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1.
Autoimmunity ; 57(1): 2347379, 2024 Apr 27.
Article in English | MEDLINE | ID: mdl-38723105

ABSTRACT

Thymoma is closely associated with myasthenia gravis (MG). However, due to the heterogeneity of thymoma and the intricate pathogenesis of MG, it remains unclear why some patients with thymoma develop MG and others do not. In this study, we conducted a comparative phenotype analysis of thymocytes in type B thymomas in patients with MG (MG (+) thymomas) and without MG (MG (-) thymomas) via fluorescence-activated cell sorting (FACS). Our results show that the developmental stages defined by the expression of CD3, CD4, and CD8 were largely maintained in both MG (+) and MG (-) thymomas, with CD4+CD8+ cells constituting the majority of thymocytes in type B thymoma, and no significant difference between this cell population was observed in MG (+) and MG (-) thymomas.We discovered that CD4+CD8+ thymocytes in MG (+) thymomas expressed low levels of αß TCR and high levels of IL-7 receptor α (IL-7Rα), whereas in MG (-) thymomas, CD4+CD8+ thymocytes exhibited the opposite pattern of αß TCR and IL-7Rα expression. These results suggest that the positive and negative selection processes of CD4+CD8+ thymocytes might differ between MG (+) thymomas and MG (-) thymomas. The expression of the Helios transcription factor is induced during negative selection and marks a group of T cells that have undergone negative selection and are likely to be deleted due to strong TCR binding with self-peptides/MHC ligands. We observed that the percentage of Helios-positive CD4SP T cells was greater in MG (-) than in MG (+) thymomas. Thus, the differentially regulated selection process of CD4+CD8+ thymocytes, which involves TCR and IL-7/IL-7Rα signaling, is associated with the presence of MG in type B thymomas.


Subject(s)
Myasthenia Gravis , Receptors, Antigen, T-Cell, alpha-beta , Thymocytes , Thymoma , Humans , Thymoma/immunology , Thymoma/pathology , Thymoma/metabolism , Myasthenia Gravis/immunology , Myasthenia Gravis/pathology , Myasthenia Gravis/metabolism , Receptors, Antigen, T-Cell, alpha-beta/metabolism , Male , Thymocytes/immunology , Thymocytes/metabolism , Female , Middle Aged , Receptors, Interleukin-7/metabolism , Receptors, Interleukin-7/immunology , Adult , Aged , Thymus Neoplasms/immunology , Thymus Neoplasms/pathology , Thymus Neoplasms/metabolism , CD4-Positive T-Lymphocytes/immunology , CD4-Positive T-Lymphocytes/metabolism , CD8-Positive T-Lymphocytes/immunology , CD8-Positive T-Lymphocytes/metabolism , Immunophenotyping
2.
Kyobu Geka ; 77(5): 394-398, 2024 May.
Article in Japanese | MEDLINE | ID: mdl-38720610

ABSTRACT

A 73-year-old woman presented with left anterior chest pain and back pain. Computed tomography (CT) scan showed an anterior mediastinal tumor. It also showed partial anomalous pulmonary venous drainage (left superior pulmonary vein draining into the left brachiocephalic vein), and the tumor was located near the left brachiocephalic vein. The operation was performed through a median sternotomy to resect the thymus and tumor with partial resection of the left upper lobe due to the tumor's adhesion to the left upper lobe. One of the vascular anomalies encountered in adult thoracic surgery is partial anomalous pulmonary venous drainage. It is important to recognize the presence of such an anomaly on imaging and to anticipate the surgical procedure with a preoperative surgical technique.


Subject(s)
Pulmonary Veins , Thymoma , Thymus Neoplasms , Tomography, X-Ray Computed , Humans , Female , Aged , Pulmonary Veins/abnormalities , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/surgery , Thymoma/surgery , Thymoma/diagnostic imaging , Thymoma/complications , Thymus Neoplasms/surgery , Thymus Neoplasms/diagnostic imaging , Thymus Neoplasms/complications
3.
BMC Cancer ; 24(1): 651, 2024 May 28.
Article in English | MEDLINE | ID: mdl-38807039

ABSTRACT

OBJECTIVES: This study aims to develop an innovative, deep model for thymoma risk stratification using preoperative CT images. Current algorithms predominantly focus on radiomic features or 2D deep features and require manual tumor segmentation by radiologists, limiting their practical applicability. METHODS: The deep model was trained and tested on a dataset comprising CT images from 147 patients (82 female; mean age, 54 years ± 10) who underwent surgical resection and received subsequent pathological confirmation. The eligible participants were divided into a training cohort (117 patients) and a testing cohort (30 patients) based on the CT scan time. The model consists of two stages: 3D tumor segmentation and risk stratification. The radiomic model and deep model (2D) were constructed for comparative analysis. Model performance was evaluated through dice coefficient, area under the curve (AUC), and accuracy. RESULTS: In both the training and testing cohorts, the deep model demonstrated better performance in differentiating thymoma risk, boasting AUCs of 0.998 and 0.893 respectively. This was compared to the radiomic model (AUCs of 0.773 and 0.769) and deep model (2D) (AUCs of 0.981 and 0.760). Notably, the deep model was capable of simultaneously identifying lesions, segmenting the region of interest (ROI), and differentiating the risk of thymoma on arterial phase CT images. Its diagnostic prowess outperformed that of the baseline model. CONCLUSIONS: The deep model has the potential to serve as an innovative decision-making tool, assisting on clinical prognosis evaluation and the discernment of suitable treatments for different thymoma pathological subtypes. KEY POINTS: • This study incorporated both tumor segmentation and risk stratification. • The deep model, using clinical and 3D deep features, effectively predicted thymoma risk. • The deep model improved AUCs by 16.1pt and 17.5pt compared to radiomic model and deep model (2D) respectively.


Subject(s)
Deep Learning , Thymoma , Thymus Neoplasms , Tomography, X-Ray Computed , Humans , Female , Thymoma/diagnostic imaging , Thymoma/pathology , Middle Aged , Male , Tomography, X-Ray Computed/methods , Risk Assessment/methods , Thymus Neoplasms/pathology , Thymus Neoplasms/diagnostic imaging , Adult , Aged , Retrospective Studies
4.
Neurology ; 102(12): e209482, 2024 Jun 25.
Article in English | MEDLINE | ID: mdl-38781559

ABSTRACT

The role of immunosenescence, particularly the natural process of thymic involution during aging, is increasingly acknowledged as a factor contributing to the development of autoimmune diseases and cancer. Recently, a concern has been raised about deleterious consequences of the surgical removal of thymic tissue, including for patients who undergo thymectomy for myasthenia gravis (MG) or resection of a thymoma. This review adopts a multidisciplinary approach to scrutinize the evidence concerning the long-term risks of cancer and autoimmunity postthymectomy. We conclude that for patients with acetylcholine receptor antibody-positive MG and those diagnosed with thymoma, the removal of the thymus offers prominent benefits that well outweigh the potential risks. However, incidental removal of thymic tissue during other thoracic surgeries should be minimized whenever feasible.


Subject(s)
Myasthenia Gravis , Thymectomy , Thymoma , Thymus Gland , Thymus Neoplasms , Humans , Thymectomy/adverse effects , Thymectomy/methods , Myasthenia Gravis/surgery , Thymus Gland/surgery , Thymus Neoplasms/surgery , Thymus Neoplasms/complications , Thymoma/surgery , Thymoma/complications , Postoperative Complications/etiology , Autoimmune Diseases/surgery
5.
BMC Cardiovasc Disord ; 24(1): 269, 2024 May 22.
Article in English | MEDLINE | ID: mdl-38778265

ABSTRACT

INTRODUCTION: Surgery remains the primary treatment modality for thymic carcinoma, with adjuvant radiotherapy being recommended to effectively mitigate local recurrence and metastasis rates subsequent to incomplete or complete resection. Chemoradiotherapy has the potential to induce coronary artery occlusion, thereby potentially impacting patients' long-term survival rates. The existing literature currently lacks comprehensive research on the lesion characteristics of coronary artery injury resulting from chemoradiotherapy. CASE PRESENTATION: The male patient, aged 55, was admitted to the hospital due to recurrent chest tightness and pain persisting for one week. Notably, the patient had previously undergone curative resection surgery for thymic carcinoma seven years ago. After the surgical procedure, the patient underwent a course of adjuvant chemotherapy comprising docetaxel and platinum. 11 months later, imaging examination diagnosed tumor recurrence, and concurrent chemoradiotherapy was administered at a total dose of 62 Gy/31F for planning gross target volume (PGTV) and 54 Gy/31F for planning target volume (PTV) with 2 cycles of paclitaxel and cisplatin. Re-admission of the patient occurred after a 7-year interval subsequent to the completion of concurrent chemoradiotherapy, leading to a subsequent diagnosis of acute non-ST segment elevation myocardial infarction. Following administration of antiplatelet, anticoagulant, and anti-myocardial ischemia therapy, coronary angiography revealed the presence of a bifurcation lesion at the distal end of the left main trunk. Intravascular ultrasound (IVUS) examination demonstrated significant negative remodeling of both the main trunk and its branches at the bifurcation site, characterized by minimal atherosclerotic plaque components. CONCLUSIONS: Chemoradiotherapy may induce damage to endothelial cells, resulting in an inflammatory response. Negative remodeling of blood vessels is likely to occur, primarily characterized by vasoconstriction but with less atherosclerotic plaque burden. Routine stent implantation in negatively remodeled areas may lead to vascular rupture, necessitating intravascular imaging examination.


Subject(s)
Thymoma , Thymus Neoplasms , Humans , Male , Thymus Neoplasms/therapy , Thymus Neoplasms/diagnostic imaging , Middle Aged , Treatment Outcome , Time Factors , Thymoma/therapy , Thymoma/diagnostic imaging , Coronary Angiography , Vascular System Injuries/etiology , Vascular System Injuries/diagnostic imaging , Vascular System Injuries/therapy , Coronary Vessels/diagnostic imaging , Coronary Vessels/injuries , Coronary Vessels/drug effects , Chemoradiotherapy/adverse effects
6.
Int J Rheum Dis ; 27(5): e15187, 2024 May.
Article in English | MEDLINE | ID: mdl-38742741

ABSTRACT

Thymic tumors are very rare neoplasms in children and account for less than 1% of mediastinal tumors in pediatric patients. One-third of the pediatric patients present with symptoms related to the compression of the tumor mass on the surrounding anatomic structures, and paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, acquired hypogammaglobulinemia, and connective tissue disorders, which rarely occur in children with thymic tumors. Herein, we report a case of thymic carcinoma mimicking the symptoms of a connective tissue disease with symmetrical polyarthritis accompanying myositis, fever, weight loss, and malaise in a 15-year-old male patient. To our knowledge, this is the first case pediatric thymic carcinoma accompany with severe polyarthritis and myopathy, thus we have reviewed the current literature regarding the cases of thymic malignancies coexisting with paraneoplastic syndromes in children.


Subject(s)
Arthritis , Myositis , Paraneoplastic Syndromes , Thymoma , Thymus Neoplasms , Humans , Male , Myositis/diagnosis , Myositis/complications , Paraneoplastic Syndromes/diagnosis , Paraneoplastic Syndromes/etiology , Thymus Neoplasms/complications , Thymus Neoplasms/diagnosis , Adolescent , Arthritis/diagnosis , Arthritis/etiology , Thymoma/complications , Thymoma/diagnosis , Treatment Outcome , Thymectomy , Biopsy
7.
Clin Immunol ; 263: 110233, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38697554

ABSTRACT

Ataxia-telangiectasia (A-T) is a rare disorder caused by genetic defects of A-T mutated (ATM) kinase, a key regulator of stress response, and characterized by neurodegeneration, immunodeficiency, and high incidence of cancer. Here we investigated NK cells in a mouse model of A-T (Atm-/-) showing that they are strongly impaired at killing tumor cells due to a block of early signaling events. On the other hand, in Atm-/- littermates with thymic lymphoma NK cell cytotoxicity is enhanced as compared with ATM-proficient mice, possibly via tumor-produced TNF-α. Results also suggest that expansion of exhausted NKG2D+ NK cells in Atm-/- mice is driven by low-level expression of stress-inducible NKG2D ligands, whereas development of thymoma expressing the high-affinity MULT1 ligand is associated with NKG2D down-regulation on NK cells. These results expand our understanding of immunodeficiency in A-T and encourage exploring NK cell biology in A-T patients in the attempt to identify cancer predictive biomarkers and novel therapeutic targets.


Subject(s)
Ataxia Telangiectasia Mutated Proteins , Killer Cells, Natural , NK Cell Lectin-Like Receptor Subfamily K , Animals , Killer Cells, Natural/immunology , Ataxia Telangiectasia Mutated Proteins/genetics , Ataxia Telangiectasia Mutated Proteins/metabolism , NK Cell Lectin-Like Receptor Subfamily K/genetics , NK Cell Lectin-Like Receptor Subfamily K/metabolism , Mice , Ataxia Telangiectasia/genetics , Ataxia Telangiectasia/immunology , Mice, Knockout , Mice, Inbred C57BL , Thymoma/immunology , Thymoma/genetics , Tumor Necrosis Factor-alpha/metabolism , Tumor Necrosis Factor-alpha/immunology , Cytotoxicity, Immunologic , Thymus Neoplasms/immunology , Thymus Neoplasms/genetics , Signal Transduction , Membrane Proteins , Histocompatibility Antigens Class I
8.
J Neuroinflammation ; 21(1): 126, 2024 May 11.
Article in English | MEDLINE | ID: mdl-38734662

ABSTRACT

Myasthenia gravis (MG) is an immune-mediated disease frequently associated with thymic changes. Increased T helper 17 (Th17) cell activity and dysfunctional regulatory T (Treg) cells have been demonstrated in subgroups of MG. On the other hand, hypoxia-inducible factor 1 (HIF-1) has been shown to regulate the Th17/Treg balance by inducing Th17 differentiation while attenuating Treg development. To identify the underlying mechanisms of different thymic pathologies in MG development, we evaluated thymic samples from thymoma-associated myasthenia gravis (TAMG), MG with hyperplasia (TFH-MG) and thymoma without MG (TOMA) patients. Differential gene expression analysis revealed that TAMG and TFH-MG cells are associated with different functional pathways. A higher RORC/FOXP3 ratio provided evidence for Th17/Treg imbalance in TAMG potentially related to increased HIF1A. The hypoxic microenvironment in thymoma may be a driver of TAMG by increasing HIF1A. These findings may lead to new therapeutic approaches targeting HIF1A in the development of TAMG.


Subject(s)
Hypoxia-Inducible Factor 1, alpha Subunit , Myasthenia Gravis , T-Lymphocytes, Regulatory , Th17 Cells , Thymoma , Thymus Gland , Thymus Neoplasms , Myasthenia Gravis/genetics , Myasthenia Gravis/immunology , Myasthenia Gravis/pathology , Thymoma/complications , Thymoma/genetics , Thymoma/immunology , Humans , Hypoxia-Inducible Factor 1, alpha Subunit/genetics , Hypoxia-Inducible Factor 1, alpha Subunit/metabolism , T-Lymphocytes, Regulatory/metabolism , T-Lymphocytes, Regulatory/immunology , Th17 Cells/metabolism , Th17 Cells/immunology , Thymus Gland/pathology , Male , Female , Thymus Neoplasms/complications , Thymus Neoplasms/genetics , Adult , Middle Aged , Aged
9.
Medicine (Baltimore) ; 103(19): e37956, 2024 May 10.
Article in English | MEDLINE | ID: mdl-38728510

ABSTRACT

This study, based on a population, explored the prognostic value of postoperative radiotherapy (PORT) for Masaoka-Koga IIB stage thymomas. Patients diagnosed with thymoma from 2004 to 2017 in the Surveillance, Epidemiology, and End Results (SEER) database were included in the retrospective study. Through propensity score matching, the baseline characteristics of the patients were successfully matched to mitigate the selection bias of PORT. Survival rates and survival curves were compared between the PORT and non-PORT groups, with potential confounding factors addressed using a multivariate Cox regression model. In this study, 785 cases of IIB stage thymoma were included from the SEER database, and 303 patients were successfully matched between PORT and non-PORT groups through propensity score matching, with no significant differences in baseline characteristics. In the PORT and non-PORT groups, 10-year overall survival rates were 65.2% versus 59.6%, and cancer-specific survival rates were 87.0% vs. 84.4%, PORT did not yield statistically significant improvements in overall survival (P = .275) or cancer-specific survival (P = .336) for stage IIB thymomas. Based on the SEER database, the results of our study indicated that PORT does not confer a significant survival benefit for IIB stage thymomas.


Subject(s)
Neoplasm Staging , Propensity Score , SEER Program , Thymoma , Thymus Neoplasms , Humans , Thymoma/radiotherapy , Thymoma/mortality , Thymoma/surgery , Thymoma/pathology , Female , Male , Middle Aged , Retrospective Studies , Thymus Neoplasms/radiotherapy , Thymus Neoplasms/mortality , Thymus Neoplasms/pathology , Thymus Neoplasms/surgery , Aged , Adult , Radiotherapy, Adjuvant , Survival Rate , Prognosis
10.
BMC Neurol ; 24(1): 173, 2024 May 23.
Article in English | MEDLINE | ID: mdl-38783232

ABSTRACT

BACKGROUND: Ectopic cervical thymoma (ECT) is an extremely rare tumor, especially in association with myasthenia gravis (MG). CASE PRESENTATION: We report a case of myasthenia gravis with an ectopic thymoma in the neck, whose myasthenic symptoms significantly improved after complete removal of the mass. A 55-year-old woman with generalized myasthenia gravis (MG) experienced worsening neuromuscular weakness after abruptly discontinuing pyridostigmine. Testing revealed acetylcholine receptor-antibody (AChR-Ab) positivity and a cervical mass initially thought to be thyroid or parathyroid was identified as a thymoma, type A. Post-surgery and radiation therapy, her myasthenic symptoms improved significantly with less prednisone and pyridostigmine requirements over time and no need for additional immunotherapies. CONCLUSIONS: Diagnosing ECTs is challenging due to rarity, atypical locations, and inconclusive fine needle aspiration cytology (FNAC) results, often misinterpreted as thyroid or parathyroid lesions. As proper management of patients with MG, including thymectomy, offers favorable clinical outcomes such as significant improvement in myasthenic complaints and reduced immunosuppressive medication requirements, clinicians should be vigilant of the ectopic locations of thymomas to ensure timely diagnosis and intervention.


Subject(s)
Myasthenia Gravis , Thymoma , Humans , Female , Myasthenia Gravis/complications , Myasthenia Gravis/diagnosis , Middle Aged , Thymoma/complications , Thymoma/diagnosis , Thymus Neoplasms/complications , Thymus Neoplasms/diagnosis , Choristoma/complications , Choristoma/pathology
11.
Rozhl Chir ; 103(2): 40-47, 2024.
Article in English | MEDLINE | ID: mdl-38697812

ABSTRACT

Thoracic surgery is increasingly influenced by the development of minimally invasive approaches which have also influenced surgery in the area of the anterior mediastinum. The previously standard approach to the thymus via partial sternotomy was gradually replaced by the videothoracoscopic approach in most cases. In recent years, robotically assisted surgery has been gaining ground worldwide in this area, as well. The aim of our paper is to provide a comprehensive overview of procedures in the field of the thymus, including their indications, and to share our first experience with robot-assisted thymus surgery. At the 3rd Department of Surgery, since the start of the robot-assisted thymus surgery program, 23 thymectomies have been performed using this approach, of which 17 were performed for thymoma, 3 for myasthenia gravis, and 3 for parathyroid adenoma localized in thymus tissue. From our experience and the available data, it follows that the length of hospitalization, the rate of complications and the resulting effect of robot-assisted procedures is comparable to VTS procedures; however, the robot-assisted surgery also allows for mini-invasive treatment even in significantly obese patients and in patients with advanced thymic tumors who would otherwise be indicated for open thymectomy.


Subject(s)
Myasthenia Gravis , Robotic Surgical Procedures , Thymectomy , Thymoma , Thymus Neoplasms , Humans , Robotic Surgical Procedures/methods , Thymectomy/methods , Thymus Neoplasms/surgery , Thymoma/surgery , Myasthenia Gravis/surgery , Parathyroid Neoplasms/surgery , Thymus Gland/surgery , Male
12.
Front Immunol ; 15: 1288045, 2024.
Article in English | MEDLINE | ID: mdl-38629065

ABSTRACT

Thymic epithelial tumors (TETs) are rare mediastinal cancers originating from the thymus, classified in two main histotypes: thymoma and thymic carcinoma (TC). TETs affect a primary lymphoid organ playing a critical role in keeping T-cell homeostasis and ensuring an adequate immunological tolerance against "self". In particular, thymomas and not TC are frequently associated with autoimmune diseases (ADs), with Myasthenia Gravis being the most common AD present in 30% of patients with thymoma. This comorbidity, in addition to negatively affecting the quality and duration of patients' life, reduces the spectrum of the available therapeutic options. Indeed, the presence of autoimmunity represents an exclusion criteria for the administration of the newest immunotherapeutic treatments with checkpoint inhibitors. The pathophysiological correlation between TETs and autoimmunity remains a mystery. Several studies have demonstrated the presence of a residual and active thymopoiesis in adult patients affected by thymomas, especially in mixed and lymphocytic-rich thymomas, currently known as type AB and B thymomas. The aim of this review is to provide the state of art in regard to the histological features of the different TET histotype, to the role of the different immune cells infiltrating tumor microenvironments and their impact in the break of central immunologic thymic tolerance in thymomas. We discuss here both cellular and molecular immunologic mechanisms inducing the onset of autoimmunity in TETs, limiting the portfolio of therapeutic strategies against TETs and greatly impacting the prognosis of associated autoimmune diseases.


Subject(s)
Myasthenia Gravis , Neoplasms, Glandular and Epithelial , Thymoma , Thymus Neoplasms , Adult , Humans , Autoimmunity , Thymus Neoplasms/complications , Neoplasms, Glandular and Epithelial/therapy , Neoplasms, Glandular and Epithelial/complications , Tumor Microenvironment
13.
J Cardiothorac Surg ; 19(1): 225, 2024 Apr 16.
Article in English | MEDLINE | ID: mdl-38627811

ABSTRACT

BACKGROUND: The purpose of this study was to evaluate the clinicopathological characteristics of patients who underwent surgical resection for thymic neuroendocrine tumors (TNET) or thymic carcinoma. METHODS: In this study, we retrospectively evaluated the clinicopathological characteristics of our surgical patients at Fukuoka University Hospital from January 1995 to December 2018. RESULTS: There were nine cases of TNET and 16 cases of thymic carcinoma. Regarding the pathological type, the TNET group included three atypical carcinoid cases, two large cell neuroendocrine tumor cases, two small cell carcinoma cases, and two other cases. The thymic carcinoma group included 15 squamous carcinoma cases and one case of adenosquamous carcinoma. Based on the Masaoka-Koga staging system, six TNET cases and 11 thymic carcinoma cases were stage III or IV. The complete resection rate was 77% in the TNET group and 81% in the thymic carcinoma group. Additional chemotherapy and/or radiotherapy was performed in five cases of TNET and 11 cases of thymic carcinoma. The five-year survival rate and five-year disease-free survival rate were 87.5% and 75.0% in the TNET group and 58.9% and 57.1% in the thymic carcinoma group, respectively, with no significant difference between the two groups (P = 0.248 and P = 0.894, respectively). In the univariate analysis, complete resection was a statistically significant prognostic factor (P = 0.017). CONCLUSION: In this study, no difference in prognosis was observed between TNET and thymic carcinomas. To understand the characteristics of these tumors, further case accumulation and multicenter clinical studies are needed. (243words).


Subject(s)
Lung Neoplasms , Neuroendocrine Tumors , Thymoma , Thymus Neoplasms , Humans , Lung Neoplasms/pathology , Neoplasm Staging , Neuroendocrine Tumors/surgery , Neuroendocrine Tumors/pathology , Prognosis , Retrospective Studies , Thymoma/pathology , Thymus Neoplasms/pathology
14.
Radiother Oncol ; 195: 110272, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38614283

ABSTRACT

PURPOSE: To assess the prognostic factors and patterns of failure of patients consecutively treated with surgery and postoperative radiation therapy (PORT) for thymic epithelial tumours (TET). PATIENTS AND METHODS: Data from 192 TET patients who were operated and received PORT at a single centre from 1990 to 2019 was retrospectively analysed. RESULTS: Most patients had thymoma (77 %, B247%), were classified Masaoka-Koga stage III (35 %) or IV (32 %) and had a R0 (75 %) resection. Radiotherapy was delivered at a median dose of 50.4 Gy (range, 42-66 Gy; ≥ 60 Gy in 17 %), 63 (33 %) patients were treated by intensity-modulated radiation therapy and elective nodal radiotherapy was used for 37 %. At a median follow-up of 10.9 years, the 10-year overall survival (OS) and progression-free survival (PFS) rates were 62 % (95 % CI: 54-70 %) and 47 % (95 % CI: 39-55 %), respectively. Locoregional recurrence (LRR) occurred in 72/192 (38 %) patients, distributed as 6 local, 45 regional and 21 both local and regional. LRR were mainly located to the pleura: 66/72 (92 %) and 16/72 (22 %; 16/192 in total, 8 %) were in-field. Distant relapse (DR) were observed in 30 patients (16 %), resulting in 10-year locoregional (LRC) and distant control rates of 58 % (95 % CI: 50-66 %) and 82 % (95 % CI: 77-88 %), respectively. In the multivariate analysis, Masaoka-Koga stage (HR [hazard ratio]: 1.9; p = 0.001), thymic carcinomas/neuroendocrine tumours (TC) (HR: 1.6; p = 0.045) and ECOG PS > 1 (HR: 1.9; p = 0.02) correlated with poorer OS. Higher Masaoka-Koga stage (HR: 2.6; p < 0.001) associated with a decreased LRC but not R1 status (HR: 1.2; p = 0.5) or WHO histology classification. TC (HR: 3.4; p < 0.001) and a younger age (HR: 2.5; p = 0.02) correlated with DR. CONCLUSION: Approximately one-third of the TET in our study experienced a LRR, mainly to the pleura, and 8% in total were in-field. The place of radiotherapy should be better defined in higher risk thymoma patients within prospective randomized studies.


Subject(s)
Neoplasms, Glandular and Epithelial , Thymus Neoplasms , Humans , Thymus Neoplasms/radiotherapy , Thymus Neoplasms/pathology , Thymus Neoplasms/mortality , Thymus Neoplasms/surgery , Male , Female , Middle Aged , Aged , Adult , Retrospective Studies , Follow-Up Studies , Neoplasms, Glandular and Epithelial/radiotherapy , Neoplasms, Glandular and Epithelial/pathology , Neoplasms, Glandular and Epithelial/mortality , Neoplasms, Glandular and Epithelial/surgery , Aged, 80 and over , Young Adult , Neoplasm Recurrence, Local , Radiotherapy, Adjuvant , Radiotherapy, Intensity-Modulated/methods , Adolescent , Thymoma/radiotherapy , Thymoma/pathology , Thymoma/mortality , Prognosis , Survival Rate
15.
J Cardiothorac Surg ; 19(1): 198, 2024 Apr 10.
Article in English | MEDLINE | ID: mdl-38600577

ABSTRACT

This case report details a rare thymic basaloid carcinoma initially misinterpreted as a mediastinal teratoma, underscoring the diagnostic challenges posed by such tumors. A 71-year-old female presented with an asymptomatic anterior mediastinal tumor discovered incidentally during a routine health examination. Surgical intervention, followed by pathological and immunohistochemical analysis including CK-pan, p63, p40, and CD117 molecules, led to a definitive diagnosis of basaloid carcinoma of the thymus. This case highlights the critical importance of differential diagnosis in mediastinal lesions, especially those presenting with multilocular thymic cysts on chest CT. The subxiphoid video-assisted thoracoscopic surgery enabled complete tumor resection with minimal trauma and favorable postoperative outcomes. The patient opted against further radiotherapy or chemotherapy and she has survived for over eight months without recurrence. This case report contributes to the growing understanding of thymic basaloid carcinoma, a rare and potentially aggressive thymic carcinoma subtype. It emphasizes the necessity for precise surgical techniques and enhanced diagnostic acumen among cardiothoracic surgeons and oncologists.


Subject(s)
Carcinoma, Squamous Cell , Mediastinal Cyst , Mediastinal Neoplasms , Teratoma , Thymoma , Thymus Neoplasms , Female , Humans , Aged , Mediastinal Neoplasms/diagnosis , Thymus Neoplasms/diagnosis , Thymus Neoplasms/surgery , Thymus Neoplasms/pathology , Mediastinal Cyst/diagnosis , Mediastinal Cyst/surgery , Mediastinal Cyst/pathology , Thymoma/pathology , Teratoma/diagnosis
17.
Am Surg ; 90(6): 1561-1569, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38584508

ABSTRACT

BACKGROUND: Current practice patterns suggest open rather than minimally invasive (MIS) approaches for thymomas >4 cm. We hypothesized there would be similar perioperative outcomes and overall survival between open and MIS approaches for large (>4 cm) thymoma resection. METHODS: The National Cancer Database was queried for patients who underwent thymectomy from 2010 to 2020. Surgical approach was characterized as either open or MIS. The primary outcome was overall survival and secondary outcomes were margin status, and length of stay (LOS). Differences between approach cohorts were compared after a 1:1 propensity match. RESULTS: Among 4121 thymectomies, 2474 (60%) were open and 1647 (40%) were MIS. Patients undergoing MIS were older, had fewer comorbidities, and had smaller tumors (median; 4.6 vs 6 cm, P < .001). In the unmatched cohort, MIS and open had similar 90-day mortality (1.1% vs 1.8%, P = .158) and rate of positive margin (25.1% vs 27.9%, P = .109). MIS thymectomy was associated with shorter LOS (2 (1-4) vs 4 (3-6) days, P < .001). Propensity matching reduced the bias between the groups. In this cohort, overall survival was similar between the groups by log-rank test (P = .462) and multivariate cox hazard analysis (HR .882, P = .472). Multivariable regression showed shorter LOS with MIS approach (Coef -1.139, P < .001), and similar odds of positive margin (OR 1.130, P = .150). DISCUSSION: MIS has equivalent oncologic benefit to open resection for large thymomas, but is associated with shorter LOS. When clinically appropriate, MIS thymectomy may be considered a safe alternative to open resection for large thymomas.


Subject(s)
Thymectomy , Thymoma , Thymus Neoplasms , Humans , Thymoma/surgery , Thymoma/mortality , Male , Female , Middle Aged , Thymectomy/methods , Thymus Neoplasms/surgery , Thymus Neoplasms/mortality , Thymus Neoplasms/pathology , Aged , Minimally Invasive Surgical Procedures/methods , Length of Stay/statistics & numerical data , Propensity Score , Retrospective Studies , Adult , Margins of Excision , Treatment Outcome
18.
Clin Radiol ; 79(6): 404-412, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38565483

ABSTRACT

Due to the rising demand in cross-sectional thoracic imaging, anterior mediastinal lesions are being identified with increasing frequency. Following iterative and multidisciplinary discussions, the BTOG Thymic Malignancies Special Interest Group have developed an algorithm to standardise the diagnostic approach for these relatively uncommon but important conditions which span from benign (thymic remnant, thymic hyperplasia and thymic cysts) to suspected localised thymomas to suspected more aggressive malignancy (thymic carcinoma, lymphoma and germ cell tumours). For each condition, we provide a brief description, an overview of the key radiological findings and a description of the proposed algorithm including the rationale behind the recommendations. We also highlight the role of magnetic resonance (MR) imaging for the characterisation of anterior mediastinal masses in specific indications when the necessary local resources and expertise exist. In addition, we hope this provides the rationale for service development in MR of the anterior mediastinum where current resource and expertise requires development. Through this standardised pathway, we hope to drive improvements in patient care by rationalising surveillance schedules, avoiding unnecessary resections of benign entities with their associated morbidity and optimising the diagnostic work-up prior to the appropriate treatment of anterior mediastinal malignancies.


Subject(s)
Algorithms , Magnetic Resonance Imaging , Mediastinal Neoplasms , Thymus Neoplasms , Humans , Mediastinal Neoplasms/diagnostic imaging , Thymus Neoplasms/diagnostic imaging , Magnetic Resonance Imaging/methods , Mediastinum/diagnostic imaging , Diagnosis, Differential , Thymoma/diagnostic imaging
19.
Lung Cancer ; 191: 107557, 2024 May.
Article in English | MEDLINE | ID: mdl-38626709

ABSTRACT

OBJECTIVES: The main objective of this report was to detail the long-term follow-up data from the REMORA study, which investigated the safety and efficacy of lenvatinib in patients with thymic carcinoma. In addition, an exploratory analysis of the association between relative dose intensity (RDI) and the efficacy of lenvatinib is presented. MATERIALS AND METHODS: The single-arm, open-label, phase 2 REMORA study was conducted at eight Japanese institutions. Forty-two patients received oral lenvatinib 24 mg once daily in 4-week cycles until the occurrence of intolerable adverse events or disease progression. The REMORA long-term follow-up data were evaluated, including overall survival (OS). RDI was calculated by dividing the actual dose administered to the patient by the standard recommended dose. This trial is registered on JMACCT (JMA-IIA00285) and on UMIN-CTR (UMIN000026777). RESULTS: The updated median OS was 28.3 months (95 % confidence interval [CI]: 17.1-34.0 months), and the OS rate at 36 months was 35.7 % (95 % CI: 21.7 %-49.9 %). When grouped by RDI of lenvatinib, the median OS was 38.5 months (95 % CI: 31.2-not estimable) in patients with ≥ 75 % RDI and 17.3 months (95 % CI: 13.4-26.2 months) in patients with < 75 % RDI (hazard ratio 0.46 [95 % CI: 0.22-0.98]; P = 0.0406) at 8 weeks. Patients who maintained their lenvatinib dose over 8 weeks had a higher objective response rate than patients whose doses were reduced (75.0 % vs 29.4 %; P = 0.0379). No new safety concerns or treatment-related deaths were reported, and lenvatinib had a tolerable safety profile. CONCLUSION: This follow-up report updated OS in patients with metastatic or recurrent thymic carcinoma. A higher RDI of lenvatinib at 8 weeks could be associated with improved outcomes.


Subject(s)
Neoplasm Recurrence, Local , Phenylurea Compounds , Quinolines , Thymoma , Humans , Phenylurea Compounds/therapeutic use , Phenylurea Compounds/administration & dosage , Phenylurea Compounds/adverse effects , Quinolines/therapeutic use , Quinolines/adverse effects , Quinolines/administration & dosage , Male , Female , Middle Aged , Aged , Follow-Up Studies , Neoplasm Recurrence, Local/drug therapy , Neoplasm Recurrence, Local/pathology , Thymoma/drug therapy , Thymoma/mortality , Thymoma/pathology , Adult , Antineoplastic Agents/therapeutic use , Antineoplastic Agents/adverse effects , Antineoplastic Agents/administration & dosage , Thymus Neoplasms/drug therapy , Thymus Neoplasms/pathology , Thymus Neoplasms/mortality , Neoplasm Metastasis , Aged, 80 and over , Treatment Outcome
20.
Eur J Surg Oncol ; 50(6): 108349, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38640605

ABSTRACT

BACKGROUD: The standard resection for early-stage thymoma is total thymectomy and complete tumour excision with or without myasthenia gravis but the optimal surgery mode for patients with early-stage non-myasthenic thymoma is debatable. This study analysed the oncological outcomes for non-myasthenic patients with early-stage thymoma treated by thymectomy or limited resection in the long term. METHODS: Patients who had resections of thymic neoplasms at Taipei Veteran General Hospital, Taiwan between December 1997 and March 2013 were recruited, exclusive of those combined clinical evidence of myasthenia gravis were reviewed. A total of 113 patients were retrospectively reviewed with pathologic early stage (Masaoka stage I and II) thymoma who underwent limited resection or extended thymectomy to compare their long-term oncologic and surgical outcomes. RESULTS: The median observation time was 134.1 months [interquartile range (IQR) 90.7-176.1 months]. In our cohort, 52 patients underwent extended thymectomy and 61 patients underwent limited resection. Shorter duration of surgery (p < 0.001) and length of stay (p = 0.006) were demonstrated in limited resection group. Six patients experienced thymoma recurrence, two of which had combined myasthenia gravis development after recurrence. There was no significant difference (p = 0.851) in freedom-from-recurrence, with similar 10-year freedom-from-recurrence rates between the limited resection group (96.2 %) and the thymectomy group (93.2 %). Tumour-related survival was also not significantly different between groups (p = 0.726).result CONCLUSION: Patients with early-stage non-myasthenic thymoma who underwent limited resection without complete excision of the thymus achieved similar oncologic outcomes during the long-term follow-up and better peri-operative results compared to those who underwent thymectomy.


Subject(s)
Neoplasm Staging , Thymectomy , Thymoma , Thymus Neoplasms , Humans , Thymectomy/methods , Thymoma/surgery , Thymoma/pathology , Thymoma/complications , Male , Thymus Neoplasms/surgery , Thymus Neoplasms/pathology , Thymus Neoplasms/complications , Female , Middle Aged , Follow-Up Studies , Retrospective Studies , Adult , Aged , Myasthenia Gravis/surgery , Survival Rate , Neoplasm Recurrence, Local , Operative Time , Length of Stay , Taiwan/epidemiology , Treatment Outcome
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