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Medicine Dec 2022Thymic epithelial tumors (TETs), including thymomas and thymic carcinomas. Thymomas are rare tumors, but are one of the most common mediastinal neoplasms in adults. The...
INTRODUCTION
Thymic epithelial tumors (TETs), including thymomas and thymic carcinomas. Thymomas are rare tumors, but are one of the most common mediastinal neoplasms in adults. The pathology of thymoma with Systemic Lupus Erythematosus (SLE) and severe pancytopenia is rarely reported. Thus, the current study demonstrates a case of thymoma with SLE and immune-related anemia with unique clinical manifestation.
PATIENT CONCERNS
A 27-year-old woman suffered from abdominal pain, arthralgia, intermittent high fever for a long time.
DIAGNOSIS
Based on the clinical and histopathological manifestations, diagnosis of thymoma with SLE and immune-related anemia was established.
INTERVENTIONS
Patient was treated with methylprednisolone and a complete thymectomy and thymomectomy, the CAP regimen was given 4 times of adjuvant chemotherapy after the operation.
OUTCOMES
After inter-disciplinary consultation as well as extensive discussion and steroid pulse therapy underwent surgery, the patient's blood count and immune function gradually entry sent back to normal.
CONCLUSION
we present the diagnosis and treatment of a case of thymoma with SLE and immune-related anemia, and provides references for the clinical diagnosis and treatment of thymoma combined with SLE, and attempts to explain that SLE patients with thymoma may contribute to the clinical remission of SLE after thymoma resection. It should arouse the attention of clinicians when diagnosing and treating related diseases.
Topics: Adult; Female; Humans; Thymoma; Thymus Neoplasms; Lupus Erythematosus, Systemic; Methylprednisolone; Anemia
PubMed: 36626535
DOI: 10.1097/MD.0000000000032077 -
Annals of Surgical Oncology Mar 2023Our previous studies have demonstrated that human parvovirus B19 (B19V) is involved in the pathogenesis of thymic hyperplasia-associated myasthenia gravis (MG). However,...
PURPOSE
Our previous studies have demonstrated that human parvovirus B19 (B19V) is involved in the pathogenesis of thymic hyperplasia-associated myasthenia gravis (MG). However, more cases need to be assessed to further elucidate the relationship between this virus and thymoma-associated MG.
MATERIALS AND METHODS
The clinicopathological characteristics, presence of B19V DNA, and B19V VP2 capsid protein expression of 708 cases of thymomas were investigated using nested polymerase chain reaction (PCR), TaqMan quantitative (q) PCR, immunohistochemistry, fluorescent multiplex immunohistochemistry, and electron microscopy.
RESULTS
Patients with MG or ectopic germinal centers (GCs) were significantly younger than those without MG (P < 0.0001) or GCs (P = 0.0001). Moreover, significantly more GCs were detected in thymomas associated with MG than in those without MG (P < 0.0001). The results of nested PCR and TaqMan qPCR were consistent, and B19V DNA positivity was only associated with presence of GCs (P = 0.011). Immunohistochemically, positive staining was primarily detected in neoplastic thymic epithelial cells (TECs) and ectopic GCs. The positive rate of B19V VP2 was significantly higher in thymoma with MG or GCs than in thymoma without MG (P = 0.004) or GCs (P = 0.006). Electron microscopy showed B19V particles in the nuclei of neoplastic TECs and B cells from GCs.
CONCLUSIONS
We conclude that the pathogenesis of MG is closely associated with the presence of GCs, and B19V infection is plausibly an essential contributor to formation of ectopic GCs in thymoma. To the best of the authors' knowledge, this is the first study to elucidate the role of B19V in thymoma-associated MG and provide new ideas for exploring the etiopathogenic mechanism of MG.
Topics: Humans; Parvovirus B19, Human; Thymoma; Myasthenia Gravis; Risk Factors; Thymus Neoplasms
PubMed: 36509875
DOI: 10.1245/s10434-022-12936-9 -
BMC Surgery Aug 2016Malignant thymomas are rarely associated with bone metastasis and T-cell lymphocytosis. (Review)
Review
BACKGROUND
Malignant thymomas are rarely associated with bone metastasis and T-cell lymphocytosis.
CASE PRESENTATION
A 47-year-old female patient was admitted to our hospital for ptosis. A diagnosis of malignant thymoma was made based on the thymectomy and pathological result. Peripheral T-cell lymphocytosis and bone metastasis were found later. T-cell lymphocytosis was relived after surgical and radiation therapy to the metastasis.
CONCLUSION
Peripheral T-cell lymphocytosis is a rare paraneoplastic phenomenon associated with thymomas. This report is the first to describe an invasive thymoma with late bone metastasis accompanied with T-cell lymphocytosis. We should be aware of peripheral T-cell lymphocytosis in thymomas and it may contribute to a better understanding of the complex physiology and pathogenesis of thymoma.
Topics: Bone Neoplasms; Diagnosis, Differential; Female; Humans; Lymphocytosis; Magnetic Resonance Imaging; Middle Aged; T-Lymphocytes; Thymoma; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 27542926
DOI: 10.1186/s12893-016-0171-0 -
Cancer Medicine May 2023Due to the low incidence and histological heterogeneity, the molecular features and underlying carcinogenic mechanisms of thymic epithelial tumors (TETs) are yet to be...
BACKGROUND
Due to the low incidence and histological heterogeneity, the molecular features and underlying carcinogenic mechanisms of thymic epithelial tumors (TETs) are yet to be fully elucidated, especially for different subtypes of TETs.
METHODS
Tumor tissue samples of 43 TETs with distinct histological subtypes were collected. We analyzed the molecular characteristics in different subtypes based on whole exome sequencing data.
RESULTS
The mutational profiles of the different subtypes of TETs varied. Compared with thymomas, thymic carcinomas (TCs) had a higher mutation frequency of MYO16 (33% vs. 3%, p = 0.024) and a lower frequency of ZNF729 mutations (0% vs. 35%, p = 0.044). No significant difference was observed in the median tumor mutation burden across different subtypes. The value of copy number variation burden, weighted genome instability index, and the number of amplified segments were all higher in TCs than thymomas, and they also tended to be higher in B3 thymoma than in non-B3 thymomas, while they had no significant differences between B3 thymoma and TCs. Clustering analyses revealed that Wnt, MAPK, Hedgehog, AMPK, and cell junction assembly signaling pathways were exclusively enriched in non-B3 thymomas, lysine degradation pathway in B3 thymoma, and extracellular matrix-receptor (ECM-receptor) interaction, positive regulation of cell cycle process, and activation of innate immune response pathways in TCs.
CONCLUSIONS
This study revealed distinct molecular landscapes of different subtypes of TETs, suggesting diverse pathogenesis of non-B3 thymomas, B3 thymomas, and TCs. Our findings warrant further validation in future large-scale studies and may provide a theoretical basis for potential personalized therapeutic strategies.
Topics: Humans; Thymoma; DNA Copy Number Variations; Thymus Neoplasms; Neoplasms, Glandular and Epithelial; Molecular Biology
PubMed: 36916520
DOI: 10.1002/cam4.5795 -
PloS One 2015Pleural spread is difficult to treat in malignancies, especially in lung cancer and thymoma. Monotherapy with surgery fails to have a better survival benefit than...
Pleural spread is difficult to treat in malignancies, especially in lung cancer and thymoma. Monotherapy with surgery fails to have a better survival benefit than palliative chemotherapy, the currently accepted treatment. Photodynamic therapy utilizes a photosensitizer to target the tumor site, and the tumor is exposed to light after performing a pleurectomy and tumor resection. However, the benefits of this procedure to lung cancer or thymoma patients are unknown. We retrospectively reviewed the clinical characteristics and treatment outcomes of patients with lung cancer or thymoma with pleural seeding who underwent pleural photodynamic therapy and surgery between 2005 and 2013. Eighteen patients enrolled in this study. The mean patient age was 52.9 ± 12.2 years. Lung cancer was the inciting cancer of pleural dissemination in 10 patients (55.6%), and thymoma in 8 (44.4%). There was no procedure-related mortality. Using Kaplan-Meier survival analysis, the 3-year survival rate and the 5-year survival rate were 68.9% and 57.4%, respectively. We compared the PDT lung cancer patients with those receiving chemotherapy or target therapy (n = 51) and found that the PDT group had better survival than non-PDT patients (mean survival time: 39.0 versus 17.6 months; P = .047). With proper patient selection, radical surgical resection combined with intrapleural photodynamic therapy for pleural spread in patients with non-small cell lung cancer or thymoma is feasible and may provide a survival benefit.
Topics: Adult; Aged; Combined Modality Therapy; Disease-Free Survival; Female; Humans; Lung Neoplasms; Male; Middle Aged; Photochemotherapy; Pleura; Prognosis; Retrospective Studies; Survival Rate; Thoracotomy; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 26193470
DOI: 10.1371/journal.pone.0133230 -
Journal of Thoracic Oncology : Official... May 2014The 2004 version of the World Health Organization classification subdivides thymic epithelial tumors into A, AB, B1, B2, and B3 (and rare other) thymomas and thymic...
INTRODUCTION
The 2004 version of the World Health Organization classification subdivides thymic epithelial tumors into A, AB, B1, B2, and B3 (and rare other) thymomas and thymic carcinomas (TC). Due to a morphological continuum between some thymoma subtypes and some morphological overlap between thymomas and TC, a variable proportion of cases may pose problems in classification, contributing to the poor interobserver reproducibility in some studies.
METHODS
To overcome this problem, hematoxylin-eosin-stained and immunohistochemically processed sections of prototypic, "borderland," and "combined" thymomas and TC (n = 72) were studied by 18 pathologists at an international consensus slide workshop supported by the International Thymic Malignancy Interest Group.
RESULTS
Consensus was achieved on refined criteria for decision making at the A/AB borderland, the distinction between B1, B2, and B3 thymomas and the separation of B3 thymomas from TCs. "Atypical type A thymoma" is tentatively proposed as a new type A thymoma variant. New reporting strategies for tumors with more than one histological pattern are proposed.
CONCLUSION
These guidelines can set the stage for reproducibility studies and the design of a clinically meaningful grading system for thymic epithelial tumors.
Topics: Antigens, CD20; CD5 Antigens; Carcinoma; Glucose Transporter Type 1; Humans; Mucin-1; Proto-Oncogene Proteins c-kit; Reproducibility of Results; Thymoma; Thymus Neoplasms; World Health Organization
PubMed: 24722150
DOI: 10.1097/JTO.0000000000000154 -
Medicine Nov 2021Although differentiating benign and malignant thymic epithelial lesions is important to avoid unnecessary treatment and predict prognosis, it is challenging because of...
Although differentiating benign and malignant thymic epithelial lesions is important to avoid unnecessary treatment and predict prognosis, it is challenging because of overlaps in the chest computed tomography (CT) findings. In this study, we investigated whether the diameter of the thymic vein and other CT findings could differentiate between benign (thymoma and thymic cysts) and malignant (thymic carcinoma, [TCa]) lesions.We conducted a retrospective study across two tertiary referral hospitals in Japan between November 2009 and June 2018. We included 12 patients with TCa, 34 patients with thymomas, and 17 patients with thymic cysts. We analyzed the receiver operating characteristic (ROC) curve to determine the best cut-off values and performed univariate and multivariate analyses of CT findings to distinguish TCa from other benign lesions. Post-hoc analysis was performed for the maximum short axis of the thymic vein using the Mann-Whitney U test, and the number of the maximum short axis of the thymic vein ≥ the cutoff was determined using the Fisher exact test with a family-wise error-correction using Bonferroni's method.ROC analysis showed that a maximum short axis of the thymic vein ≥2 mm was considerably more frequent in TCa than in the other lesions (P < .001 for both), with 83% sensitivity and 86% specificity. Univariate and multivariate analyses revealed the association with TCa of the number of the maximum short axis of the thymic vein ≥2 mm (P = .005, multivariate generalized linear model analysis), ill-defined margin (P = .001), and mediastinal lymphadenopathy (P < .001). Thymic vein diameter was in descendimg order of TCa > thymoma > thymic cysts with statistically significant differences between the groups (Ps < .05).Thymic vein diameter was significantly longer in TCa than in thymoma and thymic cysts. Measurement of the maximum short axis of the thymic vein could be a powerful diagnostic tool to differentiate TCa from thymoma and thymic cysts.
Topics: Adult; Aged; Aged, 80 and over; Diagnosis, Differential; Female; Humans; Male; Mediastinal Cyst; Middle Aged; Neoplasms, Glandular and Epithelial; Retrospective Studies; Thymoma; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 34797351
DOI: 10.1097/MD.0000000000027942 -
Interactive Cardiovascular and Thoracic... Aug 2012A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'Which stages of thymoma benefit from adjuvant... (Review)
Review
A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'Which stages of thymoma benefit from adjuvant chemotherapy post thymectomy?' Altogether more than 150 papers were found using the reported search, of which only eight represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated; these studies have mainly reported the survival and recurrence rates of post-thymectomy patients who received adjuvant radiotherapy or chemoradiotherapy, and adjuvant radiotherapy alone was only used in a small group of patients in these studies. We did not find any randomized controlled trials comparing adjuvant chemotherapy with chemo/radiotherapy and, due to a very small incidence of this tumour, it is unlikely to see any trials in future. Studies were mainly retrospective or institutional reports and showed that, despite the high sensitivity of this tumour to chemotherapy agents and the use of chemotherapy as one of the main treatment modalities in the advanced stages of thymoma, current data are not supporting postoperative chemotherapy as a sole adjuvant treatment in advanced stages of thymoma. We conclude that, in patients with thymoma, surgical resection with or without radiation therapy is the gold standard treatment for early-stage disease (I and II). Adjuvant radiotherapy/chemoradiotherapy should be considered for Masaoka stage III (A and B) or above, and it is also advised to add adjuvant therapy for all patients with cortical fenestration, even in stages I and II. But there is no evidence that chemotherapy alone improves the survival in patients with completely resected stage III and IV thymomas and thymic carcinoma. In patients with extra-radiation field disease, however, the use of chemotherapy can potentially improve survival but no follow-up data on this group of patients are available.
Topics: Benchmarking; Chemotherapy, Adjuvant; Evidence-Based Medicine; Humans; Neoplasm Staging; Patient Selection; Predictive Value of Tests; Thymectomy; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 22552797
DOI: 10.1093/icvts/ivs133 -
Journal of Thoracic Oncology : Official... Oct 2010Thymomas are neoplasms arising from the epithelial thymic cells, with a recurrence rate after complete resection ranging from 5 to 50%, according to the initial stage of... (Review)
Review
Thymomas are neoplasms arising from the epithelial thymic cells, with a recurrence rate after complete resection ranging from 5 to 50%, according to the initial stage of disease. The pleura is the most frequent site of relapse, while distant metastases occur in less than 5% of the cases. Data in the literature show that recurrences can occur several years after the resection of a thymoma; unfortunately, few reports have analyzed the treatment of recurrences of thymoma. There is no standard treatment for the recurrent disease, and most of the treatments proposed by the authors are based more on their personal experience and individualized treatment than on an evidence-based method. Moreover, most series concerning surgery for recurrent thymomas suffer from a selection bias: patients with limited disease and better performance status are usually selected for surgery, with an anticipated survival advantage in the surgical group. Surgery for recurrent thymoma is indicated if complete resection is feasible, whereas in the case of predicted debulking surgery, it may be considered as part of a multidisciplinary protocol. In the literature, a few case reports of the surgical treatment of metachronous distant metastases to liver or brain from thymoma have been described, but they are really rare, and a decision about their treatment is always on a single patient basis. We can conclude that reoperation for thymoma recurrences is feasible and relatively safe, and it should always be considered because the other treatment modalities are not curative.
Topics: Adult; Humans; Liver Neoplasms; Male; Neoplasm Recurrence, Local; Neoplasm Staging; Reoperation; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 20859131
DOI: 10.1097/JTO.0b013e3181f20f27 -
Annals of Thoracic and Cardiovascular... Dec 2022Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder. Paraneoplastic SPS associated with malignant tumors such as thymoma occurs in approximately 5% of...
INTRODUCTION
Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder. Paraneoplastic SPS associated with malignant tumors such as thymoma occurs in approximately 5% of all SPS cases. We present a rare case of thymoma accompanied by SPS successfully treated using surgery.
PRESENTATION OF CASE
A 26-year-old woman presented with lower limbs convulsions and gait disturbance and complained of leg pain. Cerebrospinal fluid and blood test results showed a high level of anti-glutamic acid decarboxylase (GAD) antibodies. Computed tomography showed anterior mediastinal tumor suggestive of a thymoma. She underwent extended thymectomy, and her symptoms gradually improved after surgery. No evidence of recurrent thymoma and SPS has been observed over 44 months.
CONCLUSION
Surgical treatment would be effective for patients with SPS and thymoma.
Topics: Humans; Female; Adult; Thymoma; Stiff-Person Syndrome; Treatment Outcome; Neoplasm Recurrence, Local; Thymus Neoplasms
PubMed: 34275989
DOI: 10.5761/atcs.cr.21-00052