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Myasthenia gravis (MG) is the most common autoimmune disorder affecting the neuromuscular junction in the USA. It is not uncommon for these patients to have concomitant autoimmune diseases including autoimmune thyroid disease. We describe here our method of performing a reproducible robotically assisted one-stage thymectomy and thyroidectomy. An African-American woman presented to our institution with a medical history of hypertension, morbid obesity, type 2 diabetes mellitus, symptomatic MG and symptomatic non-toxic substernal multinodular goitre. A one-stage minimally invasive right-sided robotic radical thymectomy and a transcervical total thyroidectomy with excision of the substernal goitre was successfully performed. The treatment of thyroid and thymus pathologies varies drastically from medical observation to surgery. This combined approach surgery clearly benefits the patient by offering similar operative time, fewer operative and postoperative recovery experiences, decreased anaesthesia risks associated with MG patients through fewer intubations, and a faster return to baseline function.

A 41-year-old female presented with dysgeusia, dry eyes, nyctalopia with progressive visual field constriction (due to autoimmune retinopathy) and gastrointestinal symptoms (due to ulcerative colitis). She was subsequently admitted to intensive care with a myasthenic crisis, and CT of the thorax demonstrated a thymoma.Following thymectomy and adjuvant radiotherapy, she has remained in complete remission from her ulcerative colitis and myasthenia gravis, her retinopathy has stabilised and there has been no thymoma recurrence over a 10-year postoperative period. There was a brief relapse of her dysgeusia (causing weight loss) and dry eye symptoms 3 years after her surgery, which resolved 8 months later. While the association of thymomas with paraneoplastic syndromes (PNS) is well established, it is unusual to present with multiple PNS, and some of these have only been documented in sparse case reports to date. Thymectomy played a crucial role in improvement and stabilisation of her PNS.

Myasthenia gravis (MG) is an autoimmune condition affecting the neuromuscular junction characterised by weakness and fatiguability, carrying a high morbidity if treatment is delayed. A clear association with thymoma has led to management with thymectomy as a common practice, but MG presenting post-thymectomy has rarely been reported. We present a case of an 82- year-old woman developing fatigue, ptosis and dysarthria 3 months after thymectomy. After a clinical diagnosis of MG was made, she responded well to prompt treatment with prednisolone and pyridostigmine. Her anti-acetylcholine receptor antibody (anti-AChR) subsequently came back positive. Our systematic review reveals that post-thymectomy MG can be categorised as early-onset or late-onset form with differing aetiology, and demonstrated correlation between preoperative anti-AChR titres and post-thymectomy MG. The postulated mechanisms for post-thymectomy MG centre around long-lasting peripheral autoantibodies. Clinicians should actively look for MG symptoms in thymoma patients and measure anti-AChR preoperatively to aid prognostication.

The robotic platform can be viewed as an advanced thoracoscopic instrument and can be utilized for any pathology amenable to thoracoscopic surgery. This ultimately comes down to surgeon comfort, but many have demonstrated the robotic approach to be useful in benign and malignant mediastinal disease in all compartments with at least equivalent-if not superior-outcomes compared to sternotomy for many metrics. There are various robotic approaches to the same compartments (such as with thymectomy), and no one robotic approach has proven superior to another. Here we describe our robotic approach to common mediastinal pathology.

BackgroundTo explore the efficiency of ectopic thymectomy by the three surgical approaches of trans-sternum, right unilateral thoracoscopy and thoracoscopic subxiphoid in patients with non-thymomatous myasthenia gravis.Methods155 consecutive non-thymomatous myasthenia gravis patients who underwent extended thymectomy by 3 approaches including trans-sternum, right unilateral thoracoscopy and thoracoscopic subxiphoid in 1st affiliated hospital of Sun Yat-Sen University from January 2017 to October 2019 were reviewed. Differences of perioperative clinical characteristics in three surgical approaches were analyzed.ResultsTime to onset of myasthenia gravis (early or late) (p = 0.018), blood loss (p p = 0.031), duration and volume of thoracic drainage (p = 0.039 andp = 0.026), length of hospitalization (p = 0.039), the efficiency of ectopic thymectomy (p = 0.037), and the detection rate of ectopic thymus in the second quadrant (p = 0.018) were different among the three surgical approaches. In univariate logistic regression analysis, higher efficiency of ectopic thymectomy were associated with transsternal (OR 2.36, 95% CI 1.32–4.22,p = 0.011) and thoracoscopic subxiphoid approaches (OR 2.07, 95% CI 1.12–3.82,p = 0.033). In the multiple logistic regression analysis, the transsternal approach (OR 2.02, 95% CI 1.10–3.71,p = 0.024) was an independent protective factor for the efficiency of ectopic thymectomy.ConclusionsBoth the right unilateral thoracoscopic and thoracoscopic subxiphoid approaches have advantages over the transsternal approach in short-term postoperative recovery. Transsternal approach is still the best choice for ectopic thymectomy while thoracoscopic subxiphoid approach show the potential as an alternative way.

A subgroup of myasthenia gravis (MG) patients fail to respond adequately to recommended treatments, a condition referred to as refractory MG. During the refractory phase, patients experience persistent debilitating symptoms with potential life-threatening events or inability to reduce immunosuppressant dosages and minimize long-term toxicities.We conducted a retrospective, single-center study of 113 MG patients to investigate the autoantibody profile and clinical characteristics of refractory MG patients, compared with nonrefractory patients, based on predefined criteria.Fifteen patients (13.3%) were classified as refractory. Double-seronegative MG (DSNMG), without detectable nicotinic acetylcholine receptor (AChR) or muscle-specific tyrosine kinase (MuSK) antibodies, was identified in six refractory patients, significantly higher than those with nonrefractory MG (40% vs 16.3%; P = .031). None of the refractory patients had MuSK antibodies. Patients in the refractory group more frequently had an earlier disease onset, thymic pathology, and thymectomy (P ≤. 03 for all).In this study, patients with refractory MG were more likely than those with nonrefractory MG to be DSN; and refractory DSNMG patients had worse MGFA classes in their recent visit compared with anti-AChR positive refractory patients. Refractory DSNMG patients may represent a distinct group that requires more individualized and targeted treatment approaches.

Introduction The effect of thymectomy on the treatment of childhood-onset myasthenia gravis (CMG) remains debatable. The objective of this study was to evaluate the clinical outcome and relevant prognostic factors of thymectomy for CMG patients. Materials and Methods A total of 32 CMG patients who underwent thymectomy before 18 years of age were included in this retrospective study. Clinical state following thymectomy was assessed by quantified myasthenia gravis (QMG) scores, myasthenia gravis–related activities of daily living (MG-ADL) scores, and Myasthenia Gravis Foundation of America postintervention status. Repeated-measures analysis of variance (ANOVA) examined the changes in postoperative scores during the 5-year follow-up. Univariate logistic regression was applied to identify factors associated with short-term (1-year postoperation) and long-term (5-year postoperation) clinical outcomes. Results Repeated-measures ANOVA showed that QMG scores (F = 6.737, p Conclusion Our results suggest that thymectomy is effective in treating CMG. Thymectomy could be recommended for CMG patients, especially for patients in the early course of GMG.

Background Parathyroidectomy for primary hyperparathyroidism (pHPT) is safely performed in the outpatient setting in the adult population. However, concern that children and adolescents have higher complication rates and are unable to recognize and communicate symptoms of hypocalcemia has limited same-day discharges in the pediatric population. Methods Nineteen patients aged 8–18 years (14.1 ± 0.7) underwent outpatient parathyroidectomy for pHPT by a single high-volume endocrine surgeon from 2002–2020. Patient demographics, disease, operations, and complications were reviewed. Results Sixteen of 19 patients were symptomatic with fatigue (62.5%), joint pain (37.5%) and nephrolithiasis (18.7%) most common. Mean preoperative Ca and PTH were 11.7 ± 0.3 mg/dL and 102.3 ± 11.8pg/mL, respectively. Ten of 19 had a single adenoma and 9 had multigland hyperplasia including one MEN1 and one MEN2A patient. We performed 11 four-gland explorations, 8 unilateral parathyroidectomies; including 9 transcervical thymectomies, 1 total thyroidectomy, and 1 bilateral central neck dissection. Mean 6-month postoperative Ca and PTH levels were 9.5 ± 0.3 mg/dL (range 7.3–10.3) and 29±5.0pg/mL (range 6.3–77), respectively. One patient developed permanent hypoparathyroidism and 1 had temporary hypocalcemia. No temporary or permanent hoarseness, unplanned same-day admission, wound complications, or Emergency Department visits occurred. Conclusion Outpatient parathyroidectomy can be safely and effectively performed in pediatric patients with primary HPT. Level of Evidence Treatment Study, Level III.

Robotic subxiphoid transthoracic thymectomy showed several surgical advantages. Nevertheless, the intercostal insertion of trocars may lead to nerve injury with enhanced post-operative pain. Moreover, the dissection of peri-diaphragmatic mediastinal fat may result challenging, in particular on left side, where the presence of the heart precludes the optimal visualization. We describe a preclinical cadaveric study of a novel full subcostal robotic-assisted technique to overcome these limitations. A total subcostal robotic-assisted radical thymectomy was evaluated on a cadaver model using the da Vinci Xi system. The exploratory procedure was divided in two steps: (a) dissection of the thymus gland, except the left mediastinal epi-diaphragmatic fat pad; (b) dissection of the left diaphragmatic mediastinal fat pad avoiding heart compression while perfectly visualizing the left phrenic nerve. Five different setups were explored based on camera and trocars insertions, patient's positioning and table's settings. Both the tasks were accomplished using the novel technique. The subxiphoid insertion of the camera and the position of two robotic arms about 8 cm distally on the subcostal made the most part of mediastinal dissection straightforward. Left peri-diaphragmatic fat pad can be better visualized and dissected positioning the camera in the left subcostal port shifting the instruments on the right side. This may permit a better control of the left phrenic nerve reducing heart compression. Full subcostal robotic-assisted thymectomy resulted feasible in cadaveric model. Clinical trial should be performed to confirm the translational use of this novel technique and the speculated advantages in living model.

Background Surgical resection of the thymus is indicated in the presence of primary thymic diseases such as thymoma. Video‐assisted thoracoscopic surgery (VATS) and robot‐assisted thoracic surgery (RATS) offer a minimally invasive approach to thymectomy. However, there is no clear conclusion whether RATS can achieve an equal or even better surgical effect when compared with VATS in treatment of thymoma. We performed this meta‐analysis to explore and compare the outcomes of RATS versus VATS for thymectomy in patients with thymoma. Methods PubMed, Cochrane Library, EMBASE, China National Knowledge Infrastructure (CNKI), Medline, and Web of Science databases were searched for full‐text literature citations. The quality of the articles was evaluated using the Newcastle–Ottawa Scale and the data analyzed using Review Manager 5.3 software. Fixed or random effect models were applied according to heterogeneity. Subgroup analysis was conducted. Results A total of 11 studies with 1418 patients, of whom 688 patients were in the RATS group and 730 in the VATS group, were involved in the analysis. Compared with VATS, RATS was associated with less blood loss in operation, lower volume of drainage, fewer postoperative pleural drainage days, shorter postoperative hospital stay, and fewer postoperative complications. There was no significant difference in operative time and patients with or without myasthenia gravis between the two groups. Conclusions RATS has more advantages over VATS, indicating that RATS is better than VATS in terms of postoperative recovery. We look forward to more large‐sample, high‐quality randomized controlled studies published in the future., Surgical resection of the thymus is indicated in the presence of primary thymic diseases such as thymoma. Video‐assisted thoracoscopic surgery (VATS) and robot‐assisted thoracic surgery (RATS) offer a minimally invasive approach to thymectomy. However, there is no clear conclusion whether RATS can achieve an equal or even better surgical effect when compared with VATS in treatment of thymoma. We performed this meta‐analysis to explore and compare the outcomes of RATS versus VATS for thymectomy in patients with thymoma. A total of 11 studies with 1418 patients, of whom 688 patients were in the RATS group and 730 in the VATS group, were involved in the analysis. Compared with VATS, RATS was associated with less blood loss in operation, lower volume of drainage, fewer postoperative pleural drainage days, shorter postoperative hospital stay, and fewer postoperative complications. There is no significant difference in operative time and patients with or without myasthenia gravis between the two groups. RATS has more advantages over VATS, indicating that RATS is better than VATS in terms of postoperative recovery. We look forward to more large‐sample, high‐quality randomized controlled studies published in the future.

In February 2020, a 51-year-old woman experienced leg myalgia and noticed calf muscle movements that resembled a rippling wave while crouching down. In June 2020, she complained of bilateral arm myalgia. In August 2020, she developed left ptosis, had difficulty raising her bilateral arms, and developed diplopia and was admitted to our hospital. Anti-acetylcholine receptor antibodies turned out to be positive. We made a diagnosis of myasthenia gravis and acquired rippling muscle disease (RMD). Her myasthenia gravis symptoms and myalgia decreased with oral prednisolone. Contrast-enhanced computed tomography revealed thymoma. She underwent extended thymectomy and was discharged from the hospital. Her myalgia worsened, but it was responsive to methylprednisolone pulse therapy. CAV3 gene mutations are recognized as causes of congenial RMD whereas acquired RMD is associated with myasthenia gravis. Acquired RMD is rarely reported in Japan, but should be kept in mind as a condition treatable with immunotherapy.

We report a 62-year-old woman with thymoma associated myasthenia gravis (MG). She had significant dysphagia and was treated with corticosteroids, intravenous immunoglobulin (IVIG), immunoadsorption plasmapheresis (IAPP), and immunosuppressive drugs, and the extended thymectomy. Her symptoms gradually improved, but 3 weeks after thymectomy, her bulbar symptoms recurred. Although she was treated with repeated IVIG and IAPP, her symptom remained. Finally, after starting eculizumab did her symptoms go into complete remission. This case suggests the efficacy of anti-complement therapy for postoperative exacerbation of MG.

The optimal extent of surgical resection for non-myasthenic patients with thymoma is controversial. The objective of this meta-analysis was to compare complete to partial thymectomy in non-myasthenic patients for oncological and postoperative clinical outcomes.We performed a PubMed and EMBASE search (from inception to January 2020) for English-language studies directly comparing partial thymectomy (thymomectomy) to complete thymectomy for thymoma resection. Clinical endpoints studied included overall and disease-free survival, Masaoka and World Health Organization staging, adjuvant therapy, postoperative complications, postoperative drainage, length of hospital stay, thymoma-related deaths, postresection development of myasthenia gravis, incomplete resection, and recurrence. Random effects meta-analyses across all clinical endpoints was done.There was no statistically significant difference between the two approaches with regard to recurrence (odds ratio [OR], 1.22; 95% confidence interval [CI], 0.78-1.92), completeness of resection (OR, 1.17; 95% CI, 0.66-2.10), adjuvant therapy (OR, 0.71; 95% CI, 0.40-1.26), or thymoma-related deaths (OR, 0.76; 95% CI, 0.12-4.66). There was a statistically significant decrease in postoperative complications (OR, 0.61; 95% CI, 0.39-0.97), drainage (mean difference [MD], -0.99; 95% CI, -1.98 to -0.01), and length of hospital length (MD, -1.88; 95% CI, -3.39 to -0.36) with partial thymectomy.The evidence appeared to suggest that partial thymectomy is oncologically equivalent to complete thymectomy for non-myasthenic patients with early-stage thymoma. There is an additional advantage of reduced postoperative complications and decreased length of hospital stay with partial thymectomy.

Although more than 90% of children born with congenital heart disease (CHD) survive into adulthood, patients face significantly higher and premature morbidity and mortality. Heart failure as well as non-cardiac comorbidities represent a striking and life-limiting problem with need for new treatment options. Systemic chronic inflammation and immune activation have been identified as crucial drivers of disease causes and progression in various cardiovascular disorders and are promising therapeutic targets. Accumulating evidence indicates an inflammatory state and immune alterations in children and adults with CHD. In this review, we highlight the implications of chronic inflammation, immunity, and immune senescence in CHD. In this context, we summarize the impact of infant open-heart surgery with subsequent thymectomy on the immune system later in life and discuss the potential role of comorbidities and underlying genetic alterations. How an altered immunity and chronic inflammation in CHD influence patient outcomes facing SARS-CoV-2 infection is unclear, but requires special attention, as CHD could represent a population particularly at risk during the COVID-19 pandemic. Concluding remarks address possible clinical implications of immune changes in CHD and consider future immunomodulatory therapies.

Thymectomy is an established treatment in adult myasthenia gravis, but its exact role in juvenile myasthenia gravis (JMG) is still uncertain. Thymectomy is frequently considered in the treatment of severe, medically refractory JMG. Surgical approaches have evolved from open median sternotomy to the more cosmesis-preserving thoracoscopic approach. This paper reviews current evidence on the effectiveness of thymectomy in JMG and discusses clinical characteristics which may be associated with improved outcomes. 17 studies including 588 patients who underwent thymectomy from 1997 to 2020 were found, which either reported uncontrolled cohorts undergoing thymectomy, or compared cohorts undergoing different surgical approaches. An improvement in clinical status or reduced requirement for medical therapy following thymectomy was seen in 453 patients (77%). Complete remission was seen in 40% (n = 172/430). Thoracoscopic approaches may provide improved outcomes, fewer complications, and better cosmetic outcomes. Better surgical outcomes may be associated with early intervention, intervention after the onset of puberty, being acetylcholine receptor antibody positive, having more severe disease and the presence of hyperplastic thymic tissue. However, analysis remains hindered by the limitations of the currently available retrospective studies of small cohorts. Nonetheless, available literature suggests a role for thymectomy in JMG patients, especially those with certain clinical characteristics.

Objective Identifying the morphological features of thymus in patients with myasthenia gravis (MG) with anti-acetylcholine receptor (AChR) antibodies and concomitant Hashimoto's thyroiditis (HT), which were recruited from a single surgical unit of a tertiary referral hospital located in the North-Eastern region of Romania, over a period of 11 years. Patients, materials and methods We retrospectively reviewed clinical, imaging, laboratory, thymic pathology, and outcome data that were obtained from medical records of patients with MG and concomitant HT, to whom a thymectomy was performed for a suspected thymic lesion. All the surgical interventions were done in the Third Clinic of Surgery, St. Spiridon Emergency County Hospital, Iasi, Romania, for an 11 years' period, i.e., from January 1, 2000 and December 31, 2010. Results Four patients (three females and one male) were included. The mean age of the patients at the time of their thymectomy was 40.25 years. Of all patients, 75% had moderate or severe MG, 100% had anti-AChR antibodies, and an electromyographic decrement greater than 25%. All patients have been diagnosed with HT in their past medical history by a full thyroid panel [high thyroid-stimulating hormone (TSH) values, low free thyroxine (fT4) values, and the presence of the anti-thyroid antibodies] and all of them have been treated with Euthyrox. Our four patients expressed different MG subtypes, each of them being associated with different thymus pathology. Thoracic computed tomography (CT) scan revealed heterogeneous mediastinal masses and established the correct diagnosis only in 25% of cases. The pathological exams also revealed a heterogeneous pattern of thymic lesions. In contrast with other studies, our patients with MG with anti-AChR antibodies and concomitant HT presented atrophic thymus more frequently (50%), but with particular morphological changes of Hassall's corpuscles. Also, 25% of cases were diagnosed with thymic lympho-follicular hyperplasia (TLFH) associated with thymic epithelial hyperplasia. In B2 thymoma, neoplastic epithelial cells expressed cytokeratin 19 (CK19) immunoreactivity, high Ki67 labeling index and strong p63 immunopositivity. Conclusions In our series, MG and HT occurred simultaneously, or one of them was diagnosed before the other, raising some new questions regarding the immune mechanism of these two autoimmune diseases. Due to the heterogeneous morphological changes of the thymus that we found in this study, we can hypothesize that thymus is involved in the pathogenic mechanism of MG with anti-AChR-antibodies and concomitant HT development.

Background The study's goal was to investigate the percentage of anxiety and depression in Chinese thymoma patients before surgery, and also the factors that influence it. Methods The study included patients who had an anterior mediastinal mass discovered by chest CT and were scheduled for video-assisted thoracoscopic surgery. The mental health rating scales were completed by all patients before surgery. Patients were divided into two groups based on the Hospital Anxiety and Depression Scale (HADS): anxiety/depression and non-anxiety/depression. The association between thymoma clinical factors and the HADS score was studied statistically. Results The study comprised eighty patients with thymoma. Before the operation, 22.5% (18/80) of the patients had anxiety and/or depression. The resigned coping style characteristics, along with myasthenia gravis (MG), were associated with preoperative anxiety and depression. The greater the score of the resigned dimension, the greater the risk of anxiety and depression, based on the results of logical regression analysis. Thymoma patients with myasthenia gravis have a higher risk of anxiety and depression. Conclusion Patients with myasthenia gravis and resigned coping style were found to have higher anxiety and depression before surgery for Chinese thymoma patients.

Lipofibroadenoma is an extremely rare thymic tumor, and the anterior mediastinum is the most common site.A 21-year-old male was admitted with fever without obvious cause for 2 months. After admission, the patient's highest temperature was 38.3°C, accompanied by diarrhea. Physical examination showed coarse breath sounds in both lungs. Chest enhanced computed tomography (CT) showed a mass of mixed density shadow on the left side of the anterior mediastinum with a size of approximately 9.2 cm × 5 cm × 2.1 cm and a clear boundary mixed with a low fat density shadow. Mediastinal tumors were removed under general anesthesia by video-assisted thoracoscopic surgery. Macroscopically, a clear boundary was shown between the tumor and the remaining thymus. Microscopically, the tumor contained a large amount of mature adipose and fibrous tissue with scattered cord-like epithelial tissue and a small number of lymphocytes scattered in the stroma. The tumor lacked thymic bodies. The neoplastic epithelial cells were oval or polygonal and arranged in fissures, the nuclei were uniform in size and mild in shape, and mitosis was rare. Epithelial cells were positive for AE1/AE3 and CK19, lymphocytes were positive for CD3 and CD20, and fat and fibrous tissue were positive for S-100 and vimentin, respectively. The Ki67 labeling index was less than 5%. Based on histological features and immunophenotype, thymic lipofibroadenoma was diagnosed. The patient was followed up 1 year after the operation, and no recurrence or residual lesions were found on the X-ray re-examination.Lipofibroadenoma is a benign thymic tumor, and thymectomy is regarded as the best treatment. The biological behavior of thymic lipofibroadenoma is good, and the recurrence rate is low.

Background 2‐deoxy‐2‐[fluorine‐18] fluoro‐d‐glucose (18F‐FDG) positron emission tomography (18F‐FDG‐PET) is a convenient modality to assess the metabolic activity within tumor cells. However, there is no consensus regarding the relationship between 18F‐FDG uptake and the immune environment in thymic epithelial tumors (TETs). We conducted a clinicopathological study to elucidate the relationship between 18F‐FDG uptake and programmed death ligands 1 and 2 (PD‐L1/PD‐L2) expression in patients with TETs. Methods: A total of 108 patients with histologically confirmed TETs classified as thymomas or thymic carcinomas who underwent surgical resection or biopsy or needle biopsy and 18F‐FDG PET before any treatment between August 2007 and March 2020 were enrolled in this study. Tumor specimens underwent immunohistochemical staining for PD‐L1, PD‐L2, GLUT1, HIF‐1α, VEGFR2, VEGF‐C, and β2 adrenergic receptor. Results: High uptakes of SUVmax, SUVmean, MTV, and TLG were identified in 28 (25.9%), 61 (56.5%), 55 (50.9%), and 55 (50.9%) of 108 patients, respectively. High uptake of SUVmax significantly correlated with PS (performance status) of 1–2, thymic carcinoma, and advanced stage, and SUVmax on 18F‐FDG uptake displayed a close association with PD‐L1 and PD‐L2 expressions, but not with MTV and TLG. Our analysis revealed that SUVmax was identified as being significant relationship for positive PD‐L1/PD‐L2 expression. GLUT1, HIF‐1α, and VEGFR2 were significantly associated with the expression of PD‐L1/PD‐L2 from the biological viewpoint. Conclusion 18F‐FDG accumulation was closely associated with the expression of PD‐L1/PD‐L2, which, in turn, was correlated with glucose metabolism and hypoxia. PD‐L1/PD‐L2 could affect the glucose metabolism and hypoxia in thymic tumor cells., High expression of PD‐L1/PD‐L2 was closely associated with high accumulation of 18F‐FDG. Angiogenetic markers were linked to the expression of PD‐L1/PD‐L2. PD‐L1 and PD‐L2 exhibited a close relationship with upregulation of tumor glucose metabolism and hypoxia.

Background The subxiphoid single-port approach for thymectomy has several advantages compared with conventional lateral transthoracic approaches. However, perioperative outcomes of subxiphoid single-port thymectomy are still lacking. The objective of this study is to present our 6-year experience of subxiphoid single-port thymectomy, including our learning curve. Methods All consecutive 49 patients undergoing subxiphoid single-port thymectomy between August 2014 and September 2020 were reviewed retrospectively. Results We analyzed data of 49 patients (27 males and 22 females) with a median age of 58 years (range 49-68). Two patients presented postoperative complications. The conversion to a different approach was required in four cases. The median follow-up time was 17 months (range 3-35) and locoregional recurrence was observed in one patient. There were no intraoperative deaths and postoperative mortality. The operation time cumulative summation learning curve analysis revealed that the curves descended from the 24th case. Conclusions The subxiphoid single-port approach for thymectomy is a safe and feasible modality with few complications.

Background The long-term outcomes of robotic thymectomy for thymic epithelial tumors (TETs) are not well known, even though the early postoperative outcomes have improved. This study aimed to report the long-term survival and recurrence in patients with TETs who underwent robotic thymectomies. Methods A total of 158 patients who underwent robotic thymectomy for TET and who were followed-up for more than 1 year were included in the study. The median follow-up time was 43 (interquartile range, 40) months, and 156 (98%) patients were followed completely until the end of the study period. Results The mean age of the patients was 55.7 ± 12.7 years, and the mean size of the tumor was 4.6 ± 2.1 cm. There was no postoperative mortality and postoperative complications occurred in 7 (4.4%) patients. The median length of the postoperative hospital stay was 2 (interquartile range, 1-20) days. Thymoma was the most common cell type (n = 132, 84%), and thymic carcinoma (n = 24, 15%) and neuroendocrine tumors (n = 2, 1%) were the next most common types. Advanced stages more than stage III were identified in 15 patients (stage IIIA: n = 7, 4%; stage IVA: n = 5, 3%; and stage IVB: n = 3, 2%). The 5-year disease-specific survival was 100% in thymoma and 95% in thymic carcinoma. The 5-year recurrence-free survival was 94% in thymoma and 79% in thymic carcinoma. Conclusions Robotic thymectomy could achieve favorable long-term survival and recurrence rates, comparable to open or thoracoscopic thymectomy.

Background There were very few reports of Rosai–Dorfman disease in the thymus, which known as sinus histiocytosis with massive lymphadenopathy. It usually accompanied with other systemic symptoms such as fever, malaise, night sweats, or weight loss in the short term. Case presentation We herein report a rare case of RDD of thymic origin and a review of the literature concerning the clinical and pathological features of this disease, which is often misdiagnosed as thymoma. The patient was underwent thymectomy to extirpate the lesion completely by video-assisted thoracic surgery. Conclusions To the best of our knowledge, this is the fourth thymus occurring RDD case with proof via histology and IHC. Our findings suggest the difficulty of making a diagnosis before surgery and more cases will need to be reported in order to facilitate the preoperative diagnosis of such a rare tumor.

Pure red cell aplasia (PRCA) is a rare paraneoplastic syndrome observed in 2-5 % of thymomas. Literature reports great variability in its management. Based on an illustrative clinical case, we present a systematic literature review whose main objective is to evaluate the therapeutic management of PRCA. The literature search was performed based on the PICO method in the Medline and Scopus databases. The reference clinical case concerns a 51-year-old woman with stage IVa thymoma. After initial response to chemotherapy, a locoregional progression occurred with PRCA development that responded favorably under second line chemotherapy. The patient finally died in a context of bicytopenia with febrile neutropenia. The systematic review covers 135 articles published between 1950 and 2019. Thymectomy alone or in combination with other therapies showed a 31 % complete remission (CR) rate for PRCA of, whereas none was reported with anti-tumor treatments without thymectomy. Among immunomodulatory therapies, cyclosporin gave the highest percentage of CR (74 %). Finally, the combination of thymectomy and immunomodulatory treatments showed a CR rate of 45 %. Thymectomy appeared to be the most effective anti-tumor treatment for PRCA. Immunomodulatory therapies, particularly cyclosporine, are shown effective, but the risk of infectious complications must be considered. The optimal place of anti-tumor and immunomodulatory therapies against PRCA has yet to be determined.

Objective: To determine the effect of tetanus toxoid (TT) revaccination on circulating B-, T-and NK-cell com-partments in myasthenia gravis (MG) patients.Methods: Lymphocyte (sub)populations and differentiation stages were assessed by flow cytometry in 50 TT revaccinated MG patients. TT-specific proliferative responses were explored in PBMC cultures. Results: In patients treated with azathioprine B-and NK cell numbers were strongly decreased. Lymphocyte (sub) populations remained unaffected upon TT revaccination. t All patients showed a significant TT-induced prolif-erative response.Conclusion: TT revaccination is effective in MG patients with stable disease irrespective of their thymectomy status and medication and does not alter the composition of the lymphocyte compartment.

Background Several retrospective studies have identified risk factors associated with ocular myasthenia gravis (OMG) generalization in non-surgical patients. However, the outcomes of OMG after thymectomy have not been investigated fully. This study aimed to explore the clinical predictors of post-thymectomy OMG prognosis. Methods We performed a retrospective review of OMG patients who underwent thymectomy at our institution from January 2012 to December 2021. Kaplan–Meier and Cox proportional hazard regression analyses were used to evaluate associations between clinical features and prognosis. The main outcome measures were OMG conversion, complete stable remission (CSR), and clinical improvement. Results Fifty-eight patients were identified for conversion analysis. Thirteen (22.4%) developed generalized myasthenia gravis (GMG) at a median time of 12.7 (3–37.3) months from symptom onset. Repetitive nerve stimulation (RNS)-positivity was associated with increased risk of conversion to GMG (P = 0.002). Patients with histotype B2/B3 thymoma showed a higher risk of conversion (P = 0.002) than did patients with hyperplasia and AB/B1 thymoma. Fifty-two patients fulfilled the criteria for CSR and improvement. Sixteen (30.8%) achieved CSR at a median time of 28.7 (15–54) months after thymectomy. Fifteen (28.8%) showed clinical improvement at last follow up. Patients who achieved CSR showed a younger age of onset (P = 0.022), lower percentage of acetylcholine receptor antibody-seropositivity (P = 0.029). Histologically, patients with thymic hyperplasia and stage I thymoma showed a higher chance of CSR (P = 0.010) than did patients with stage II/III thymoma. Multivariate analysis revealed that RNS-positivity (hazard ratio [HR] 6.007, P = 0.021) and histotype B2/B3 thymoma (HR 4.611, P = 0.048) were associated with OMG conversion. Thymic hyperplasia and stage I thymoma (HR 0.300, P = 0.026) were associated with OMG CSR after thymectomy. Conclusion For OMG patients after thymectomy, RNS-positivity and histotype B2/B3 thymoma are independent predictors of conversion to GMG. On the other hand, thymic hyperplasia and stage I thymoma independently predict CSR.

The aim of this study was to evaluate the long-term results of robot-assisted (RATS) thymectomy in the treatment of large thymomas, defined as larger than 5 cm.We retrospectively reviewed 106 thymectomies from 2010 to 2020, creating two groups based on the surgical approach (open or RATS) and size. Kaplan-Meier and Cox-regression were used to estimate and identify risk factors of oncological outcomes. To perform a well-balanced analysis, a propensity score matched (PSM) analysis was conducted for large thymomas.From 2015, we performed 54 RATS thymectomies: 53.7% (n = 29) for small and 46.3% (n = 25) for large thymomas. Conversions were similar and all patients had a complete resection. The overall (82% vs. 92%, p = 0.57) and disease-free survival were comparable between RATS and open (92.5% vs. 93%, p = 0.67), outcomes confirmed after PSM for large thymomas.RATS thymectomy could be considered a valid option in selected patients with large thymomas.

Background Myasthenia gravis (MG) is the most common paraneoplastic syndrome in thymoma. However, the association between MG and postoperative outcomes is controversial. Therefore, we examined the effect of MG on the surgical outcomes of patients with thymoma. Methods This study enrolled 145 consecutive patients with thymoma who underwent surgical resection at our institution between January 2000 and December 2020. The patients were classified into thymoma with MG (MG group) and without MG (non-MG group). Data about characteristics of patients, surgical outcomes, and prognostic factors were compared between the two groups. Results Of 145 patients, 47 (32%) presented with MG and 98 (68%) did not. There was no significant difference in terms of the incidence of postoperative complications, overall survival (OS), and recurrence-free survival (RFS) between the two groups. The deaths were not caused by thymoma. Among the patients aged >60 years, the MG group had a lower survival rate than the non-MG group. In the univariate analysis, age ≥60 years was a poor prognostic factor for OS, whereas in the multivariate analysis, Masaoka stage III and IV classifications were poor prognostic factors for RFS. Conclusion The incidence of postoperative complications did not differ between patients with thymoma and without MG. In the MG group, age ≥60 years was a poor prognostic factor for OS. The postoperative follow-up of patients aged ≥60 years with thymoma with MG should focus on not only recurrence but also progression of diseases other than thymoma.

Thymectomy specimens are uncommon in routine histopathology practice. However, awareness of various pathologic entities and definite reporting of these specimens is paramount to optimal patient management. Our objective was to determine the histomorphologic spectrum of thymectomy specimens spanning the non-neoplastic, benign to malignant spectrum.Thymectomies received over an 8-year period were retrospectively analyzed by reviewing clinical details and histologic findings in detail, incorporating the latest World Health Organization (WHO) 2015 histologic classification. A total of 303 thymectomy specimens (179 males/124 females, mean age 45.3 years [3-84 years]) were included. Around 51.2% (n = 155) patients had associated myasthenia gravis (MG), while 17.5% (n = 53) had incidentally detected anterior mediastinal mass (AMM). Non-neoplastic and benign pathologies comprised 31% (n = 94) cases and showed stronger association with MG (P = 0.009). Thymic follicular hyperplasia (TFH) was the commonest non-neoplastic pathology (n = 32), while the benign tumor group included thymic hemangioma/lymphangioma, thymolipoma, and ectopic parathyroid adenoma. Thymic epithelial tumors (TETs) comprised 64.7% cases, with majority being thymomas (185/303; 61.1%). Thymoma type B2 was the commonest histologic subtype and Stage I/T1 was the most frequent stage. Type A and AB thymomas affected older patients (P = 0.005) and were in lower stage (both Masaoka and American Joint Committee on Cancer [AJCC]) than type B thymomas (P = 0.007). No significant association between MG and thymoma subtype, patient sex or Masaoka stage was seen (P0.05). Thymic carcinomas comprised 11 cases and showed no association with MG (0/11, P0.001); squamous cell carcinoma was the commonest histologic type (8/11; 72.7%).TETs are the commonest thymic lesions; however, a diverse spectrum of pathologic processes can affect the thymus.

Thymic cancer associated with spontaneous regression of thymic cysts is a rare disease. A 47-yearold man was referred to our hospital for right chest pain and chest abnormal shadow. Chest computed tomography( CT) revealed a solid lesion 1.3 cm in diameter and a cystic lesion 1.0 cm in diameter at the right anterior mediastinum. A second CT study after six months showed a solid lesion increased to 1.7 cm in diameter and a cystic lesion reduced to 0.7 cm in diameter. A second magnetic resonance imaging (MRI) showed a cystic lesion reduced and high signal intensity region in the thymus enlarged on T2-weighted imaging. Under the diagnosis of thymoma associated with multilocular thymic cysts, total thymectomy was performed for these mediastinal lesions by video-assisted thoracic surgery. Histopathological finding was thymic squamous cell carcinoma (Masaoka stage II) associated with multilocular thymic cysts. Additional postoperative radiotherapy was performed, and there has been no recurrence after one postoperative year.

Thymic neoplasms are a relatively uncommon tumor, with the anterior mediastinum being the most common. Median sternotomy is the procedure of choice for the treatment of thymomas. With the advent of thoracoscopy, an increasing number of countries are adopting the right thoracic approach for the treatment of thymomas, but there are still no clear surgical standards or modalities to treat thymic carcinoma. We propose a modified subxiphoid subcostal arch thoracoscopic enlarged thymectomy to treat thymic carcinoma based on various reviews. We have also reviewed the relevant literature on the subject of evidence-based medicine. The evaluation of CD70 in combination with CD5 and CD117 or preferentially expressed antigen in melanoma in combination with CD5 and CD117 may help to diagnose thymic squamous cell carcinoma (TSCC) more accurately. The modified thoracoscopic expanded thymic resection under the costal arch of the xiphoid process is not only suitable for TSCC but also for thymic cyst, thymoma, locally invasive thymoma, and thymic carcinoma.

OBJECTIVES About one-third of patients with thymoma have myasthenia gravis (MG). It remains controversial whether MG affects the prognosis of patients with thymoma. The aim of this study was to evaluate the effect of MG on the prognosis of patients with thymoma in a multicentre database. METHODS Patients with thymoma who underwent thymectomy were identified from 2 prospectively collected databases in 2 medical centres from 2010 to 2018. Kaplan–Meier curves and the log-rank test were used to assess overall survival and recurrence-free survival, and a Cox proportional hazards model was used to determine significant contributors to survival. Propensity score matching was performed to eliminate selection bias. RESULTS A total of 514 patients with thymoma were included in this study, of whom 320 patients were MG-free and 194 had MG. Patients with MG were younger (median age 50 vs 54 years, P = 0.001) and had smaller tumours (4.4 ± 2.0 vs 4.9 ± 2.3 cm, P = 0.020). Pathological analysis showed that type B tumours especially B2−B3 (B2 + B3 + mix B tumours, 55.2%) are more common in patients with MG, while type AB (37.2%) was the most common in patients without MG. A larger proportion of Masaoka III–IV stage tumour (25.7% vs 11.0%, P CONCLUSIONS Thymoma patients with MG have smaller tumours and a higher percentage of late-stage tumours, which are mainly of WHO B types, especially B2−B3 types. In addition, MG is significantly associated with worse overall survival and recurrence-free survival in thymoma.

The value of thymectomy in the treatment of non-thymomatous myasthenia gravis has been controversially discussed. The relatively low incidence and prevalence of this disease, the inconsistent documentation in various studies and the necessity of a long-term follow-up to assess the therapeutic effects has made the generation of valid data difficult. The publication in 2016 of the MGTX trial in the New England Journal of Medicine delivered the first randomized controlled data in which patients aged 18-65 years with generalized myasthenia gravis and positive for acetylcholine receptor antibodies showed a significant benefit after surgical resection of the thymus via median sternotomy. Despite a lack of validation of the advantages of thymectomy by minimally invasive surgery from randomized controlled studies, this technique seems to positively influence the outcome of certain patient groups in a similar way. Video-assisted thoracoscopic surgery (VATS) and robotic-assisted thoracic surgery (RATS) using subxyphoidal and transcervical access routes showed not only esthetic advantages but also showed no relevant inferiority in the influence on clinical outcomes of myasthenia gravis compared to median sternotomy; however, not only the benefits and the esthetic results show differences but also the advantages in the various subtypes of myasthenia gravis show divergent prospects of success with respect to remission. The clinical spectrum of myasthenia is heterogeneous with respect to the occurrence of antibodies, the body region affected and the age of the patient at first diagnosis. Ultimately, thymectomy is an effective causal treatment of myasthenia gravis.Der Stellenwert der Thymektomie in der Therapie der thymomfreien Myasthenia gravis blieb bis vor einiger Zeit umstritten. Die relativ geringe Inzidenz und Prävalenz der Erkrankung, die uneinheitliche Dokumentation in den verschiedenen Studien sowie die notwendige Langzeitbeobachtung zur Erfassung therapeutischer Effekte erschwerten das Generieren valider Daten. Die Veröffentlichung des MGTX-Trials 2016 im New England Journal of Medicine lieferte die ersten randomisiert-kontrollierten Daten, nach denen Patienten mit Acetylcholin-Rezeptor-Antikörper-positiver generalisierter Myasthenia gravis im Alter von 18 bis 65 Jahren von der chirurgischen Resektion des Thymus über eine mediane Sternotomie profitieren. Trotz fehlender Validierung des Vorteils der Thymektomie über minimal-invasive Techniken durch randomisiert-kontrollierte Studien scheinen diese das Outcome bestimmter Patientengruppen in ähnlicher Form positiv zu beeinflussen. So haben videoassistiert-thorakoskopische, roboterassistierte, subxiphoidale und transzervikale Zugangswege nicht nur ästhetische Vorteile, sondern zeigen in der Beeinflussung des Krankheitsverlaufs der Myasthenia gravis keine relevante Unterlegenheit gegenüber der medianen Sternotomie. Doch nicht nur der Nutzen und das ästhetische Ergebnis differieren, sondern auch die Erfolgsaussichten im Hinblick auf die Remission sind bei den Unterformen der Myasthenia gravis unterschiedlich. Die heterogene Gruppe der Myasthenien unterscheidet sich bezüglich des Auftretens von Autoantikörpern, der betroffenen Körperregionen und des Alters der Patienten bei Erstdiagnose. Schließlich ist die Thymektomie eine wirksame kausale Therapie der Myasthenia gravis.

Background Myasthenia gravis is a rare autoimmune disease caused by antibodies that probably originate from the thymus glands. This study examined the epidemiology of patients with MG, who underwent thymectomy over the last three decades. Methods The objectives of this observational study were to investigate the clinicopathological features, treatment modalities, and prognostic factors for patients with thymic masses, over three decades at the Royal Hospital, Muscat, Oman. Results There were 100 patients who underwent thymectomy with a mean (SD) age of 32.0 (8.6) years, of which 20% were men and 80% were women. Their follow up period, cardiac and neurology clinics, ranged from 1.5 to 12.0 years with a mean (SD) of 6.0 (3.0) years. Small percentage of MG patients had diabetes and hypertension and 10% of patients have positive family history of MG. Symptoms at the onset of the disease were ophthalmoplegia in 75%, limb weakness in 39%, bulbar symptoms in 57% and respiratory symptoms in 39% of patients. The Osserman grading was Grade I – 5%, Grade IIA – 39%, Grade IIB – 34%, and Grade III – 22%. Post thymectomy, 21% of patients had complete clinical remission, 76% of patients had significant clinical improvement and 3% had no apparent improvement in their clinical status. Histologically, hyperplasia was found in 57% and involuted thymus in 18% of patients. Conclusion Thymectomy can reduce patient’s need for medication and reduce the severity of MG regardless of age, sex, severity, or length of sickness, or thymic masses. The early-onset, sever M.G, female, thymic hyperplasia benefit the most. Patients classified as Osserman Class IIA and IIB benefit most from this procedure.

Background The effects of thymectomy on late-onset non-thymomatous myasthenia gravis (NTMG) remain controversial. The objective of this study was to conduct a systematic review in order to answer two questions pertinent to late-onset NTMG: (1) do patients with late-onset NTMG experience the same effects from thymectomy as their early-onset counterparts? (2) Compared with conservative treatment, does thymectomy have any benefits for late-onset NTMG patients? Methods We searched the PubMed, EMBASE, and Cochrane Library databases for studies published from January 1, 1950 to March 10, 2021. Outcomes were measured via clinical stable remission/pharmacological remission (CSR/PR) and improvement rates. We used Stata software to analyze the data. Results We ultimately included a total of 12 observational articles representing the best evidence answering the questions of our study objective. Of these, nine studies, which included 896 patients overall (766 early-onset and 230 late-onset), compared postoperative outcomes between early- and late-onset NTMG. The remaining three articles, which included 216 patients (75 in the thymectomy group and 141 in the conservative-treatment group), compared thymectomy with conservative treatment for late-onset NTMG. The early- versus late-onset NTMG studies demonstrated that patients in the former category were 1.95× likelier than their late-onset counterparts to achieve clinical remission (odds ratio [OR] 1.95; 95% confidence interval [CI] 1.39–2.73; I2 = 0%). No difference was seen in improvement or remission + improvement rates between these two groups. When comparing thymectomy with conservative treatments in late-onset NTMG patients, neither did we observe any difference in CSR/PR. Conclusion We found that late-onset NTMG patients had a lower chance of achieving CSR after thymectomy than early-onset patients. Thymectomy in late-onset NTMG also yielded no benefit to CSR or PR compared with conservative treatments. In late-onset NTMG patients, thymectomy should therefore be performed with caution, and the appropriate cutoff between early- and late-onset MG should be further explored in order to tailor and execute the proper therapeutic strategies.

Background/aim Adaptive radiation therapy (ART) is a technique capable of reducing radiation dose to normal tissue without compromising local control. For potentially resectable thymoma, induction therapy is standard of care. Because large disease volume is common in this context, ART has been suggested to reduce toxicity from induction chemoradiation. This has not been previously illustrated in the literature. Case report A 38-year-old man with initially unresectable thymoma was treated with induction chemoradiation including cisplatin and etoposide. He received 45 Gy in 25 fractions and ART was utilized to shrink the radiotherapy field for the final 10 fractions. Results Thymectomy showed Masaoka stage III disease with negative margins. He experienced no treatment-related toxicity and has no evidence of disease 8 years after diagnosis. Conclusion Induction chemoradiotherapy with ART appears to be feasible, safe, and efficacious for locally advanced intact thymoma.

A 19-year-old patient presented for syncope with third-degree AV block (TDAVB) at ECG. A chest-CT showed a thymic mass that could be responsible for TDAVB due to extrinsic vagal nerve compression. Thymectomy led to complete AV block resolution. An extrinsic vagal compression mechanism should be considered among causes of complete atrioventricular block.

Introduction. Micronodular thymoma with lymphoid stroma (MNTLS) is a rare thymoma subtype characterized by distinctive histological appearance, unique clinical profile, and indolent course with good prognosis. In addition to its distinctive morphology, MNTLS may be associated with diverse histological features, such as thymic cysts and conventional thymoma, complicating the diagnostic picture further. Materials and methods. We report herein an account of 3 elderly patients (male—02; female—01), who presented with anterior mediastinal mass, and underwent robotic thymectomy. Microscopic examination revealed MNTLS in all 3 cases. In addition, 2 cases showed associated histological features in the form of a multilocular thymic cyst and conventional (type B2) thymoma, respectively. All 3 patients are doing well without recurrence or metastasis at 34, 28, and 19 months postsurgery. Conclusions. Awareness of this rare thymoma subtype is vital among pathologists to avoid misdiagnosis and ensure appropriate patient management. To date, only a few cases of this rare thymoma subtype have been reported in the literature, mostly as single case reports.