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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 -
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 -
Medicine Dec 2018Malignant thymoma in the spine is a rare disease without standard curative managements so far. The objective of this article is to report a very rare case of recurrent... (Review)
Review
RATIONALE
Malignant thymoma in the spine is a rare disease without standard curative managements so far. The objective of this article is to report a very rare case of recurrent malignant thymoma with sacrum metastases causing severe lumbosacral pain, which was presented with acute radiculopathy and treated with 2 operations combined with stabilization and cement augmentation. The management of these unique cases is not well-documented.
PATIENT CONCERNS
A 75-year-old man presented with lumbosacral pain, radiating pain and numbness of the left extremity. The patient underwent thymectomy in 2008, and posterior spinal cord decompression, tumor resection and a stabilization procedure in 2011. Pathologic results confirmed malignant thymomas of the spine. Imaging studies revealed the density of soft tissues, obvious bony destruction in the sacrum, and significant spinal cord obstruction.
DIAGNOSES
We believe this is a less-documented case of metastatic thymoma of the sacral spine presenting with back pain and radiculopathy, and presenting as a giant solid tumor.
INTERVENTIONS
The patient underwent osteoplasty via a posterior approach. Pathologic results confirmed malignant thymomas of the sacral spine.
OUTCOMES
The patient's neurological deficits improved significantly after the surgery, and the postoperative period was uneventful at the 6-month and 1-year follow-up visit. There were no other complications associated with the operation during the follow-up period.
LESSONS
This article emphasizes metastatic thymoma of the spine, although rare, should be part of the differential when the patient presents with back pain and radiculopathy. We recommend the posterior approach for spinal decompression of the metastatic thymoma when the tumor has caused neurological deficits. Osteoplasty by cement augmentation is also a good choice for surgical treatment.
Topics: Aged; Humans; Low Back Pain; Magnetic Resonance Imaging; Male; Sacrum; Spinal Neoplasms; Thymoma; Thymus Neoplasms; Whole Body Imaging
PubMed: 30572538
DOI: 10.1097/MD.0000000000013796 -
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 -
Oncology (Williston Park, N.Y.) Oct 2012Thymomas are unusual tumors, representing no more than 1% of all malignancies. However, thymomas are the most common epithelial tumors of the anterior mediastinum....
Thymomas are unusual tumors, representing no more than 1% of all malignancies. However, thymomas are the most common epithelial tumors of the anterior mediastinum. Unfortunately, there is no general agreement regarding the best parameters to use to predict clinical behavior in these tumors.This review considers the status of the different histological classifications thus far presented for thymomas and offers an analysis of the association between histology and clinical behavior. It also emphasizes the importance of proper staging of thymomas, delineating the benefits and shortcomings of different proposed staging systems and offering thoughts on a better and more accurate staging stratification for patients with these tumors. All of the different parameters are presented in relation to survival rates. Based on current information, staging with proper stratification remains the most important parameter for predicting prognosis. For tumors limited to the mediastinal compartment, surgical resection is the most effective treatment, while induction therapy is a good alternative for patients in whom surgical resection is not possible.
Topics: Clinical Trials as Topic; Combined Modality Therapy; Humans; Neoplasm Staging; Prognosis; Thymoma; Thymus Neoplasms
PubMed: 23176011
DOI: No ID Found -
The Journal of Medical Investigation :... Feb 2008Thymoma is the most common tumor of the anterior mediastinum. This tumor is associated with unique paraneoplastic syndromes (myasthenia gravis, pure red cell aplasia,... (Review)
Review
Thymoma is the most common tumor of the anterior mediastinum. This tumor is associated with unique paraneoplastic syndromes (myasthenia gravis, pure red cell aplasia, hypogammaglobulinemia, and other autoimmune diseases). The rarity of this tumor has somewhat obscured the optimal treatment. Although the histologic classification of thymoma has remained a subject of controversy for many years, the WHO classification system, published in 1999, appeared to be an advance in our understanding of thymoma. The optimal treatment for thymoma depends on its clinical stage. Surgery remains the mainstay of treatment for thymic epithelial tumors. Thymomas also have a high response rate to chemotherapy or radiotherapy. Only surgical resection is performed for patients with stage I (non-invasive) thymoma. The value of postoperative radiotherapy in completely resected stage II or III tumors is questionable. Multimodality therapy involving surgery, chemotherapy and radiotherapy appears to increase the rate of complete resection and survival in advanced (stage III and IV) thymomas.
Topics: Antineoplastic Agents; Chemotherapy, Adjuvant; Cisplatin; Combined Modality Therapy; Humans; Neoplasm Staging; Prognosis; Radiotherapy Dosage; Radiotherapy, Adjuvant; Thymectomy; Thymoma; Thymus Neoplasms; World Health Organization
PubMed: 18319541
DOI: 10.2152/jmi.55.17 -
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 -
Thoracic Cancer Jun 2021Thymomas are the most common type of anterior mediastinal tumors. Calcification is sometimes observed in thymomas using computed tomography (CT), and it is more frequent...
BACKGROUND
Thymomas are the most common type of anterior mediastinal tumors. Calcification is sometimes observed in thymomas using computed tomography (CT), and it is more frequent in invasive thymomas than in noninvasive thymomas. However, the significance of calcification in thymomas remains unknown. This study aimed to evaluate the significance of calcification in thymomas on invasiveness to surrounding organs and investigate the characteristics of thymoma cases with calcification at our institution.
METHODS
We included thymoma patients treated at our institution between 2000 and 2016, and evaluated their characteristics, including demographics, calcification on CT, histology, Masaoka stage, and myasthenia gravis status. The patients were categorized into calcification (C) and noncalcification (NC) groups.
RESULTS
Among 51 included patients, 11 (21.6%) had calcification. A higher proportion of group C patients had World Health Organization histological type B2 and B3 tumors (high-risk) than type A, AB, and B1 tumors (low-risk; p = 0.0477). The number of patients with Masaoka stages III and IV were significantly higher in the C group than in the NC group (p < 0.0001). The C group patients had significantly higher rates of invasion to the mediastinal pleura, pericardium, lung, phrenic nerve, and chest wall and pleural dissemination than the NC group patients.
CONCLUSIONS
Calcification reflects invasiveness of tumors to surrounding organs and tissues, and may thus predict thymoma stage and histologically high-risk thymomas. Calcification in thymomas may also predict the pathological stage and help decide therapeutic methods and surgical approaches to treat thymomas based on the calcification status according to CT findings.
Topics: Calcinosis; Female; Humans; Male; Middle Aged; Neoplasm Invasiveness; Thymoma
PubMed: 33955164
DOI: 10.1111/1759-7714.13964 -
Scientific Reports Feb 2023To determine the prognostic CT features in patients with untreated thymic epithelial tumors (TETs). Clinical data and CT imaging features of 194 patients with...
To determine the prognostic CT features in patients with untreated thymic epithelial tumors (TETs). Clinical data and CT imaging features of 194 patients with pathologically confirmed TETs were retrospectively reviewed. The subjects included 113 male and 81 female patients between 15 and 78 years of age, with a mean age of 53.8 years. Clinical outcomes were categorized according to whether relapse, metastasis or death occurred within 3 years after the first diagnosis. Associations between clinical outcomes and CT imaging features were determined using univariate and multivariate logistic regression analyses, while the survival status was analyzed by Cox regression. In this study, we analyzed 110 thymic carcinomas, 52 high-risk thymomas and 32 low-risk thymomas. Percentages of poor outcome and patient death in thymic carcinomas were much higher than those in patients with high-risk and low-risk thymomas. In the thymic carcinomas groups, 46 patients (41.8%) experienced tumor progression, local relapse or metastasis and were categorized as having poor outcomes; vessel invasion and pericardial mass were confirmed to be independent predictors by logistic regression analysis (p < 0.01). In the high-risk thymoma group, 11 patients (21.2%) were categorized as having poor outcomes, and the CT feature pericardial mass was confirmed to be an independent predictor (p < 0.01). In survival analysis, Cox regression showed that CT features of lung invasion, great vessel invasion, lung metastasis and distant organ metastasis were independent predictors for worse survival in the thymic carcinoma group (p < 0.01), while lung invasion and pericardial mass were independent predictors for worse survival in high-risk thymoma group. No CT features were related to poor outcome and worse survival in the low-risk thymoma group. Patients with thymic carcinoma had poorer prognosis and worse survival than those with high-risk or low-risk thymoma. CT can serve as an important tool for predicting the prognosis and survival of patients with TETs. In this cohort, CT features of vessel invasion and pericardial mass were related to poorer outcomes in those with thymic carcinoma and pericardial mass in those with high-risk thymoma. Features including lung invasion, great vessel invasion, lung metastasis and distant organ metastasis indicate worse survival in thymic carcinoma, whereas lung invasion and pericardial mass indicate worse survival in high-risk thymoma.
Topics: Humans; Male; Female; Middle Aged; Thymoma; Prognosis; Retrospective Studies; Neoplasm Recurrence, Local; Thymus Neoplasms; Neoplasms, Glandular and Epithelial; Lung Neoplasms
PubMed: 36801902
DOI: 10.1038/s41598-023-30041-z -
Journal of Thoracic Oncology : Official... Oct 2010Thirty years have gone by since the Masaoka staging system of thymoma was proposed in 1981. Although the Masaoka staging system has been accepted by many surgeons and... (Review)
Review
INTRODUCTION
Thirty years have gone by since the Masaoka staging system of thymoma was proposed in 1981. Although the Masaoka staging system has been accepted by many surgeons and pathologists, some proposals of revision and improvements have been suggested. At this time, I reinvestigated the Masaoka staging system based on the recent follow-up study of the thymomas resected at Nagoya City University.
METHODS
Using the follow-up results of 211 thymomas in Nagoya, I analyzed the following aspects: (1) evaluation of the Masaoka staging system as a prognostic factor in the Nagoya series and (2) critical assessment of the proposals of revision to the Masaoka staging system.
RESULTS
(1) Univariate analysis showed that Masaoka stages were significantly prognostic for overall survival (p < 0.0001). (2) The difference of survivals between stage I and II was not significant, but progression-free survival of stage I was 100% for up to 20 years, whereas one tumor death case in stage II was found. (3) Differences of survival between the cases with and without great vessel invasion in stage III were not significant. (4) Prognosis of N tumors was yet better defined.
CONCLUSION
(1) The Masaoka staging system remains a valuable prognostic factor. (2) Combination of stage I with II and separation of stage III into subgroups are not recommended. (3) At the moment, it is better to include N tumors in stage IVb.
Topics: Humans; Neoplasm Staging; Prognosis; Thymoma; Thymus Neoplasms
PubMed: 20859124
DOI: 10.1097/JTO.0b013e3181f20c05