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Cureus Dec 2023Myasthenia gravis (MG), a chronic, autoimmune disease affecting the neuromuscular junction, arises from various autoantibodies, including those against the...
Myasthenia gravis (MG), a chronic, autoimmune disease affecting the neuromuscular junction, arises from various autoantibodies, including those against the acetylcholine receptor (AChR). Recently, efgartigimod, a human IgG1 antibody Fc fragment engineered to reduce the pathogenic IgG autoantibody level, was developed as a treatment for MG. However, the long-term effects of the treatment are still unclear. The present report describes two novel cases of thymoma-associated MG exacerbation following efgartigimod treatment related to anti-AChR antibody overshoot. Both cases shared certain characteristics, including anti-AChR antibody positivity and post-thymectomy status. After a few cycles of efgartigimod treatment, their MG deteriorated, and their anti-AChR antibody titer exceeded the level before efgartigimod therapy. Prior studies show that anti-AChR antibody titer does not correlate with the disease severity of MG. However, previous studies have reported antibody overshoot following plasma exchange, which, like efgartigimod, reduces the level of plasma IgG and autoantibodies. Thus, MG exacerbation with anti-AChR antibody overshoot may be an adverse effect of both efgartigimod and plasma exchange. When clinical symptoms in patients with thymoma-associated MG receiving efgartigimod deteriorate despite low IgG, assessing the anti-AChR antibody level can be important for reconsidering the treatment strategy.
PubMed: 38229781
DOI: 10.7759/cureus.50692 -
Journal of Cancer Research and Clinical... Jan 2024Thymic carcinoma and thymic neuroendocrine tumor (NET) are rare and are more likely to develop second malignancies. The purpose of this study was to explore the...
OBJECTIVES
Thymic carcinoma and thymic neuroendocrine tumor (NET) are rare and are more likely to develop second malignancies. The purpose of this study was to explore the incidence and lifetime risk of second malignancies in thymic carcinoma and thymic NET.
METHODS
The standardized incidence ratio (SIR) and the age-adjusted cancer incidence of the thymic carcinoma and thymic NET patients with second malignancies were retrospectively calculated by using the Surveillance, Epidemiology, and End Results (SEER) database. Prognosis results were also determined by Kaplan-Meier analysis and Cox regression.
RESULTS
1130 patients with thymic carcinoma (73 patients had second malignancies) and 263 patients with thymic NET (19 patients had second malignancies) from 2000 to 2018 are included. Patients with thymic carcinoma (SIR: 1.36, 95% CI 1.08-1.69) and with thymic NET (SIR: 1.73, 95% CI 1.13-2.54) demonstrate an increased overall risk of developing second malignancies in various organ systems. The age-adjusted cancer incidence of second malignancies in patients with thymic carcinoma is 3058.48 per 100,000 persons (4178.46 per 100,000 persons in patients with thymic NET). Age at diagnosis is a significant risk factor for the development of second malignancies.
CONCLUSION
The incidence of second malignancies in patients with thymic carcinoma and thymic NET is significantly higher than the patients in the normal population. The occurrence of second malignancies is not related to the use of different treatments. It is important to extend the follow-up period and add other screening methods.
Topics: Humans; Thymoma; Neuroendocrine Tumors; Neoplasms, Second Primary; Incidence; Retrospective Studies; Thymus Neoplasms
PubMed: 38227201
DOI: 10.1007/s00432-023-05522-3 -
Cancers Dec 2023Thymic epithelial tumors, comprising thymic carcinomas and thymomas, are rare neoplasms. They differ in histology, prognosis, and association with autoimmune diseases... (Review)
Review
Thymic epithelial tumors, comprising thymic carcinomas and thymomas, are rare neoplasms. They differ in histology, prognosis, and association with autoimmune diseases such as myasthenia gravis. Thymomas, but not thymic carcinomas, often harbor GTF2I mutations. Mutations of CDKN2A, TP53, and CDKN2B are the most common thymic carcinomas. The acquisition of mutations in genes that control chromatin modifications and epigenetic regulation occurs in the advanced stages of thymic carcinomas. Anti-angiogenic drugs and immune checkpoint inhibitors targeting the PD-1/PD-L1 axis have shown promising results for the treatment of unresectable tumors. Since thymic carcinomas are frankly aggressive tumors, this report presents insights into their oncogenic drivers, categorized under the established hallmarks of cancer.
PubMed: 38201593
DOI: 10.3390/cancers16010166 -
The Journal of International Medical... Jan 2024This study was performed to evaluate the clinical efficacy of subcostal thoracoscopy and median sternotomy as surgical approaches for thymoma resection and lymph node...
OBJECTIVE
This study was performed to evaluate the clinical efficacy of subcostal thoracoscopy and median sternotomy as surgical approaches for thymoma resection and lymph node dissection. The feasibility, safety, and clinical outcomes of subcostal thoracoscopy were compared with those of median sternotomy.
METHODS
The clinical data of 335 patients with thymoma were retrospectively analyzed. The patients were divided into the subcostal thoracoscopy group and the median sternotomy group. Propensity score matching was performed to obtain comparable subsets of 50 patients in each group. A comparative analysis was conducted on various parameters.
RESULTS
All surgeries were successful, and no conversions to open thoracotomy were required in the subcostal thoracoscopy group. Significant differences in the operative time, intraoperative blood loss, chest tube drainage duration, postoperative hospital stay, patient satisfaction scores, pain assessment, and postoperative complications were observed between the two groups. However, there was no significant difference in the number of lymph nodes or lymph node stations dissected intraoperatively between the two groups.
CONCLUSION
Subcostal thoracoscopy is not inferior to median sternotomy as a surgical approach for thymoma resection and lymph node dissection. Our research provides important new comparative data on minimally invasive thymoma resection.
Topics: Humans; Thymoma; Sternotomy; Propensity Score; Retrospective Studies; Thymus Neoplasms; Treatment Outcome; Thoracoscopy
PubMed: 38194488
DOI: 10.1177/03000605231214470 -
Cancer Imaging : the Official... Jan 2024To determine whether histogram analysis based on unenhanced CT can play a role in the differential diagnosis of thymoma and lymphoma from thymic hyperplasia and cyst...
OBJECTIVE
To determine whether histogram analysis based on unenhanced CT can play a role in the differential diagnosis of thymoma and lymphoma from thymic hyperplasia and cyst (mean CT attenuation > 10 HU).
MATERIALS AND METHODS
This retrospective study included consecutive asymptomatic participants who have prevascular mediastinal lesions incidentally detected by unenhanced CT between December 2013 and August 2020, and with definitive diagnosis by pathology or additional radiologic work-ups. A total of thirteen histogram parameters on enhanced CT were calculated for each lesion, then were compared between tumor (thymoma + lymphoma) and non-tumor (hyperplasia + cyst). Receiver operating characteristic analysis was conducted to investigate the performance of histogram parameter for identifying tumor.
RESULTS
The study population included 192 patients (106 men and 86 women) with a mean age of 50.5 years at the time of CT examination. Of them, 94 patients have tumor (87 thymomas and 7 lymphoma) and 98 have non-tumor (48 thymic hyperplasia and 50 cysts). Nine of the thirteen histogram parameters revealed significant difference between the two groups, including median, minimum, range, 10th percentile, 90th percentile, kurtosis, skewness, uniformity and entropy. No significant difference was observed in the mean CT attenuation between groups. Higher median was found to be independent predictors for distinguishing tumor from non-tumor, and can achieve an area under the curve (AUC) of 0.785 (95% confidence interval [95% IC], 0.720-0.841).
CONCLUSIONS
Histogram analysis based on unenhanced CT may be able to provide some help in the differential diagnosis of incidental lesions in prevascular mediastinal.
GRAND SUPPORT
This study was sponsored by Natural Science Foundation of Shanghai (No. 21ZR1459700).
Topics: Male; Humans; Female; Middle Aged; Thymoma; Retrospective Studies; Thymus Hyperplasia; China; Thymus Neoplasms; Lymphoma; Tomography, X-Ray Computed; Cysts; Diagnosis, Differential
PubMed: 38178266
DOI: 10.1186/s40644-023-00617-z -
Thoracic Cancer Feb 2024Thymic carcinoma is a rare disease with an incidence of around 0.5 cases per million with a poor prognosis. The aim of this study was to assess patient outcomes with...
BACKGROUND
Thymic carcinoma is a rare disease with an incidence of around 0.5 cases per million with a poor prognosis. The aim of this study was to assess patient outcomes with advanced thymic carcinoma receiving first-line chemotherapy.
METHODS
In our retrospective cohort study, we included patients who underwent treatment for metastatic thymic carcinoma between January 2013 to December 2019 in our hospital. Overall survival, progression-free survival (PFS), objective response rates (ORR) and chemotherapy regimens were assessed and analyzed.
RESULTS
A total of 27 patients were retrospectively analyzed. All patients received a platinum (cisplatin or carboplatin) based regimen as first-line chemotherapy (29.6% received ADOC, 11.1% received PE, 40.7% received CP, 14.8% received CAP). The median PFS on first-line chemotherapy was 199 days. The response rate was 40.7%. Median overall survival (OS) was 585 days. Positive CD5 staining was associated with better PFS.
CONCLUSION
We highlight the critical role of platinum-based chemotherapy agents as a primary treatment modality in advanced thymic carcinoma, underscoring the efficacy of platinum as a first-line option for recurrent disease, even in cases previously treated with platinum. Additionally, our findings indicate that CD5 positivity could be associated with improved PFS, suggesting its potential as a prognostic marker.
Topics: Humans; Thymoma; Retrospective Studies; Antineoplastic Combined Chemotherapy Protocols; Antineoplastic Agents; Cisplatin; Platinum; Thymus Neoplasms; Treatment Outcome
PubMed: 38149471
DOI: 10.1111/1759-7714.15198 -
Frontiers in Immunology 2023Multi-antibody-positive myasthenia gravis (MG) presentations are relatively rare, often found in older patients, and generally predict a poor prognosis. We report a case...
Multi-antibody-positive myasthenia gravis (MG) presentations are relatively rare, often found in older patients, and generally predict a poor prognosis. We report a case of a female patient with generalized MG, testing positive for Titin antibodies (Titin-Ab), ryanodine receptor antibodies (RyR-Ab), and acetylcholine receptor antibodies (AChR-Ab), and resistant to acetylcholinesterase inhibitors. Following unsuccessful traditional therapies, she received Telitacicept, leading to significant improvements. This case underscores Telitacicept's potential efficacy for similar patients and offers insights into the clinical characteristics of multi-antibody MG.
Topics: Humans; Female; Aged; Connectin; Acetylcholinesterase; Thymoma; Myasthenia Gravis; Autoantibodies; Thymus Neoplasms
PubMed: 38124751
DOI: 10.3389/fimmu.2023.1270011 -
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 -
Mediastinum (Hong Kong, China) 2023Mediastinal lesions are uncommon. However, because of the vital structures in the mediastinum, large lesions specifically can lead to life-threatening situations.... (Review)
Review
BACKGROUND AND OBJECTIVE
Mediastinal lesions are uncommon. However, because of the vital structures in the mediastinum, large lesions specifically can lead to life-threatening situations. Treatment and management vary considerably with the disease. Therefore, the correct histopathologic diagnosis is important. Here we review lesions that have the potential to present as a giant lesion in the mediastinum. While we focus on the review of histopathologic, immunohistochemical (IHC), and molecular features of these lesions, clinical symptoms and characteristics and prognosis will also be discussed.
METHODS
"Giant" was arbitrarily defined as a size of at least 10 cm in greatest dimension. The 2021 World Health Organization (WHO) classification of mediastinal tumors was searched for tumors reported to be larger than 10 cm. Tumors that can present as giant mediastinal lesions based on our own experience were also included. PubMed search was then performed for these lesions.
KEY CONTENT AND FINDINGS
A great variety of mediastinal lesions can present as giant mass. Those include for instance tumors of blood and lymph vessels, tumors of neurogenic origin, mesenchymal neoplasms, thymic epithelial tumors (TETs), and non-neoplastic cysts. Lesions range from benign to malignant. This review focuses on the most common lesions.
CONCLUSIONS
Many benign and malignant lesions can become a large mass in the mediastinum. Their correct diagnosis is important for the treatment and management of the patient.
PubMed: 38090032
DOI: 10.21037/med-23-23 -
International Journal of Molecular... Dec 2023Angiogenesis significantly influences the carcinogenesis of thymic epithelial tumors (TET). Both thymomas and thymic carcinoma (TC) overexpress VEGF-A and VEGFR-1 and... (Review)
Review
Angiogenesis significantly influences the carcinogenesis of thymic epithelial tumors (TET). Both thymomas and thymic carcinoma (TC) overexpress VEGF-A and VEGFR-1 and -2. This review aims to provide an appraisal of the use of anti-angiogenics in the treatment of TET. The literature research identified 16 studies that were deemed eligible for further analysis. Seven studies assessed the clinical efficacy of sunitinib and five studies the use of apatinib and/or anlotinib. The multicenter Japanese phase II REMORA trial investigated the efficacy of lenvatinib, which is a multi-targeted inhibitor of VEGFR, FGFR, RET, c-Kit, and other kinases. The objective response rate was 38% (25.6-52%), which is the highest documented in TET that progressed after first-line chemotherapy. Anti-angiogenic agents may be useful in the treatment of TET, which are not amenable to curative treatment. Their toxicity profile seems to be acceptable. However, angiogenesis inhibitors do not appear to have a major influence on either thymomas or TC, although multikinase inhibitors may have some effect on TC. The current evidence suggests that the most active agent is lenvatinib, whereas sunitinib could be proposed as an acceptable second-line therapy for TC. Further research concerning the combination of immune checkpoint inhibitors with anti-angiogenic drugs is warranted.
Topics: Humans; Thymoma; Angiogenesis Inhibitors; Sunitinib; Thymus Neoplasms; Neoplasms, Glandular and Epithelial; Multicenter Studies as Topic
PubMed: 38069386
DOI: 10.3390/ijms242317065