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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 -
International Journal of Molecular... Nov 2023Despite recent advancements, therapies against advanced oral squamous cell carcinoma (OSCC) remain ineffective, resulting in unsatisfactory therapeutic outcomes. Cold...
Despite recent advancements, therapies against advanced oral squamous cell carcinoma (OSCC) remain ineffective, resulting in unsatisfactory therapeutic outcomes. Cold atmospheric plasma (CAP) offers a promising approach in the treatment of malignant neoplasms. Although the effects of CAP in abrogating OSCC have been explored, the exact mechanisms driving CAP-induced cancer cell death and the changes in microRNA (miRNA) expression are not fully understood. We fabricated and calibrated an argon-CAP device to explore the effects of CAP irradiation on the growth and expression of oncogenic miRNAs in OSCC. The analysis revealed that, in OSCC cell lines following CAP irradiation, there was a significant reduction in viability; a downregulation of miR-21, miR-31, miR-134, miR-146a, and miR-211 expression; and an inactivation of the v-akt murine thymoma viral oncogene homolog (AKT) and extracellular signal-regulated kinase (ERK) signals. Pretreatment with blockers of apoptosis, autophagy, and ferroptosis synergistically reduced CAP-induced cell death, indicating a combined induction of variable death pathways via CAP. Combined treatments using death inhibitors and miRNA mimics, alongside the activation of AKT and ERK following the exogenous expression, counteracted the cell mortality associated with CAP. The CAP-induced downregulation of miR-21, miR-31, miR-187, and miR-211 expression was rescued through survival signaling. Additionally, CAP irradiation notably inhibited the growth of SAS OSCC cell xenografts on nude mice. The reduced expression of oncogenic miRNAs in vivo aligned with in vitro findings. In conclusion, our study provides new lines of evidence demonstrating that CAP irradiation diminishes OSCC cell viability by abrogating survival signals and oncogenic miRNA expression.
Topics: Humans; Animals; Mice; MicroRNAs; Mouth Neoplasms; Carcinoma, Squamous Cell; Proto-Oncogene Proteins c-akt; Mice, Nude; Squamous Cell Carcinoma of Head and Neck; Head and Neck Neoplasms; Cell Line, Tumor; Cell Proliferation; Gene Expression Regulation, Neoplastic
PubMed: 38068984
DOI: 10.3390/ijms242316662 -
Frontiers in Immunology 2023Good syndrome (GS) is a rare adult-onset immunodeficiency first described in 1954. It is characterized by the coexistence of a thymoma and hypogammaglobulinemia,...
INTRODUCTION
Good syndrome (GS) is a rare adult-onset immunodeficiency first described in 1954. It is characterized by the coexistence of a thymoma and hypogammaglobulinemia, associated with an increased susceptibility to infections and autoimmunity. The classification and management of GS has been long hampered by the lack of data about the underlying immune alterations, a controversy existing on whether it is a unique diagnostic entity . a subtype of Common Variable Immune Deficiency (CVID).
METHODS
Here, we used high-sensitive flow cytometry to investigate the distribution of up to 70 different immune cell populations in blood of GS patients (n=9) compared to age-matched CVID patients (n=55) and healthy donors (n=61).
RESULTS
All 9 GS patients displayed reduced B-cell counts -down to undetectable levels (<0.1 cells/μL) in 8/9 cases-, together with decreased numbers of total CD4 T-cells, NK-cells, neutrophils, and basophils age-matched healthy donors. In contrast, they showed expanded TCRγδ T-cells (p ≤ 0.05). Except for a deeper B-cell defect, the pattern of immune cell alteration in blood was similar in GS and (age-matched) CVID patients. In depth analysis of CD4 T-cells revealed significantly decreased blood counts of naïve, central memory (CM) and transitional memory (TM) TCD4 cells and their functional compartments of T follicular helper (TFH), regulatory T cells (Tregs), T helper (Th)2, Th17, Th22, Th1/Th17 and Th1/Th2 cells. In addition, GS patients also showed decreased NK-cell, neutrophil, basophil, classical monocyte and of both CD1c and CD141 myeloid dendritic cell counts in blood, in parallel to an expansion of total and terminal effector TCRγδ T-cells. Interestingly, those GS patients who developed hypogammaglobulinemia several years after the thymoma presented with an immunological and clinical phenotype which more closely resembled a combined immune humoral and cellular defect, with poorer response to immunoglobulin replacement therapy, as compared to those in whom the thymoma and hypogammaglobulinemia were simultaneously detected.
DISCUSSION
Our findings provide a more accurate definition of the immune cell defects of GS patients and contribute to a better discrimination among GS patients between those with a pure B-cell defect . those suffering from a combined immunodeficiency with important consequences on the diagnosis and management of the disease.
Topics: Adult; Humans; Thymoma; Agammaglobulinemia; Immunologic Deficiency Syndromes; Common Variable Immunodeficiency; Thymus Neoplasms; Primary Immunodeficiency Diseases
PubMed: 38035080
DOI: 10.3389/fimmu.2023.1285088 -
The Medical Journal of Malaysia Nov 2023Ultrasound guided lung biopsy (USLB) is a minimally invasive diagnostic tool with short examination time and real-time monitoring conducted bedside for accurate...
INTRODUCTION
Ultrasound guided lung biopsy (USLB) is a minimally invasive diagnostic tool with short examination time and real-time monitoring conducted bedside for accurate diagnosis in order to provide the best treatment. However, it is not widely performed by pulmonologists. We aim to explicate the efficacy and safety of USLB led by pulmonologists. The objective of this study is to assess safety and efficacy of USLB performed by pulmonologists in an outpatient setting.
MATERIALS AND METHODS
We retrospectively enrolled patients who underwent the procedure from January 2018 to April 2022. Under real time ultrasound (Hitachi Medical ProSound F37), thoracic lesions adjacent to the chest wall were sampled with a full-core biopsy needle (CT Core Single Action Biopsy Device, 18G × 15 cm, Vigeo, Italy). Chest x-ray was performed 30 minutes post procedure ruling out pneumothorax. Patients were discharged home 1-2 hours post biopsy. Data was analysed using Microsoft Excel 2010 and Statistical Package for Social Science (SPSS) Version 26.
RESULTS
A total of 18 patients (14 males, 4 females) underwent USLB for lung tumours. Biopsies were histologically deemed adequate with an overall diagnostic yield of 77.8% (14/18). A total of 57% were positive for thoracic malignancy (21% squamous cell carcinoma, 21% adenocarcinoma, 15% small cell carcinoma) and another 43% were positive for extra thoracic malignancy (1 hepatocellular carcinoma, 2 DLBCL, 1 Hodgkin's lymphoma, 1 seminoma, 1 thymoma). Four patients had inconclusive results but managed to get positive results from surgical or lymph node biopsy (thymoma and adenocarcinoma). Statistical analysis showed more than two passes are needed to achieve a positive HPE yield (p value<0.05). There were nil complications to all the cases done.
CONCLUSIONS
USLB can safely and effectively be performed by trained pulmonologists with excellent accuracy and low complication rate in outpatients.
Topics: Male; Female; Humans; Retrospective Studies; Pulmonologists; Thymoma; Malaysia; Tomography, X-Ray Computed; Lung; Image-Guided Biopsy; Lung Neoplasms; Thoracic Neoplasms; Adenocarcinoma; Thymus Neoplasms; Ultrasonography, Interventional
PubMed: 38031216
DOI: No ID Found -
Respiratory Medicine Case Reports 2023Atypical type A thymomas exhibit more aggressive features than conventional type A thymomas. Type AB thymomas rarely have atypical type A components. We report a rare...
Atypical type A thymomas exhibit more aggressive features than conventional type A thymomas. Type AB thymomas rarely have atypical type A components. We report a rare case of type AB thymoma with an atypical type A component, that was identified after pulmonary metastasectomy 11 years after the primary surgery and long-term follow-up after recurrence. A 61-year-old female underwent extended thymectomy for an anterior mediastinal tumor 11 years prior and was diagnosed with type AB thymoma (Masaoka stage II). Five years ago, follow-up computed tomography showed well-circumscribed pulmonary nodules up to 1.0 cm in both lungs. All the pulmonary nodules grew slowly; however, one of the nodules grew to 1.6 cm, and thoracoscopic wedge resection was performed for diagnosis. Pathologically, the pulmonary nodule was consisted of type A thymoma component. Conventional type AB thymomas are usually locally aggressive neoplasms; thus, we reviewed the tissue slides of primary thymomas. Histologically, cytological atypia, hypercellularity, and increased mitosis are observed in the type A component. Consequently, the diagnosis was revised to a type AB thymoma with an atypical type A component. The pulmonary nodule exhibited the same atypical type A features. Pulmonary metastasectomy was performed two more times as volume-reduction surgery. The residual metastasis was located only in the lung with slow growth, 4 years after the first pulmonary resection; therefore, we followed up as an outpatient without treatment.
PubMed: 38025250
DOI: 10.1016/j.rmcr.2023.101944 -
Thoracic Cancer Jan 2024Multilocular thymic cysts (MTC) are acquired multilocular cysts caused by inflammation. The rarity of such lesions and a lack of recognition make diagnosis and treatment...
Multilocular thymic cysts (MTC) are acquired multilocular cysts caused by inflammation. The rarity of such lesions and a lack of recognition make diagnosis and treatment difficult. Herein, we present our experience with a multilocular mediastinal cyst that resulted in the development of thymic cancer with metastasis over a period of 13 years. Computed tomography findings revealed an anterior mediastinal mass that was suspected to be an MTC in a 49-year-old man. The mass shrank gradually over a period of 7 years; however, growth was observed at 10 years after initial detection. At 13 years after detection, thymic carcinoma with multiple lung metastases was diagnosed. Resection was recommended during the follow-up period, but the patient refused treatment. A multilocular wall and location are factors that indicate MTC. However, even if a definitive diagnosis is not made, resection of multilocular anterior mediastinal cysts should be considered as determining the preoperative diagnosis is difficult. Nevertheless, our case suggests that the coexistence of tumors with cysts is possible, and the potential for malignant tumor development exists.
Topics: Male; Humans; Middle Aged; Mediastinal Cyst; Thymoma; Mediastinum; Lung Neoplasms; Thymus Neoplasms
PubMed: 38018322
DOI: 10.1111/1759-7714.15174 -
BMC Cancer Nov 2023Thymic epithelial tumors (TETs) are the most common primary neoplasms of the anterior mediastinum. Different risk subgroups of TETs have different prognosis and...
BACKGROUND
Thymic epithelial tumors (TETs) are the most common primary neoplasms of the anterior mediastinum. Different risk subgroups of TETs have different prognosis and therapeutic strategies, therefore, preoperative identification of different risk subgroups is of high clinical significance. This study aims to explore the diagnostic efficiency of quantitative computed tomography (CT) parameters combined with preoperative systemic inflammatory markers in differentiating low-risk thymic epithelial tumors (LTETs) from high-risk thymic epithelial tumors (HTETs).
METHODS
74 Asian patients with TETs confirmed by biopsy or postoperative pathology between January 2013 and October 2022 were collected retrospectively and divided into two risk subgroups: LTET group (type A, AB and B1 thymomas) and HTET group (type B2, B3 thymomas and thymic carcinoma). Statistical analysis were performed between the two groups in terms of quantitative CT parameters and preoperative systemic inflammatory markers. Multivariate logistic regression analysis was used to determine the independent predictors of risk subgroups of TETs. The area under curve (AUC) and optimal cut-off values were calculated by receiver operating characteristic (ROC) curves.
RESULTS
47 TETs were in LTET group, while 27 TETs were in HTET group. In addition to tumor size and CT value of the tumor on plain scan, there were statistical significance comparing in CT value of the tumor on arterial phase (CTv-AP) and venous phase (CTv-VP), and maximum enhanced CT value (CE) of the tumor between the two groups (for all, P < 0.05). For systemic inflammatory markers, HTET group was significantly higher than LTET group (for all, P < 0.05), including platelet-to-lymphocyte ratio (PLR), neutrophil-to-lymphocyte ratio (NLR) and systemic immune-inflammation index (SII). Multivariate logistic regression analysis showed that NLR (odds ratio [OR] = 2.511, 95% confidence interval [CI]: 1.322-4.772, P = 0.005), CTv-AP (OR = 0.939, 95%CI: 0.888-0.994, P = 0.031) and CTv-VP (OR = 0.923, 95%CI: 0.871-0.979, P = 0.008) were the independent predictors of risk subgroups of TETs. The AUC value of 0.887 for the combined model was significantly higher than NLR (0.698), CTv-AP (0.800) or CTv-VP (0.811) alone. The optimal cut-off values for NLR, CTv-AP and CTv-VP were 2.523, 63.44 Hounsfeld Unit (HU) and 88.29HU, respectively.
CONCLUSIONS
Quantitative CT parameters and preoperative systemic inflammatory markers can differentiate LTETs from HTETs, and the combined model has the potential to improve diagnostic efficiency and to help the patient management.
Topics: Humans; Thymoma; Retrospective Studies; Thymus Neoplasms; Tomography, X-Ray Computed; Neoplasms, Glandular and Epithelial
PubMed: 38012604
DOI: 10.1186/s12885-023-11332-0 -
Thoracic Cancer Jan 2024Thymic epithelial tumors (TETs) are rare malignant tumors with limited treatment options. No established second-line treatment regimen is available following the...
BACKGROUND
Thymic epithelial tumors (TETs) are rare malignant tumors with limited treatment options. No established second-line treatment regimen is available following the preferred first-line chemotherapy, resulting in unsatisfactory efficacy and poor prognosis for patients with advanced TETs. This study aimed to evaluate the efficacy of small molecule multitarget antiangiogenic inhibitors as well as the prognostic factors for advanced TETs.
METHODS
A retrospective study was conducted using data from a real-world database. Clinical information and survival follow-up data were collected from 52 patients with advanced TETs who received small molecule multitarget antiangiogenic inhibitors at Zhejiang Cancer Hospital between August 10, 2016 and August 10, 2022. The short-term efficacy of the treatments, survival time of the patients, and relevant prognostic factors of advanced TETs were analyzed.
RESULTS
Out of the 52 patients included in this study, 16 had thymoma and 36 had thymic carcinoma. The 52 patients had an overall response rate of 21.1% and a disease control rate of 94.2%. In addition, the median progression-free survival (PFS) was 8.05 months, and the overall survival (OS) was 25.00 months. Apatinib was given to 33 patients, anlotinib to 15 patients, and sunitinib or lenvatinib to four patients. Only seven patients received antiangiogenic inhibitors as their first-line therapy, 27 patients as their second-line therapy, and 18 patients as third-line or subsequent therapy. Meanwhile, 42 patients received monotherapy with an antiangiogenesis inhibitor, while 10 patients received combination therapy. Univariate analysis indicated that the combined treatment was associated with a superior OS (p = 0.044); multivariate analysis indicated that the combined treatment was an independent prognostic factor for PFS (p = 0.014) and OS (p = 0.012).
CONCLUSION
The findings suggest that small molecule multitarget antiangiogenic inhibitors are efficacious as second or post-line treatments as a viable alternative treatment option for patients with advanced TETs.
Topics: Humans; Retrospective Studies; Thymus Neoplasms; Thymoma; Neoplasms, Glandular and Epithelial
PubMed: 38011005
DOI: 10.1111/1759-7714.15167