-
Radiology and Oncology Mar 2023Thymomas belong to rare tumors giving rise to thymic epithelial tissue. There is a classification of several forms of thymoma: A, AB, B1, B2, B3, thymic carcinoma (TC)... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Thymomas belong to rare tumors giving rise to thymic epithelial tissue. There is a classification of several forms of thymoma: A, AB, B1, B2, B3, thymic carcinoma (TC) and thymic neuroendocrine thymoma. In this meta-analysis study, we have focused on thymoma using articles based on the disease's next-generation sequencing (NGS) genomic profiling.
MATERIALS AND METHODS
We conducted a systematic review and meta-analysis of the prevalence of studies that discovered the genes and variants occurring in the less aggressive forms of the thymic epithelial tumors. Studies published before 12 December 2022 were identified through PubMed, Web of Science (WoS), and SCOPUS databases. Two reviewers have searched for the bases and selected the articles for the final analysis, based on well-defined exclusion and inclusion criteria.
RESULTS
Finally, 12 publications were included in the qualitative as well as quantitative analysis. The three genes, , , and , emerged as disease-significant in the observed studies. The Odds Ratio for all three extracted genes (OR = 1.58, CI [1.51, 1.66] p < 0.00001) (OR = 1.36, CI [1.12, 1.65], p < 0.002), and (OR = 1.02, CI [1.00, 1.04], p < 0.001).
CONCLUSIONS
According to obtained data, we noticed that the gene exhibits a significant prevalence in the cohort of observed thymoma patients. Moreover, analyzing published articles NGS has suggested , and genes as the most frequently mutated genes in thymoma that have pathogenic single nucleotide variants (SNV) and Insertion/Deletion (InDel), which contribute to disease development and progression. These variants could be valuable biomarkers and target points specific to thymoma.
Topics: Humans; Thymoma; Thymus Neoplasms; Neoplasms, Glandular and Epithelial; High-Throughput Nucleotide Sequencing; Transcription Factors, TFIII
PubMed: 36942904
DOI: 10.2478/raon-2023-0013 -
Cancer Reports (Hoboken, N.J.) Mar 2023Thymic epithelial tumors are rare and include thymomas and thymic carcinomas. There is scarce literature characterizing prognostic factors and long-term outcomes in... (Review)
Review
BACKGROUND
Thymic epithelial tumors are rare and include thymomas and thymic carcinomas. There is scarce literature characterizing prognostic factors and long-term outcomes in these tumors.
AIMS
This review aims to describe disease features of thymomas and thymic carcinomas and to report clinical differences among thymoma histological subtypes.
METHODS AND RESULTS
A retrospective chart review was performed at the University of Florida Shands Hospital, a tertiary care academic medical center in Gainesville, Florida, USA. The review included clinical data of adults with thymic epithelial tumors diagnosed between 2001 and 2021. Significant associations among demographics, histology, stage, and outcomes were investigated. Thymoma subgroup analysis was performed using histological subtype and sex. Forty patients with thymoma and seven patients with thymic carcinoma were included in the final analysis. Among those with thymomas, patients with subtype B1, B2, or B3 tumors were younger, had larger tumors, and presented with higher stage disease when compared to those with subtypes A or AB. Tumor recurrence was most common in subtype B2 and B3 tumors (50.0% and 16.7% vs. 0%; p < .01). However, there was no significant difference in overall survival between histologic subtypes. Compared to females, males with thymomas had superior overall survival (103.0 vs. 62.9 months; p = .021) despite presenting with larger tumors (9.8 vs. 5.8 cm; p = .041). Concomitant myasthenia gravis was associated with increased recurrence but not worsened mortality. Compared to thymomas, patients with thymic carcinoma presented with higher-stage disease and had poorer 5-year survival (50.0% vs. 93.1%; p < .01).
CONCLUSION
This study affirmed pathologic stage and resectability as prognostic factors for thymic epithelial tumors. New findings include inferior overall survival in female patients and higher recurrence rates in those with thymomas and concomitant myasthenia gravis.
Topics: Adult; Male; Humans; Female; Thymoma; Prognosis; Retrospective Studies; Neoplasm Recurrence, Local; Thymus Neoplasms; Neoplasms, Glandular and Epithelial; Myasthenia Gravis
PubMed: 36369906
DOI: 10.1002/cnr2.1750 -
Acta Haematologica 2022The co-occurrence of thymoma and T-lymphoblastic lymphoma/leukemia is an extremely rare but previously reported association that poses a diagnostic and therapeutic... (Review)
Review
The co-occurrence of thymoma and T-lymphoblastic lymphoma/leukemia is an extremely rare but previously reported association that poses a diagnostic and therapeutic challenge. We describe a 67-year-old patient with long-standing untreated B1 thymoma that presented with constitutional symptoms and a painless soft tissue mass on the right chest wall. Pathological analysis of the biopsy from the mass demonstrated T-lymphoblastic leukemia/lymphoma. The patient went through a complicated course, was refractory to several lines of therapy, and eventually underwent allogeneic hematopoietic stem cell transplantation in complete remission from a matched related donor. The association between thymoma and malignant neoplasms has been described in the literature, most notably with colorectal adenocarcinoma and thyroid cancer. Thymoma-associated leukemia is, however, extremely unusual, with limited reports in the literature. Distinguishing between thymoma and leukemia can be challenging and often requires meticulous diagnostic efforts. For patients with a past history of thymoma, awareness of this particular association should be bared in mind to allow earlier diagnosis and therapy.
Topics: Aged; Allografts; Biopsy; Hematopoietic Stem Cell Transplantation; Humans; Male; Neoplasms, Second Primary; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma; Thymoma; Thymus Neoplasms
PubMed: 34537764
DOI: 10.1159/000519033 -
Acta Neurologica Belgica Feb 2020Management of myasthenia gravis (MG) in the presence of comorbidities may be difficult. We report the effect of comorbidities in the outcome of MG. The patients with MG...
Management of myasthenia gravis (MG) in the presence of comorbidities may be difficult. We report the effect of comorbidities in the outcome of MG. The patients with MG during 1991-2016 were included and evaluated including their demographic variables, clinical findings, Myasthenia Gravis Foundation of America (MGFA) score. The patients were categorized into early onset (≤ 40 years) and late onset (> 40 years) MG. The comorbidities (autoimmune and miscellaneous) and iatrogenic complications were compared between early and late onset, and in good and poor outcome groups. Out of 81 patients with MG, 48 patients had early and 33 late onset. In 71 (88%) patients, comorbidities were present and were autoimmune in 8 (10%) and miscellaneous in all the patients (88%). Iatrogenic complications were present in 54 (67%) patients. Thymectomy was done in 19 patients; 16 had thymoma and 3 thymic hyperplasia. Myasthenic crisis occurred in 28 patients; 5 (18%) had autoimmune and all had miscellaneous comorbidities. The patients with poor outcome had ≥ 2 comorbidities, myasthenic crisis, leukocytosis, elevated serum bilirubin and creatinine, and increased number of hospital admissions (P < 0.05). Myasthenia gravis is associated with comorbidities in majority of patients especially in late onset group, and more than two comorbidities are related to poor outcome.
Topics: Adolescent; Adult; Age of Onset; Aged; Autoimmune Diseases; Child; Comorbidity; Female; Humans; Iatrogenic Disease; Male; Middle Aged; Myasthenia Gravis; Outcome Assessment, Health Care; Thymectomy; Thymoma; Thymus Hyperplasia; Thymus Neoplasms; Young Adult
PubMed: 30972663
DOI: 10.1007/s13760-019-01102-w -
Pathologica Oct 2021In this study, the authors report on the activity of Juan Rosai, one of the pathologists most engaged in the definition of cells, diseases and tumors occurring in the... (Review)
Review
In this study, the authors report on the activity of Juan Rosai, one of the pathologists most engaged in the definition of cells, diseases and tumors occurring in the thymus and in the mediastinum during the last 60 years. With his morphological skills and tireless interest in clarification of disease patterns, he contributed extraordinarily to expand our knowledge of the mediastinal diseases and to improve our diagnostic approach. He determined extraordinary advances also in trasmission electron microscopy and in immunohistochemistry as powerful diagnostic tools. Moreover, he proposed and promoted, together with an international panel of Pathologists, the World Health Classification of Thymic tumors as a definite progress in our comprehension and diagnostics of thymic epithelial tumors (TET). Our purpose is to review J. Rosai's achievements in thymic normal structure, in TET and particularly in the entity now definied as "thymoma", in distinction from the thymic carcinoma. To do this, our narrative will also be based on personal memories, longstanding collaborations and/or friendship with J. Rosai.
Topics: Humans; Immunohistochemistry; Male; Mediastinum; Neoplasms, Glandular and Epithelial; Thymoma; Thymus Neoplasms
PubMed: 34837094
DOI: 10.32074/1591-951X-539 -
Cancers Apr 2022Thymic epithelial neoplasms are rare tumors that constitute the majority of anterior mediastinal masses. They are classified as thymomas, thymic carcinomas, and thymic... (Review)
Review
Thymic epithelial neoplasms are rare tumors that constitute the majority of anterior mediastinal masses. They are classified as thymomas, thymic carcinomas, and thymic neuroendocrine neoplasms. Biopsy diagnosis is not common, and most tumors are surgically resected. Biopsy, including cytology, is indicated when a non-surgical entity is suspected or in cases of locally advanced disease. Smears of thymomas consist of round or spindle epithelial cells admixed with varying amounts of lymphocytes depending on the type of thymoma. Smears of thymic carcinoma and thymic neuroendocrine neoplasms are often indistinguishable from corresponding tumor types from other organs. Accurate cytological diagnosis can be difficult due to the histological diversity of thymomas, as well as the morphological features that certain thymic tumors share with similar tumors from other organs. However, fine needle aspiration (FNA) of anterior mediastinal masses can provide clinically actionable information and can be used to determine whether lesions require surgical, systemic, or local noninvasive treatments. Ancillary studies, namely, immunocytochemical stains, flow cytometry, and radiology, are important tools in the evaluation of thymic aspirates. This review discusses the utility and limitations of thymic FNAs and illustrates the diagnostic features and pitfalls of these specimens.
PubMed: 35454918
DOI: 10.3390/cancers14082013 -
World Journal of Surgical Oncology Jul 2023Thymoma is the most common primary tumor in the anterior mediastinum. The prognostic factors of patients with thymoma still need to be clarified. In this study, we aimed...
PURPOSE
Thymoma is the most common primary tumor in the anterior mediastinum. The prognostic factors of patients with thymoma still need to be clarified. In this study, we aimed to investigate the prognostic factors of patients with thymoma who received radical resection and establish the nomogram to predict the prognosis of these patients.
MATERIALS AND METHODS
Patients who underwent radical resection for thymoma with complete follow-up data between 2005 and 2021 were enrolled. Their clinicopathological characteristics and treatment methods were retrospectively analyzed. Progression-free survival (PFS) and overall survival (OS) were estimated using the Kaplan-Meier method and compared by the log-rank test. Univariate and multivariate Cox proportional hazards regression analyses were performed to identify the independent prognostic factors. According to the results of the univariate analysis in the Cox regression model, the predictive nomograms were created.
RESULTS
A total of 137 patients with thymoma were enrolled. With a median follow-up of 52 months, the 5-year and 10-year PFS rates were 79.5% and 68.1%, respectively. The 5-year and 10-year OS rates were 88.4% and 73.1%, respectively. Smoking status (P = 0.022) and tumor size (P = 0.039) were identified as independent prognostic factors for PFS. Multivariate analysis showed that a high level of neutrophils (P = 0.040) was independently associated with OS. The nomogram showed that the World Health Organization (WHO) histological classification contributed more to the risk of recurrence than other factors. Neutrophil count was the most important predictor of OS in patients with thymoma.
CONCLUSION
Smoking status and tumor size are risk factors for PFS in patients with thymoma. A high level of neutrophils is an independent prognostic factor for OS. The nomograms developed in this study accurately predict PFS and OS rates at 5 and 10 years in patients with thymoma based on individual characteristics.
Topics: Humans; Thymoma; Prognosis; Retrospective Studies; Thymus Neoplasms; World Health Organization
PubMed: 37430268
DOI: 10.1186/s12957-023-03068-9 -
Contrast Media & Molecular Imaging 2022The values of machine learning-based computed tomography (CT) imaging in histological classification and invasion prediction of thymoma were investigated.
PURPOSE
The values of machine learning-based computed tomography (CT) imaging in histological classification and invasion prediction of thymoma were investigated.
METHODS
181 patients diagnosed with thymoma by surgery or biopsy in Shantou Central Hospital between February 2017 and March 2022 were selected. According to the concept of simplified histological classification and the latest histological classification by the WHO, thymoma was divided into two groups, including low-risk (types A, AB, B1, and metaplastic type) and high-risk groups (types B2 and B3). CT images were reconstructed by filtering back projection (FBP) algorithm. CT image features were collected for statistical analysis.
RESULTS
The main symptoms of patients diagnosed with thymoma included respiratory tract infection, chest distress and shortness of breath, and chest pain. 35.91% of them suffered from complicated myasthenia gravis. Tumor size and position in low-risk and high-risk groups showed no statistical significance ( > 0.05). Tumor morphology and boundary between the two groups suggested statistical difference ( < 0.05). Whether tumor invaded adjacent tissues was apparently correlated with simplified histological classification ( < 0.01). The sensitivity and specificity of CT images for the invasion of mediastinal pleura or pericardium were around 90% and negative predictive values both reached above 95%. Those of the CT images for lung invasion were over 80%. The negative and positive predictive values were 93.54% and 63.82%, respectively. Those of the CT images for blood vessel invasion were 67.32% and 97.93%. The negative and positive predictive values were 98.21% and 83%, respectively.
CONCLUSION
The machine learning-based CT image had significant values in the prediction of different histological classification and even invasion level.
Topics: Humans; Machine Learning; Sensitivity and Specificity; Thymoma; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 36051922
DOI: 10.1155/2022/4594757 -
ESMO Open Oct 2023Thymic epithelial tumors (TETs) are rare neoplasms arising in the mediastinum, including thymic carcinomas and thymomas. Due to their rarity, little is known about the...
BACKGROUND
Thymic epithelial tumors (TETs) are rare neoplasms arising in the mediastinum, including thymic carcinomas and thymomas. Due to their rarity, little is known about the genomic profiles of TETs. Herein, we investigated the genomic characteristics of TETs evaluated in a large comprehensive genomic profiling database in a real-world setting.
METHODS
We included data from two different cohorts: Foundation Medicine Inc. (FMI) in the United States and the Center for Cancer Genomics and Advanced Therapeutics (C-CAT) in Japan. Samples profiled were examined for all classes of alterations in 253 genes targeted across all assays. Tumor mutational burden (TMB) and microsatellite instability (MSI) were also evaluated.
RESULTS
A total of 794 patients were collected in our study, including 722 cases from FMI and 72 cases from C-CAT. In the FMI data, CDKN2A (39.9%), TP53 (30.2%) and CDKN2B (24.6%) were frequently altered in thymic carcinoma, versus TP53 (7.8%), DNMT3A (6.8%), and CDKN2A (5.8%) in thymoma. TMB-high (≥10 mutations/Mb) and MSI were present in 7.0% and 2.3% of thymic carcinomas, and 1.6% and 0.3% of thymomas, respectively. Within C-CAT data, CDKN2A (38.5%), TP53 (36.5%) and CDKN2B (30.8%) were also frequently altered in thymic carcinoma, while alterations of TSC1, SETD2 and LTK (20.0% each) were found in thymoma.
CONCLUSIONS
To the best of our knowledge, this is the largest cohort in which genomic alterations, TMB and MSI status of TETs were investigated. Potential targets for treatment previously unbeknownst in TETs are identified in this study, entailing newfound opportunities to advance therapeutic development.
Topics: Humans; Thymoma; Thymus Neoplasms; Neoplasms, Glandular and Epithelial; Genomics
PubMed: 37703595
DOI: 10.1016/j.esmoop.2023.101627 -
Aging Jun 2022Immune microenvironment and microRNAs serve as common predictors for diagnosis and prognosis of tumors.
INTRODUCTION
Immune microenvironment and microRNAs serve as common predictors for diagnosis and prognosis of tumors.
METHODS
Expression of 122 genes and 126 microRNAs in thymoma was obtained from TCGA database. The proportion of tumor-infiltrating cells was calculated, and IMRS was constructed. TREM2hi score was calculated before functional enrichment analysis on gene sets.
RESULTS
IMRS3, TREM2hi score, and CD8+ T lymphocyte abundance were significantly different among WHO classifications. WHO classification, Masaoka staging, and miR-130b-5p, miR-1307-3p, miR-425-5p, CD8, CD68, and CCL18 expression were prognostic factors for relapse-free survival and overall survival. IMRS3 upregulation polarized macrophages into M2, which rejected CD8+ T and other effector lymphocytes to promote thymoma malignant progression.
CONCLUSIONS
BRRS may present a novel immune-related microRNA signature for TET prognosis.
Topics: Biomarkers, Tumor; Gene Expression Regulation, Neoplastic; Humans; MicroRNAs; Neoplasm Recurrence, Local; Prognosis; Thymoma; Thymus Neoplasms; Tumor Microenvironment
PubMed: 35675033
DOI: 10.18632/aging.204108