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The aim of this study was to investigate the surgical and long-term neurological outcomes of patients with acetylcholine-receptor-antibody-associated myasthenia gravis (AChR-MG) who underwent robotic thymectomy (RATS). We retrospectively analyzed the clinical-pathological data of all patients with AChR-MG who underwent RATS using the DaVinci (R) Robotic System at the MUMC + between April 2004 and December 2018. Follow-up data were collected from 60 referring Dutch hospitals. In total, 230 myasthenic patients including 76 patients with a thymoma (33.0%) were enrolled in this study. Mean follow-up time, procedure time and hospitalization were, respectively 65.7 +/- 43.1 months, 111 +/- 52.5 min and 3.3 +/- 2.2 days. Thymomatous patients had significantly more frequently and more severe complications than nonthymomatous patients (18.4% vs. 3.9%, p < 0.001). Follow up data was available in 71.7% of the included patients. The Myasthenia Gravis Foundation of America postintervention score showed any kind of improvement of MG-symptoms after RATS in 82.4% of the patients. Complete stable remission (CSR) or pharmacological remission (PR) of MG was observed in 8.4% and 39.4% of the patients, respectively. Mean time till CSR/PR remission after thymectomy was 26.2 +/- 29.2 months. No statistical difference was found in remission or improvement in MGFA scale between thymomatous and nonthymomatous patients. RATS is safe and feasible in patients with MG. The majority of the patients (82.4%) improved after thymectomy. CSR and PR were observed in 8.4% and 39.4% of the patients, respectively, with a mean of 26.2 months after thymectomy. Thymomatous patients had more frequently and more severe complications compared to nonthymomatous patients.(c) 2023 The Author(s). Published by Elsevier B.V. This is an open access article under the CC BY license ( http://creativecommons.org/licenses/by/4.0/ )

BACKGROUND Thymectomy is required for the treatment of thymoma-associated myasthenia gravis (MG). However, MG may develop only after thymectomy, a condition known as post-thymectomy MG. This study aimed to investigate the risk factors for post-thymectomy MG in patients with thymoma. METHODS We retrospectively identified 235 patients with thymoma who underwent thymectomy at a single hospital from January 2008 to December 2017: 44 with preoperatively diagnosed MG were excluded, leaving 191 patients in the final analysis. Univariable survival analyses using Cox proportional hazards regression model and Kaplan-Meier estimate were conducted to identify risk factors for post-thymectomy MG. RESULTS Post-thymectomy MG developed in 4.2% (8/191) of the patients with thymoma between 18 days and 108 mo after surgery. Hazard ratios (HRs) of pre- and postoperative anti-acetylcholine receptor antibody (AChR-Ab) titers were 2.267 (P = .002) and 1.506 (P

The patient presented to our hospital with systemic eruptions and scalp hair loss. Six years ago, he underwent extended thymectomy for a diagnosis of thymoma complicated by myasthenia gravis. Three years post-surgery, recurrence manifested as left-sided pleural dissemination and liver metastasis.

Thymoma is a rare epithelial tumor of the thymus gland. Despite rarity, it is the most common tumor of the anterior mediastinum. Surgical resection in the form of extended thymectomy is the gold standard operation. Conventionally and even in the current era of significant advances in the minimally invasive surgery, open transsternal extended thymectomy is considered the gold standard, particularly for advanced-stage tumors. There is however significant evidence now available for the use of minimally invasive approaches for early-stage thymomas. This article aims to discuss the various minimally invasive approaches currently being employed for thymomas.

BACKGROUND: Approaches of thoracoscopic thymectomy for myasthenia gravis (MG) are debatable. The subxiphoid approach is widely utilized recent years for its better visualization of the anterior mediastinum. In the present study, we compared perioperative outcomes and mid-term effects of the extended thymectomy for MG between the subxiphoid approach and the routine right-thoracic approach. METHODS: One hundred and thirty-one MG patients treated with thoracoscopic extended thymectomy were analyzed. Among them, 68 patients were operated on via the subxiphoid approach and the other 63 via the conventional right-side unilateral approach. The patient outcomes were retrospectively reviewed and evaluated. Mid-term clinical outcome was assessed according to the classification system proposed by the Myasthenia Gravis Foundation of America (MGFA). Clinical efficacy and variables influencing outcome were evaluated by the Kaplan-Meier method and Cox proportional hazards regression analysis. RESULTS: Compared with the right thoracic approach, the duration of the procedure via the subxiphoid approach was significantly shorter (P=0.035), the rates of total thymectomy were higher (P=0.028), and the pain scores on postoperative days 1, 3, and 7 were significantly lower (P

Anaemia characterised by near absence of red cell precursors in the bone marrow is referred to as “pure red cell aplasia (PRCA).” It has an unusual and intriguing association with thymoma and auto-immunity. Here, we report such a case which was successfully treated with extended thymectomy by minimally invasive approach. A 68-year-old anaemic lady presented to our institute with weakness and exertional dyspnoea for almost a year. She was transfused with blood for the same periodically but remained transfusion dependent. Apart from pallor, her systemic examination was unremarkable. Peripheral smear showed reduced haemoglobin (Hb) mass and reduced reticulocytes. Bone marrow biopsy implied PRCA. High-resolution computed tomography (HRCT) of thorax revealed a well-defined soft tissue lesion in the anterior mediastinum suggesting thymoma. Biopsy showed lymphocyte-rich type thymoma. Thymus along with fibrofatty tissue was resected completely using video-assisted thoracoscopic surgery (VATS) approach. Histopathological examination of the resected specimen revealed World Health Organisation (WHO) type B1—thymoma. Immediate post-operative period was uneventful. However, a fall in Hb which required blood transfusions necessitated initiation of immunosuppression with corticosteroids. Patient was given 6 weeks of adjuvant immunosuppression with corticosteroids. After 8 months post-thymectomy, she was leading a symptom-free life and no longer in need for blood transfusions. VATS thymectomy is a safe and feasible approach for the treatment of thymoma-induced PRCA with added advantage of minimal invasive approach. Long-term complete remission is possible with adjuvant immunosuppression.

We herein report a 63-year-old rippling muscle disease (RMD) patient who presented with painless stiffness, muscle hypertrophy and muscle contractions elicited by mechanical stimulation. He also showed irregular toe jerks and a slightly elevated level of anti-acetylcholine receptor antibody (AChR-Ab). Since he had a mediastinal mass mimicking thymoma, which was later revealed to be a bronchial cyst, he underwent extended thymectomy. The irregular toe jerks disappeared within a week after the operation. The other muscle symptoms completely remitted 27 months after the onset. This is the first report of a sporadic case of RMD with irregular toe jerks that resolved after extended thymectomy.

Background The length of time for clinical improvement of patients with thymomatous myasthenia gravis (MG) after extended thymectomy is not clear. The purpose of this study was to determine the length of time after thymectomy in patients with thymomatous MG to achieve a 3-point reduction of Quantitative Myasthenia Gravis Score (QMGS), and identify variables associated with a failure to achieve the reduction. Methods The records of patients with thymomatous MG who underwent extended thymectomy from January 2005 to December 2018 were retrospectively reviewed. The primary end point was a reduction of 3 points of QMGs and the secondary end point was another reduction of 3 points of QMGs. Results A total of 481 patients were included in the analysis, the mean age of the patients was 41.63 ± 8.55 years, and approximately 60% were male. The median time to achieve a 3 point decrease in QMGS was 6 months, and the median time to achieve another 3 point decrease was 30 months. Multivariable analysis indicated that age ≥ 42 years and Masaoka-Koga stage > I were associated with a lower probability of achieving a 3 point decrease in QMGS (HR = 0.55 and 0.65, respectively). Likewise, multivariable analysis indicated that age ≥ 42 years and Masaoka-Koga stage > I were associated with a lower probability of achieving a second 3 point decrease in QMGS (HR = 0.53 and 0.53, respectively). Conclusions In patients with thymomatous MG who receive thymectomy, age ≥ 42 years and Masaoka-Koga stage > I are associated with a worse prognosis and failure to achieve a 3 point decrease in QMGS.

A 72‐year‐old man was diagnosed as having myasthenia gravis (MG). He underwent computed tomography which revealed an anterior mediastinal tumour. Laboratory examination revealed elevated levels (106.3 U/ml) of carbohydrate antigen (CA) 19‐9 in serum. However, no malignant disease was detected on fluorodeoxyglucose‐positron emission tomography. A diagnosis of thymoma associated with MG was considered and an extended thymectomy was performed. Histopathologically, thymoma was categorized as stage I based on the Masaoka classification, and as type AB according to the World Health Organization classification. Immunohistochemistry was positive for CA 19‐9. The serum levels returned to the normal range post‐operatively (16.7 U/ml). Herein, we report an extremely rare case of thymoma with raised levels of CA 19‐9., A 72‐year‐old man was diagnosed with thymoma and elevated serum carbohydrate antigen (CA) 19‐9. We report a case of serum CA 19‐9‐producing thymoma that was confirmed histologically.

Introduction Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder. Paraneoplastic SPS associated with malignant tumors such as thymoma occurs in approximately 5% of all SPS cases. We present a rare case of thymoma accompanied by SPS successfully treated using surgery. Presentation of case A 26-year-old woman presented with lower limbs convulsions and gait disturbance and complained of leg pain. Cerebrospinal fluid and blood test results showed a high level of anti-glutamic acid decarboxylase (GAD) antibodies. Computed tomography showed anterior mediastinal tumor suggestive of a thymoma. She underwent extended thymectomy, and her symptoms gradually improved after surgery. No evidence of recurrent thymoma and SPS has been observed over 44 months. Conclusion Surgical treatment would be effective for patients with SPS and thymoma.

This report was about a 60‐year‐old asymptomatic female patient who presented to our clinic with an anterior mediastinal mass found on routine chest computed tomography (CT). Chest CT revealed an irregular poorly enhanced anterior mediastinal mass which showed signs of infiltration to adjacent structures with sparse calcifications. The preliminary diagnosis was thymic carcinoma. The patient underwent extended thymectomy via median sternotomy and complete excision of the tumor. A small draining vein to the left brachiocephalic vein and phleboliths were identified in the tumor. A definitive diagnosis was made of mediastinal venolymphatic malformation (VLM). The patient had an uneventful clinical course and was discharged without further complication. This report highlights that it is possible to misdiagnose mediastinal VLM as thymic carcinoma and could serve as a useful reminder to physicians in the future.

La Timectomía es parte del tratamiento de la Miastenia Gravis debido a que puede producir remisiones de la sintomatología. Los parámetros que determinan el éxito de la cirugía son únicamente cuantitativos: cantidad de medicación o presencia/ausencia de síntomas, sin tomar en consideración los cambios del campo psicosocial. Este estudio, de tipo cualitativo retrospectivo, determina los cambios psicosociales en los pacientes que han sido tratados con Timectomía por Miastenia Gravis no Timomatosa. Variables como sexo, edad, medicación, grupo de Osserman pre y post quirúrgico, escala de Oosterhuis, escala de Millichap y escala de la Fundación Americana de MG, fueron recolectadas de las fichas médicas de los pacientes entre 2003 al 2013. Diecisiete personas cumplieron los criterios de inclusión: Ser mayores de 18 años con diagnóstico de Miastenia Gravis no Timomatosa a los que se les realizó timectomía extendida. Estas personas fueron encuestadas telefónicamente mediante el cuestionario SF-36. Los datos recopilados se analizaron con SPSS 22.0. La calidad de vida, antes y después de la cirugía, fue comparada mediante la prueba no paramétrica de Wilcoxon. El resultado quirúrgico de los pacientes fue: 52.9% tuvo una mejoría clínica significativa de acuerdo a la escala Oosterhuis (remisión total = 5, síntomas leves = 3, discapacidad leve = 1), de estos, el 17.6% presentó una remisión completa estable, sin medicación, y el 35.2% presentó remisión farmacológica con dosis bajas. Los resultados del cuestionario SF-36 indican que los pacientes tratados mejoraron su nivel medio de vida (p

Background: Myastenia gravis (MG) is a neuromuscular junction disorder that causes significant disability in the patient. Plasmaparesis and thymectomy are some therapeutic modalities in MG patients. Patients with MG of 10-15% had thymoma and 60% occurred thymic hyperplasia. Thymectomy may improve MG outcomes in 54-94% of patients (thymoma cases) or 21-42% of patients (cases of thymic hyperplasia). Plasmaparesis perioperatif still controversial. Case report: The 60-year-old man comes with a complaint both eyelids often close and both hands and feet experience fluctuating weakness, improved at rest. Patients with a history of DM and pulmonary TB. On the physical examination obtained eyelid twitch response and improved ptosis on ice test. Laboratory shows hyperglycemia and thrombocytopenia. Electrophysiological examination supports neuromuscular junction lesions. In thoracic and thoracic rectal images the mediastinal mass was obtained. Patients were diagnosed with myastenia gravis with suspected thyroid mediastinal mass. Treatment given mestinon 60 mg every 8 hours peroral, thymectomy plan, platelet transfusion, and plasmaparesis. Obtained a tumor with a large 10x8x7 cm with adhesions around the organ. Microscopic features support a thymoma A. Patients receive perioperative plasmaparesis 1 series (5x administration) divided, 2 preoperative and 3 postoperative times. The patient used mechanical ventilation for less than 24 hours and no residual symptoms. Conclusion: Myastenia gravis grade IIa (Osserman) with thymoma A performed extended thymectomy. Plasmaparesis given pre and post surgery showed clinical improvement and duration of short ventilator use. Good prognosis associated with mild MG degree, no myastenia crisis. Keywords: myastenia gravis, thymectomy, plasmaparesis

Compared to the intercostal approach, subxiphoid uniportal video-assisted thoracoscopic surgery (VATS) is considered to be less invasive as it may cause minimal postoperative pain. Besides, it provides an excellent view of the bilateral pleural cavities. In this paper, we describe our technique and overview a 2-year experience results in this approach for the surgical treatment of anterior mediastinal and pulmonary lesions. In a retrospective study of data collected prospectively between October 2014 and December 2016, 38 patients underwent surgery for an anterior mediastinal tumor or myasthenia gravis at our institution. Intraoperative factors like duration of operation and amount of blood loss were analyzed as well as postoperative ones, including duration of chest drains, amount of postoperative fluid drainage, and length of hospital stay. The median age of patients was 59 years (36-80 years) with 19 females (50%). Overall, 28 patients (74%) underwent extended thymectomy. Seven patients (19%) experienced a combined lung resection and extended thymectomy, 3 (7%) a resection of pericardial (2) or bronchogenic (1) cysts. The median diameter of the lesions was 2.93 cm (1.2-7.7 cm). Postoperatively, 30-day mortality was 0%. Subxiphoid uniportal VATS is a convenient approach for minimally invasive mediastinal surgery. The excellent exposure of the anterior mediastinum and the possibility of conducting complex procedures, such as extended thymectomies and combined mediastinal and pulmonary resections with good results of minimal morbidity, represent the strong points of this technique. Thoracic surgeons experienced in VATS can safely perform subxiphoid uniportal VATS for mediastinal surgery.

Background: To describe a technique of non-intubated uniportal subxiphoid thoracoscopic extended thymectomy.Methods: Data were collected retrospectively. A single 3-cm transverse incision was made below the xiphoid process. This method for extended thymectomy entails adoption of uniportal subxiphoid VATS combined with use of non-intubated anaesthesia for thymoma associated with myasthenia gravis.Results: 10 consecutive patients underwent this procedure successfully. Mean operative time was 102.5 minutes. Conversion to intubated ventilation or thoracotomy was not required. Mean chest tube duration was 3.5 days. Mean postoperative hospital stay was 4.7 days. Histologic examination showed early-stage thymomas. Complications were rare. Quantitative MG scores decreased during follow-up. Conclusions: Patients were uneventfully discharged with fast recovery. This technique may merge the potential benefits of a subxiphoid incision and the non-intubated anesthesia protocol.

Highlights • Minimally invasive thymectomy is the primary surgical means for myasthenia gravis. • Subxiphoid VATS method has the benefit of less blood loss and shorter hospital stay. • Subxiphoid surgical port placement eliminates postoperative intercostal neuralgia. • Subxiphoid uniportal thymectomy is safe and feasible for selected patients., Introduction and importance Myasthenia gravis (MG) is a well-known disease in adults, but the prevalence in children population is low. One treatment option is thymectomy. Transsternal procedure have been the primary approach, but recent trends lean toward minimally invasive technique with triportal and, recently, uniportal thoracoscopic method. This is related to the suggested less blood loss and hospital length of stay. Case presentation Hereby we report a case of a 4-year-old boy suffering from Juvenile Myasthenia Gravis (JMG) with thymic hyperplasia who underwent extended thymectomy via subxiphoid uniportal Video-Assisted Thoracoscopic Surgery (VATS) in our hospital. Pathology evaluation reported a case of thymic hyperplasia. The postoperative outcome was uneventful and satisfactory. Clinical discussion Subxiphoid uniportal approach for thymectomy is relatively new and has not been widely reported. It is important to highlight its advantages and disadvantages compared to other means for thymectomy. Conclusion Subxiphoid uniportal VATS is a safe and feasible approach for thymectomy.

Background Myasthenia gravis (MG) is an autoimmune disease and early thymectomy has been recommended. After the introduction of VATS, the safety and effectiveness of carbon dioxide (CO2) insufflation in thoracic cavity (capnothorax) has been continuously controversial. This study aimed to compare the safety and effectiveness of ventilation methods in bilateral video-assisted thoracoscopic extended thymectomy (BVET) with capnothorax.Methods We retrospectively investigated the medical records of MG patients who underwent BVET between August 2016 and January 2018.Patients were divided into two groups: group D (n=26) for one-lung ventilation and group S (n=28) for two lung-ventilation. We set nine anesthesia time points (T0–T8) and collected respiratory and hemodynamic variables including arterial O2 index (PaO2/FiO2).Results The EtCO2 at T0, T1–T4, and T7 were insignificantly higher in group D than those in group S. The SpO2 at T1–T3 and T8 were significantly lower in group D than those in group S. The FiO2 in group S was lower than that in group D at all-time points. The number of PaO2/FiO2 ≤ 300 and PaO2/FiO2 ≤ 200 were significantly higher in group D than those in group S. Hemodynamic variables were not insignificantly different between the two groups at all-time points. The duration of surgery and anesthesia was shorter in group S than that in group D. Conclusions This retrospective study suggests that anesthesia using two-lung ventilation during BVET with capnothorax was a safe and effective method to improve lung oxygenation and reduce the operation and anesthesia time.

OBJECTIVES Resection of thymic tumours including the removal of both the tumour and the thymus gland (thymothymectomy; TT) is the procedure of choice and is recommended in most relevant articles in the literature. Nevertheless, in recent years, some authors have suggested that resection of the tumour (simple thymomectomy; ST) may suffice from an oncological standpoint in patients with early-stage thymoma who do not have myasthenia gravis (MG) (non-MG). The goal of our study was to compare the short- and long-term outcomes of ST versus TT in non-MG early-stage thymomas using the European Society of Thoracic Surgeons thymic database. METHODS A total of 498 non-MG patients with pathological stage I thymoma were included in the study. TT was performed in 466 (93.6%) of 498 patients who had surgery with curative intent; ST was done in 32 (6.4%). The completeness of resection, the rate of complications, the 30-day mortality, the overall recurrence and the freedom from recurrence were compared. We performed crude and propensity score-adjusted comparisons by surgical approach (ST vs TT). RESULTS TT showed the same rate of postoperative complications, 30-day mortality and postoperative length of stay as ST. The 5-year overall survival rate was 89% in the TT group and 55% in the ST group. The 5-year freedom from recurrence was 96% in the TT group and 79% in the ST group. CONCLUSION Patients with early-stage thymoma without MG who have a TT show significantly better freedom from recurrence than those who have an ST, without an increase in postoperative morbidity rate.

The recent increase in robotic thymectomy research has been met with great enthusiasm and expectation. Previous studies with substantial number of cases have shown that robotic extended thymectomy is a safe and alternative approach to open thymectomy, providing patients with myasthenia gravis (MG) and/or thymoma less postoperative pain, quicker postoperative recovery, and improved cosmesis. Besides, retrospective evidence has suggested efficacy of robotic extended thymectomy on MG in terms of improving symptoms and inducing remission. At present, no consensus on the ideal approach for robotic extended thymectomy has been reached. The left-sided approach might be preferable because of anatomical considerations, including better visualization of the contralateral phrenic nerve, easier management of the upper poles, and more comprehensive access to the common sites of ectopic thymic tissue. After accumulating enough experience, we have also adopted this elegant approach in some special care conditions, such as juvenile MG, elderly MG, relapse MG, and MG with a high BMI (≥30 kg/m2). In this chapter, we describe our technique of robotic extended thymectomy via the left-sided approach and discuss on patient selection, perioperative management, and current evidence and controversies.

Background: infiltration of superior vena cava (SVC) is not rare. The extent of SVC resection depends on the width of the area of neoplastic invasion. Aims: To evaluate the safety of extended thymectomy for Thymic Epithelial Neoplasm (TEN) with SVC resection. Methods: Retrospectively review of thymectomy for TEN in the last twenty years according to STROBE methodology. Disease-free survival (DFS) and overall survival (OS) were calculated using the Kaplan – Meier method. A backward stepwise Cox regression multivariate analysis was performed to determine factors associated with long-term outcomes. Results: 78 patients underwent surgery for advanced-stage TEN (1998 - 2019). 17.9% underwent thymectomy with resection of SVC. Thymic carcinoma was a negative prognostic factor (HR = 2.26; 95% CI = 1.82 – 6.18; p = 0.038) at the multivariate analysis. The median OS and the DFS of SVC resected patients were 50 months (range: 5 – 207) and 31 months (range: 5 – 151). There was no significant difference in OS and DFS between SVC resected and not resected patients. Conclusions: SVC resection is a safe and effective procedure and does not seem to affect survival and disease recurrence. This surgical approach is oncologically reliable to allow radical resection of locally advanced TEN.