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BMC Nephrology Feb 2024Minimal change nephrotic syndrome (MCNS) can be complicated by thymoma; however, no standard therapy for thymoma-associated MCNS has yet been established. We herein...
BACKGROUND
Minimal change nephrotic syndrome (MCNS) can be complicated by thymoma; however, no standard therapy for thymoma-associated MCNS has yet been established. We herein describe a case of steroid-resistant MCNS associated with thymoma, treated effectively with rituximab.
CASE PRESENTATION
A 71-year-old Japanese man was referred to our department with severe proteinuria (20 g/gCr). Renal biopsy showed minimal change disease and computed tomography revealed an anterior mediastinal mass. Based on these findings, he was diagnosed with thymoma-associated MCNS. He was treated with oral prednisolone (50 mg/day) and cyclosporine, and underwent thymectomy and plasma exchange. However, no improvement in proteinuria was observed. He therefore received intravenous rituximab 500 mg, resulting in a marked decrease in proteinuria from 5328 to 336 mg/day after 1 week.
CONCLUSIONS
This case suggests that rituximab might be an effective therapy in patients with steroid-resistant MCNS associated with thymoma.
Topics: Male; Humans; Aged; Thymoma; Cyclosporine; Nephrosis, Lipoid; Rituximab; Thymectomy; Thymus Neoplasms; Nephrotic Syndrome; Prednisolone; Proteinuria
PubMed: 38336671
DOI: 10.1186/s12882-024-03485-2 -
Mediastinum (Hong Kong, China) 2024Locally invasive thymic neoplasms are challenging clinical scenarios and typically require a multidisciplinary approach. The involvement of major mediastinal veins such... (Review)
Review
Locally invasive thymic neoplasms are challenging clinical scenarios and typically require a multidisciplinary approach. The involvement of major mediastinal veins such as the superior vena cava (SVC) used to be a contraindication to surgery, but with improved surgical technique and outcomes, this paradigm has shifted. In some situations, complex resections and reconstructions may be indicated and required to improve the long-term outcome of these patients. We report two of our cases along with a current review of literature. We also describe the preoperative workup, operative techniques, postoperative management, complications, and outcomes of patients with invasive thymic neoplasms that involve the mediastinal veins. Our first case describes a patient who was diagnosed with a thymoma extending from the diaphragm to the base of the neck that was also encasing major vascular structures including the SVC and left innominate vein. Our second case describes a patient who was also diagnosed with a large anterior mediastinal mass encasing the great veins and invading the chest wall. We describe the management of these patients and then delve deeper into operative techniques including SVC resection and reconstruction. We describe the types of conduits that can be used and complications to be mindful of when clamping the great veins, such as the SVC. Improvements in conduit materials and neoadjuvant and adjuvant therapies over the years have made it more feasible for patients with invasive thymic neoplasms to undergo surgery.
PubMed: 38322190
DOI: 10.21037/med-20-69 -
Nigerian Journal of Clinical Practice Jan 2024Myasthenia gravis (MG) is an antibody-mediated autoimmune disease with the cardinal feature being exertional voluntary skeletal muscle weakness and fatigability. It can... (Review)
Review
Myasthenia gravis (MG) is an antibody-mediated autoimmune disease with the cardinal feature being exertional voluntary skeletal muscle weakness and fatigability. It can be an isolated finding or in association with other autoimmune conditions such as Hashimoto's thyroiditis, Graves' disease, systemic lupus erythematosus (SLE), or rheumatoid arthritis. Thymectomy is recommended for most patients with MG whose symptoms begin before the age of 60 years. Patients with thymoma or thymic hyperplasia do respond to thymectomy compared to those without thymoma or enlarged thymus. Those with enlarged goiter would benefit from thyroidectomy. The management of these patients requires a multidisciplinary approach as performed in a low-resource setting. We are reporting the case of a 24-year-old who presented with MG with toxic goiter and had good control on medication. A computed tomography scan of the chest showed a superior mediastinal mass and a soft tissue scan of the neck was done which showed a diffusely enlarged thyroid gland. She subsequently had thymectomy and subtotal thyroidectomy with a satisfactory outcome. We highlight this case to show that MG with thymoma and goiter could coexist. Reports of such findings are infrequently reported in our environment.
Topics: Female; Humans; Middle Aged; Young Adult; Adult; Thymoma; Myasthenia Gravis; Thymus Neoplasms; Thymectomy; Goiter
PubMed: 38317049
DOI: 10.4103/njcp.njcp_206_23 -
Advanced Science (Weinheim,... Apr 2024This study investigates Bacillus Calmette-Guérin (BCG) as a potential treatment for hepatocellular carcinoma (HCC), a condition often associated with unfavorable...
This study investigates Bacillus Calmette-Guérin (BCG) as a potential treatment for hepatocellular carcinoma (HCC), a condition often associated with unfavorable treatment outcomes. Exploiting BCG's recognized immune-boosting properties, preclinical trials are conducted using HCC mice, with a single subcutaneous dose of BCG administered post-tumor formation. Results indicate that BCG treatment effectively diminishes tumor burden and extends survival in both male and female HCC mice. Positive influences on hepatic fibrosis and metabolism are observed, leading to a reduction in lipid levels. Spatial analysis underscores BCG's tumor-specific effects, inducing the enrichment of metabolic pathways and inhibiting various cancer-related pathways. Furthermore, BCG promotes immune cell infiltration, including CD4+, CD8+ T cells, and M1 macrophages, in both v-akt murine thymoma viral oncogene homolog 1(AKT)/neutoblastoma RAS viral oncogene homolog (RAS) and β-catenin positive HCC models. Interestingly, blocking T cells, trained immunity, and Interferon-γ (IFN-γ) function reverses BCG's anti-HCC effects. In conclusion, BCG emerges as a promising treatment option for HCC, characterized by a favorable safety profile and efficacy in inhibiting fibrosis, improving metabolism, and engaging both trained immunity and T cells in therapeutic mechanisms.
Topics: Male; Mice; Animals; Female; Carcinoma, Hepatocellular; BCG Vaccine; Proto-Oncogene Proteins c-akt; Liver Neoplasms; Mycobacterium bovis
PubMed: 38308164
DOI: 10.1002/advs.202308242 -
Cureus Jan 2024Thymic epithelial tumors (TETs), particularly type B2 thymomas, are rare neoplasms primarily found in the anterior mediastinum. The current therapeutic approach includes...
Thymic epithelial tumors (TETs), particularly type B2 thymomas, are rare neoplasms primarily found in the anterior mediastinum. The current therapeutic approach includes surgery, chemotherapy, and radiotherapy, but there is limited research on radiotherapy as a standalone treatment. This case report aims to elucidate the clinical outcomes of hypofractionated radiotherapy as a standalone treatment for locally advanced type B2 thymoma, offering insights into its potential efficacy and role in clinical practice.
PubMed: 38304685
DOI: 10.7759/cureus.51528 -
Scientific Reports Feb 2024B-cell subsets in peripheral blood (PB) and tumor microenvironment (TME) were evaluated to determine myasthenia gravis (MG) severity in patients with thymoma-associated...
B-cell subsets in peripheral blood (PB) and tumor microenvironment (TME) were evaluated to determine myasthenia gravis (MG) severity in patients with thymoma-associated MG (TMG) and the distribution of B cells in type B TMG. The distribution of mature B cells, including Bm1-Bm5, CD19 and CD20 B cells and non-switched (NSMBCs) and switched (SMBCs) memory B cells, were determined in 79 patients with thymoma or TMG. Quantitative relationships between the T and TMG groups and the TMG-low and TMG-high subgroups were determined. NSMBCs and SMBCs were compared in TME and PB. Type B thymoma was more likely to develop into MG, with types B2 and B3 being especially associated with MG worsening. The percentage of CD19 B cells in PB gradually increased, whereas the percentage of CD20 B cells and the CD19/CD20 ratio were not altered. The (Bm2 + Bm2')/(eBm5 + Bm5) index was significantly higher in the TMG-high than in thymoma group. The difference between SMBC/CD19 and NSMBC/CD19 B cell ratios was significantly lower in the thymoma than TMG group. NSMBCs assembled around tertiary lymphoid tissue in thymomas of patients with TMG. Few NSMBCs were observed in patients with thymoma alone, with these cells being diffusely distributed. MG severity in patients with TMG can be determined by measuring CD19 B cells and Bm1-Bm5 in PB. The CD19/CD20 ratio is a marker of disease severity in TMG patients. Differences between NSMBCs and SMBCs in PB and TME of thymomas can synergistically determine MG severity in patients with TMG.
Topics: Humans; Thymoma; B-Lymphocyte Subsets; Thymus Neoplasms; B-Lymphocytes; Myasthenia Gravis; Tumor Microenvironment
PubMed: 38302676
DOI: 10.1038/s41598-024-53250-6 -
Journal of Cardiothoracic Surgery Jan 2024Thymoma and myasthenia gravis (MG) interact with each other. This study aimed to evaluate the effects of thymoma on neurological outcome of MG patients after thymectomy...
BACKGROUND
Thymoma and myasthenia gravis (MG) interact with each other. This study aimed to evaluate the effects of thymoma on neurological outcome of MG patients after thymectomy using the propensity score matching (PSM) method.
METHODS
Consecutive patients with MG who underwent thymectomy at Beijing Hospital between January 2012 and August 2021 were retrospectively enrolled. Clinical and follow-up data were collected. Statistical analysis was performed using SPSS 23.0 software. PSM was performed to eliminate selection bias.
RESULTS
A total of 456 patients were included in this study. Thymoma was present in 138 (30.3%) patients. The median follow-up time was 72 (range, 12-135) months. At the last follow-up, a lower proportion of thymomatous MG patients achieved complete stable remission (CSR) compared with non-thymomatous MG patients (P = 0.011), and the effective rate [CSR + pharmatologic remission (PR) + minimal manifestations (MM)] of thymomatous MG patients was also lower (P = 0.037). Considering time to CSR, Kaplan-Meier analysis showed thymomatous MG patients had lower cumulative CSR rate than non-thymomatous MG patients (log-rank, P = 0.019). After PSM, 105 pairs of patients were matched successfully. For the matched patients, thymomatous MG patients had a lower CSR rate and a lower effective rate (P = 0.002, 0.039, respectively), and K-M analysis still showed thymomatous MG patients had lower cumulative CSR rate (log-rank, P = 0.048). Multivariate Cox analysis demonstrated that thymoma (HR: 0.592, 95% CI 0.389-0.900, P = 0.014), older age at the time of surgery (HR: 0.971, 95% CI 0.953-0.990, P = 0.003), and preoperative course of MG > 12 months (HR: 0.474, 95% CI 0.317-0.708, P = 0.000) were negative predictive factors for CSR.
CONCLUSIONS
Thymoma had a negative effect on the neurological outcome of MG after thymectomy. MG patients with old age and a preoperative course of longer than one year had a lower probability of achieving CSR.
Topics: Humans; Thymoma; Thymectomy; Propensity Score; Retrospective Studies; Thymus Neoplasms; Myasthenia Gravis; Pathologic Complete Response; Treatment Outcome
PubMed: 38297367
DOI: 10.1186/s13019-024-02511-6 -
Kardiochirurgia I Torakochirurgia... Dec 2023Mediastinal tumors encompass a diverse range of malignancies, originating within or spreading to the mediastinum. The administration of radiotherapy within the... (Review)
Review
Mediastinal tumors encompass a diverse range of malignancies, originating within or spreading to the mediastinum. The administration of radiotherapy within the anatomical confines of the mediastinum presents unique challenges owing to the close proximity of critical organs, including the heart, lungs, esophagus, and spinal cord. However, recent progress in imaging techniques, treatment modalities, and our understanding of tumor biology has significantly contributed to the development of effective and safe therapeutic strategies for mediastinal diseases. This review article aims to explore the latest innovations in radiotherapy and their practical applications in the management of mediastinal tumors, with a primary focus on lymphomas, thymomas, and thymic carcinomas. By examining these advancements, we seek to provide valuable insights into the current state of the art in radiotherapy for mediastinal malignancies, ultimately fostering improved patient outcomes and clinical decision-making.
PubMed: 38283558
DOI: 10.5114/kitp.2023.134132 -
Medicine International 2024The present study reports the case of a patient with acute myeloid leukemia post-cytotoxic therapy (AML-pCT) that developed following chemotherapy for thymoma. A...
The present study reports the case of a patient with acute myeloid leukemia post-cytotoxic therapy (AML-pCT) that developed following chemotherapy for thymoma. A 64-year-old female patient underwent surgical resection for a mediastinal tumor and was diagnosed with stage IVa thymoma. She received chemotherapy, including carboplatin/etoposide, carboplatin/paclitaxel and amrubicin monotherapy. At 56 months following surgery, she developed blastosis and was diagnosed with AML-pCT. As demonstrated herein, although treatment for thymoma is associated with a markedly lower frequency of myeloid neoplasms post-cytotoxic therapy (MN-pCT) than treatment for other malignancies, such as breast carcinoma, it is important to be aware that MN-pCT may occur as a late complication of thymoma treatment.
PubMed: 38283133
DOI: 10.3892/mi.2024.133 -
Journal of Clinical Medicine Jan 2024Thymomas are rare tumors of the anterior mediastinum with peculiar clinical and pathological features. They have been deeply analyzed by pioneer authors, who strictly... (Review)
Review
Thymomas are rare tumors of the anterior mediastinum with peculiar clinical and pathological features. They have been deeply analyzed by pioneer authors, who strictly linked their name to the main pathological and staging classifications. Before the latest edition of the WHO classification of thymic epithelial tumors, the history of thymoma pathological classification inherited the name of the pathologists who systematically addressed the issue, from Levine-Rosai to Muller-Hermelink. Similarly, the thymoma staging system is intimately related to the name of two surgeons, Masaoka and Koga, who historically dealt with this disease. More recently, the traditional tumor-nodes-metastasis (TNM) system has been developed for the staging of this condition, in a rational attempt to put thymomas in conformity with the other solid tumors. The efforts of the International Thymic Malignancies Interest Group (ITMIG) and the Thymic Domain of the Staging and Prognostic Factors Committee (TD-SPFC) of the International Association for the Study of Lung Cancer (IASLC) resulted in the TNM classification of thymic tumors, which have been included in the eighth edition of the American Joint Committee on Cancer's (AJCC) Cancer Staging Manual. Herein, we report a narrative review of the evolution of the thymic epithelial tumors (TET) staging system and present a critical appraisal of the actual TNM classification compared with the historical Masaoka-Koga classification, with special focus on the proposal for the ninth edition of the TNM, expected in 2024.
PubMed: 38276116
DOI: 10.3390/jcm13020610