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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 -
Frontiers in Immunology 2024Thymic epithelial tumors (TETs) are rare mediastinal cancers originating from the thymus, classified in two main histotypes: thymoma and thymic carcinoma (TC). TETs... (Review)
Review
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.
Topics: Adult; Humans; Thymoma; Autoimmunity; Thymus Neoplasms; Neoplasms, Glandular and Epithelial; Myasthenia Gravis; Tumor Microenvironment
PubMed: 38629065
DOI: 10.3389/fimmu.2024.1288045 -
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 -
Medicine Nov 2021Sclerosing thymoma (ST) is quite a rare disease, as denoted in previous literature. Less than 20 cases of ST have been reported to date. However, the combined thymoma,...
RATIONALE
Sclerosing thymoma (ST) is quite a rare disease, as denoted in previous literature. Less than 20 cases of ST have been reported to date. However, the combined thymoma, composed of both type AB thymoma and ST, has never been described before.
PATIENT CONCERNS
The subject, a 49-year-old woman, came in with the chief complaint of cough for 10 days.
DIAGNOSES
Both the contrast-enhanced computed tomography scan and the ultrasonography showed a huge mass located in the right thoracic cavity with inhomogeneous contrast accompanied by the invasion of the pericardium and pleura. Subsequently, computed tomography-guided core-needle biopsy revealed type B2 thymoma, and type AB thymoma could not be excluded. Based on postsurgical histopathology and immunohistochemical finding, this tumor was given the final diagnosis of ST and type AB thymoma.
INTERVENTIONS
After 6 months of adjuvant chemotherapy and local radiotherapy, total thymectomy was performed.
OUTCOMES
The patient has been duly followed up for 1 year without any tumor recurrence.
LESSONS
ST is a very rare mediastinal neoplasm. Moreover, ST in combination with AB thymoma and affecting a large area, is unprecedented. Whether radiotherapy and chemotherapy have a certain effect on ST requires further investigation. In addition, due to the unclear recurrence rate of ST, long-term follow-up evaluation seems necessary.
Topics: Biopsy; Chemotherapy, Adjuvant; Cough; Female; Humans; Mediastinal Neoplasms; Middle Aged; Radiotherapy; Thymectomy; Thymoma; Thymus Neoplasms; Tomography, X-Ray Computed; Treatment Outcome; Ultrasonography
PubMed: 34797329
DOI: 10.1097/MD.0000000000027873 -
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 -
Medicine Dec 2023Patients who have myasthenia gravis or dermatomyositis show clinical signs of muscular weakening. Ocular muscle involvement is uncommon, and symmetrical proximal limb...
RATIONALE
Patients who have myasthenia gravis or dermatomyositis show clinical signs of muscular weakening. Ocular muscle involvement is uncommon, and symmetrical proximal limb weakness is the typical presentation of dermatomyositis. However, the earliest and most noticeable sign in those with myasthenia gravis is extraocular muscular paralysis. Dermatomyositis is frequently complicated by malignancy, and the common malignancies associated with dermatomyositis vary by region and ethnicity, while thymoma is relatively rare. About 10% to 15% of people with myasthenia gravis have thymoma, which is involved in the etiology of the disease.
PATIENT CONCERNS
A 68-year-old female presented with ocular muscle weakness for 10 days that manifested as bilateral blepharoptosis with the phenomenon of "light in the morning and heavy in the evening." Imaging examination showed anterior mediastinal thymic tumor with metastasis.
DIAGNOSES
After a thorough physical examination, we discovered bilateral upper limbs with grade IV muscle strength and the typical rash of dermatomyositis. In combination with elevated serum kinase levels and electromyography suggesting myogenic damage, the patient was finally diagnosed as dermatomyositis with multiple metastases of thymoma.
INTERVENTIONS
The patient received oral hydroxychloroquine sulfate, topical corticosteroids, and tacrolimus ointment, but these did not work very well. Subsequently, the patient underwent surgery combined with radiotherapy for the thymoma.
OUTCOMES
Muscle weakness in the patient improved after effective treatment of tumor, and the rash mostly disappeared.
CONCLUSION
Ocular muscle weakness and thymoma are more common in myasthenia gravis, but we cannot ignore the possibility of dermatomyositis. To further establish the diagnosis, a thorough physical examination and laboratory findings are required. Further tumor screening should be performed for patients with dermatomyositis. Early detection and management of possible tumors are essential to the treatment of dermatomyositis linked to malignancies.
Topics: Female; Humans; Aged; Thymoma; Dermatomyositis; Myasthenia Gravis; Thymus Neoplasms; Muscle Weakness; Exanthema
PubMed: 38115296
DOI: 10.1097/MD.0000000000036234 -
Indian Journal of Pathology &... 2022Myasthenia gravis (MG) is a prototypic T-cell-dependent antibody-mediated autoimmune disease that leads to ocular or generalized muscular weakness. The disease is most...
BACKGROUND
Myasthenia gravis (MG) is a prototypic T-cell-dependent antibody-mediated autoimmune disease that leads to ocular or generalized muscular weakness. The disease is most commonly caused by antibodies to the acetylcholine receptors, often with underlying thymic pathology.
AIMS
This study is aimed at analyzing the pathological spectrum of the excised thymuses in patients with myasthenia.
MATERIALS AND METHODS
This was a retrospective 10-year study of 68 thymectomy specimens performed as a part of the treatment of patients with MG.
STATISTICAL ANALYSIS
Nil.
RESULTS
There were 47 males and 21 females (male to female ratio of 2.2:1) with a mean age of 41 years. Only three patients presented with ocular myasthenia. The thymus was normal in 9 patients (13.2%) and atrophic in 17 patients (25%). Follicular hyperplasia and thymomas were seen in 6 and 36 patients, respectively.
CONCLUSION
The thymectomies performed in patients of MG had a fairly variable spectrum on histology; the thymic tumors were predominantly of the cortical phenotype.
Topics: Adolescent; Adult; Aged; Female; Histological Techniques; Humans; Male; Middle Aged; Myasthenia Gravis; Retrospective Studies; Thymectomy; Thymoma; Young Adult
PubMed: 35074977
DOI: 10.4103/IJPM.IJPM_935_20 -
Zhongguo Fei Ai Za Zhi = Chinese... Jan 2021Thymomas are the most common primary malignant tumors of anterior mediastinal. However, there are no specific laboratory indicator for the diagnosis the diagnosis of...
BACKGROUND
Thymomas are the most common primary malignant tumors of anterior mediastinal. However, there are no specific laboratory indicator for the diagnosis the diagnosis of thymoma. The aim of this study was to screen out a tumor marker for diagnosis of thymoma by mRNA microarray analysis and confirmed it.
METHODS
By mRNA microarray analysis of 31 thymomas and peritumoral thymic tissues, we found that the transcription level of neuronal pentraxin 1 (NPTX1) gene was up-regulated more than 4 times in thymomas. To further verify the above results, we detected the transcription and expression level of NPTX1 in 60 thymoma and 30 thymic cyst patients by quantitative Real-Time polymerase chain reaction (PCR), immunohistochemistry and enzyme-linked immunosorbent assay (ELISA). Furthermore, the diagnostic value of NPTX1 in thymoma by receiver operating characteristic curve (ROC) was analyzed.
RESULTS
The transcription level of NPTX1 mRNA in thymoma tissues was significantly higher than that in the thymic tissues of control group [(2.88±1.02) vs (1.35±0.47), P<0.001); The expression level of NPTX1 in thymoma tissues was significantly higher than that in the thymic tissues of control group (2 vs 1, P<0.001); The preoperative serum level of NPTX1 protein in thymoma patients were significantly higher than that in the thymic cyst patients of control group [(1,018.29±209.38) pg/mL vs (759.95±66.02) pg/mL, P<0.001]; At the threshold of 842.22 pg/mL, sensitivity and specificity of NPTX1 as a serologic marker were 85.00% and 93.33%, respectively for thymoma. ROC showed that the area the under curve (AUC) of NPTX1 was 0.902.
CONCLUSIONS
NPTX1 was highly expressed in thymoma patients, and had diagnostic value for thymoma.
Topics: Adult; Aged; Biomarkers, Tumor; C-Reactive Protein; Diagnosis, Differential; Female; Humans; Lung Neoplasms; Male; Middle Aged; Nerve Tissue Proteins; ROC Curve; Thymoma; Young Adult
PubMed: 33478183
DOI: 10.3779/j.issn.1009-3419.2021.102.03 -
Medicine Jul 2020Intraoperative radiotherapy (IORT) has been used to treat different residual solid tumors after tumor removal and has shown many advantages over other treatment methods.... (Observational Study)
Observational Study
Intraoperative radiotherapy (IORT) has been used to treat different residual solid tumors after tumor removal and has shown many advantages over other treatment methods. However, the use of IORT for invasive thymoma has not been reported. Therefore, in this study, we tried to determine the safety and efficacy of INTRABEAM IORT for the treatment of invasive thymoma.Among the patients admitted to our hospital from September to December 2016 who were diagnosed with invasive thymoma, 14 were selected as study subjects. With medical histories taken beforehand, 8 of these patients were diagnosed with Masaoka stage IIA and 6 with Masaoka stage IIB; furthermore, 5 of the patients were diagnosed with myasthenia gravis (MG). INTRABEAM radiation (8-10 Gy, low energy) was delivered to the postoperative tumor bed of each patient during surgery. The intra- and postoperative complications were observed and evaluated, and the improvement in symptoms was assessed. An additional 23 patients with stage II thymoma undergoing radical surgery from April to August 2016 were chosen as the control group.One month after the operation, only 1 patient in the IORT group had cough, increased levels of leucocytes and neutrophils, and pulmonary inflammation on chest computed tomography. Reactive inflammation and pleural effusion in the 2 groups were similar (P > .05). There was no significant difference between the 2 groups in the improvement of myasthenia gravis (P > .05). Postoperative chest computed tomography and routine blood examination at 3 and 12 months showed that all the patients recovered, with normal hemogram levels and no pulmonary fibrosis around the radiation field. In addition, ultrasonic cardiography and electrocardiography demonstrated no significant difference before or after surgery within the IORT group. At the end of the follow-up, all the patients were alive, no relapse or remote metastasis was observed in the IORT group, and 2 inpatients in the control group had experienced relapse at 24 and 26 months. There was a significant difference in disease-free survival between the 2 groups (P = .00).It is safe to administer low-energy INTRABEAM IORT at a dose of approximately 10 Gy in patients with stage II invasive thymoma. INTRABEAM IORT does not significantly increase operation- or radiation-related complications and has no significant effect on vital organs such as the lungs and heart. Its long-term efficacy is worth expecting.
Topics: Adult; Aged; Case-Control Studies; Female; Humans; Male; Middle Aged; Myasthenia Gravis; Radiotherapy Dosage; Radiotherapy, Adjuvant; Thoracic Surgery, Video-Assisted; Thymoma; Thymus Neoplasms
PubMed: 32629705
DOI: 10.1097/MD.0000000000020964 -
Chinese Medical Journal Apr 2018The co-existence of myasthenia gravis (MG) and thymoma makes the surgical treatment more complicated and adjuvant radiation more controversial. The aim of this study was...
BACKGROUND
The co-existence of myasthenia gravis (MG) and thymoma makes the surgical treatment more complicated and adjuvant radiation more controversial. The aim of this study was to investigate adjuvant radiotherapy for thymoma with MG after extended thymectomy.
METHODS
A total of 181 patients with both MG and thymoma were recruited between 2003 and 2014 at Tongren Hospital, China. Among all the patients, 157 patients received radiation therapy after surgery (Group A); whereas the other 24 patients did not receive radiation therapy (Group B). According to the time that patients started mediastinal radiation therapy, we subdivided the 157 patients in Group A into subgroups (1-month subgroup, n = 98; 2-month subgroup, n = 7; and 3-month subgroup, n = 52). We then compared the effect of the mediastinal radiation therapy across these different groups using the survival rate, the rate of postoperative myasthenic crisis, and the complete stable remission (CSR) rate as the primary endpoints.
RESULTS
There was a significant difference in the occurrence of postoperative myasthenic crisis between 1-month subgroup and Group B (χ = 4.631, P = 0.031). The rates of reaching CSR were 32.6% in 1-month subgroup, 25% in 3-month subgroup, and 22.7% in Group B, respectively. The overall survival rates of 1-month subgroup, 3-month subgroup, and Group B were 88.8%, 83.3%, and 77.3%, respectively. Analysis on the Kaplan-Meier survival curves demonstrated that within 8 years after surgery, there was no significant difference in aspects of overall survival and disease-free survival between 1-month subgroup and Group B, and between 3-month subgroup and Group B; over 8 years after surgery, the disease-free survival rates in 1-month subgroup, 3-month subgroup and Group B were 79.4%, 70.6%, and 55.3%, respectively.
CONCLUSIONS
Adjuvant radiation within 1 month after extended thymectomy may be helpful in controlling postoperative MG, such as decreasing the possibility of postoperative myasthenic crisis, and raising cumulative probabilities of reaching CSR.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Myasthenia Gravis; Postoperative Period; Radiotherapy, Adjuvant; Thymectomy; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 29664052
DOI: 10.4103/0366-6999.229894