and they were divided into a TNT group and a SVATS group according to the operation method. The intraoperative blood loss, conversion rate, postoperative visual analogue score (VAS), postoperative hospital stay time and postoperative complications were compared between the two groups. Results We finally included 435 patiets. There were 168 patients with 83 males and 85 females at an average age of 61.920±9.210 years in the TNT group and 267 patients with 147 males and 120 females at an average age of 61.460±8.119 years in the SVATS group. There was no death or postoperative myasthenic crisis in both groups. There was no statistical difference in postoperative hospital stay (1.540±0.500 d vs. 3.400±0.561 d, P=0.000), intraoperative blood loss (13.450±5.498 mL vs. 108.610±54.462 mL, P=0.000), postoperative 24 h VAS score (4.960±1.757 points vs. 3.600±1.708 points, P=0.000), or postoperative complication rate (3.0% vs. 11.6%, P=0.001).Conclusion TNT is a more efficient, safe, and effective surgical approach for treating thymic tumors, which can shorten hospital stay time and reduce postoperative complications. However, SVATS can minimize postoperative pain.
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.
[Abstract]This study reports a case of an 83-year-old female patient with systemic severe myasthenia gravis (American Myasthenia Foundation class Ⅳb) and multiple comorbidities who achieved minimal clinical status through preoperative alemtuzumab treatment (10 mg/kg, once per week for 4 consecutive weeks). After undergoing robotic thymectomy, the patient remained clinically stable in the early postoperative period until the 5th day after surgery when she developed dysphagia. Despite intravenous immunoglobulin and other therapeutic interventions, the patient’s myasthenic symptoms continued to worsen, coughing was impaired, respiratory insufficiency intensified, non-invasive ventilation support was required, and pulmonary infection occurred. Clinical symptoms further deteriorated, with diarrhea, urinary tract infection, and progressive respiratory tract infection. She was subsequently transferred to the intensive care unit for invasive mechanical ventilation and therapeutic plasmapheresis. These interventions failed to halt the disease progression, which eventually led to multiple organ dysfunction syndrome, and the patient died. Notably, among the other 12 patients receiving alemtuzumab treatment during the same period, none experienced such severe complications. This case is exceptional, and a direct causal relationship between alemtuzumab and mortality risk cannot be established at this time. Further research is needed to clarify perioperative immune management strategies.
Thymectomy is a major surgical procedure for patients with non-thymomatous myasthenia gravis，and can enhance their symptomatic remission rate and cure rate. There is still much controversy about appropriate surgical approach and extent of resection of thymectomy. The majority of thoracic surgeons believe that the completeness of thymectomy is closely associated with clinical symptom improvement，and perform complete resection of encapsulated thymus and surroun-ding fat tissues via mid-sternotomy. But minimally invasive thymectomies are often more acceptable by patients. On the contrary，in view of common existence of ectopic thymus tissue，some thoracic surgeons advocate a combination of cervical incision and sternotomy in order to further completely remove all thymus tissue.