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Endocrine Nov 2023Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome that combines endocrine and non-endocrine tumors. Thymic neuroendocrine tumors are uncommon components that...
BACKGROUND
Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome that combines endocrine and non-endocrine tumors. Thymic neuroendocrine tumors are uncommon components that predict poor prognosis in patients with MEN1. We aimed to summarize the clinical characteristics of thymoma in MEN1 by reviewing the current reports from the literature.
METHODS
A patient with multiple endocrine neoplasia type 1 (parathyroid hyperplasia, pituitary adenoma, and insulinoma) was found to have a 2 × 1.5 cm thymic mass during long-term follow-up. Thoracoscope surgery was performed, and a histopathology examination revealed WHO Type B3 thymoma. A pathogenic mutation of c.783 + 1G > A in the MEN1 gene was identified. We further searched PubMed and EMBASE for thymoma in association with MEN1.
RESULTS
A comprehensive overview of the literature concerning characteristics of MEN1-related thymoma was summarized. Clinical characteristics and differences between thymoma and thymic carcinoid are highlighted.
CONCLUSIONS
Besides carcinoid, other tumors, including thymoma, need to be identified for thymic space-occupying lesions in MEN1 patients. The impact of thymoma on the long-term prognosis of MEN1 patients needs further investigation.
Topics: Humans; Multiple Endocrine Neoplasia Type 1; Thymoma; Thymus Neoplasms; Carcinoid Tumor; Pancreatic Neoplasms
PubMed: 37668926
DOI: 10.1007/s12020-023-03440-5 -
European Journal of Surgical Oncology :... Dec 2007This article reviews the literature on the clinical features, diagnosis and management of primary intrapulmonary thymoma. (Review)
Review
AIM
This article reviews the literature on the clinical features, diagnosis and management of primary intrapulmonary thymoma.
METHODS
Medline, Embase and Cochrane Library searches were performed on all relevant Anglo-Saxon language articles. The search words included "primary pulmonary thymoma" and "intrapulmonary thymoma". Secondary references were obtained from key articles. Prognostic and treatment strategies were analyzed by the Kaplan-Meier method, comparisons between curves were made using log rank test.
RESULTS
The searches yielded 25 cases of primary intrapulmonary thymoma. Median follow-up was 9 months (1 day to 13 years). At follow-up, 14 patients were tumor free, one patient had a local recurrence 8 years after radiotherapy, one patient responded favorably to radiotherapy, six patients died and three patients were lost to follow-up. The presence of a paraneoplastic syndrome decreased survival (P=0.02), however, histological subgroup (P=0.216), clinical stage (P=0.63) and tumor size (P=0.288) did not affect survival. Survival in surgically managed patients was significantly better than in conservatively managed patients (P=0.039). Adjuvant radiotherapy did not provide any benefit (P=0.4).
CONCLUSION
Complete resection of primary intrapulmonary thymomas appears sufficient in non-malignant tumors. Because of the risk of late local recurrence, long-term regular clinical follow-up is warranted.
Topics: Adolescent; Adult; Aged; Choristoma; Female; Humans; Lung Neoplasms; Male; Middle Aged; Pneumonectomy; Prognosis; Radiotherapy; Thymoma; Thymus Neoplasms
PubMed: 17442530
DOI: 10.1016/j.ejso.2007.02.033 -
Frontiers in Oncology 2022Thymomas are derived from the epithelial component of the thymus and constitute the most common tumor of the anterior mediastinum. These neoplasms are considered...
Thymomas are derived from the epithelial component of the thymus and constitute the most common tumor of the anterior mediastinum. These neoplasms are considered malignant for their potential for invasion and metastases. Several histopathologic subclassification schemes have been proposed over the years, however, correlation of histotypes with prognosis remains controversial. In contrast, studies invariably have shown that staging and resection status correlate with oncologic behavior and disease outcomes. In this regard, several staging systems have been presented, though transcapsular invasion and degree of involvement of adjacent anatomic structures are common denominators of all schemes. Involvement of the great vessels and heart most commonly results from direct invasion, which may lead to unusual clinical presentations such as superior vena cava syndrome. Moreover, intravascular and intracardiac growth with or without direct mural invasion rarely occurs. We provide an overview of thymomas with intravascular and intracardiac involvement.
PubMed: 35814455
DOI: 10.3389/fonc.2022.881553 -
Journal of Thoracic Oncology : Official... Nov 2022The aim of this guideline was to provide recommendations for the most effective therapy for patients with thymic epithelial tumors, including thymoma, thymic carcinoma,... (Review)
Review
INTRODUCTION
The aim of this guideline was to provide recommendations for the most effective therapy for patients with thymic epithelial tumors, including thymoma, thymic carcinoma, and thymic neuroendocrine tumors (NETs). This guideline is intended to be used by all health care professionals managing patients with thymic epithelial tumors.
METHODS
The guideline was developed by Ontario Health (Cancer Care Ontario)'s Program in Evidence-Based Care and by the Lung Cancer Disease Site Group through a systematic review of the evidence, expert consensus, and formal internal and external reviews.
RESULTS
Evidence-based recommendations were developed to improve the management of patients with thymic epithelial tumors. The guideline includes recommendations for surgical, radiation, and systemic treatments for patients with thymoma, thymic carcinoma, and thymic NETs separated by stage of disease using the TNM staging system. Recommendations for patients with thymic NETs were endorsed from the 2021 National Comprehensive Cancer Network Neuroendocrine and Adrenal Tumors Guideline.
CONCLUSIONS
This guideline reflects the new staging system for patients with thymoma and thymic carcinoma and includes supporting evidence from the best available studies.
Topics: Humans; Thymoma; Lung Neoplasms; Thymus Neoplasms; Neoplasms, Glandular and Epithelial; Neoplasm Staging; Neuroendocrine Tumors; Retrospective Studies
PubMed: 36031176
DOI: 10.1016/j.jtho.2022.08.007 -
Thoracic Cancer Jan 2022Surgical resection of the thymus is indicated in the presence of primary thymic diseases such as thymoma. Video-assisted thoracoscopic surgery (VATS) and robot-assisted... (Comparative Study)
Comparative Study Meta-Analysis
BACKGROUND
Surgical resection of the thymus is indicated in the presence of primary thymic diseases such as thymoma. Video-assisted thoracoscopic surgery (VATS) and robot-assisted thoracic surgery (RATS) offer a minimally invasive approach to thymectomy. However, there is no clear conclusion whether RATS can achieve an equal or even better surgical effect when compared with VATS in treatment of thymoma. We performed this meta-analysis to explore and compare the outcomes of RATS versus VATS for thymectomy in patients with thymoma.
METHODS
PubMed, Cochrane Library, EMBASE, China National Knowledge Infrastructure (CNKI), Medline, and Web of Science databases were searched for full-text literature citations. The quality of the articles was evaluated using the Newcastle-Ottawa Scale and the data analyzed using Review Manager 5.3 software. Fixed or random effect models were applied according to heterogeneity. Subgroup analysis was conducted.
RESULTS
A total of 11 studies with 1418 patients, of whom 688 patients were in the RATS group and 730 in the VATS group, were involved in the analysis. Compared with VATS, RATS was associated with less blood loss in operation, lower volume of drainage, fewer postoperative pleural drainage days, shorter postoperative hospital stay, and fewer postoperative complications. There was no significant difference in operative time and patients with or without myasthenia gravis between the two groups.
CONCLUSIONS
RATS has more advantages over VATS, indicating that RATS is better than VATS in terms of postoperative recovery. We look forward to more large-sample, high-quality randomized controlled studies published in the future.
Topics: Humans; Robotic Surgical Procedures; Thoracic Surgery, Video-Assisted; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 34806328
DOI: 10.1111/1759-7714.14234 -
The Cochrane Database of Systematic... Aug 2013Thymic carcinoma or advanced thymoma is a rare cancer of the thymus gland that tends to be aggressive and infiltrate neighbouring organs, making total resection very... (Review)
Review
BACKGROUND
Thymic carcinoma or advanced thymoma is a rare cancer of the thymus gland that tends to be aggressive and infiltrate neighbouring organs, making total resection very difficult. Induction or adjuvant chemotherapy, or both, are often used in a multimodality approach to treat people affected by this condition, but the effectiveness of chemotherapy for thymic carcinoma or advanced thymoma remains uncertain.
OBJECTIVES
To assess the role of chemotherapy in adults with thymic carcinoma or advanced thymoma.
SEARCH METHODS
We searched the Cochrane Central Register of Controlled Trials (CENTRAL 2012, Issue 7), MEDLINE (accessed via Ovid from 1966 to July 2012), EMBASE (accessed via Ovid, from 1980 to July 2012), Latin American and Caribbean Literature on Health Sciences (LILACS), the Chinese Biological Medicine Database (CBM, 1978 to July 2012), China National Knowledge Infrastructure (CNKI, 1980 to July 2012) and the Chinese scientific periodical database VIP Information (VIP, 1989 to July 2012). There was no language restriction in searching for studies.
SELECTION CRITERIA
We planned to include randomised controlled trials (RCTs) of trials using chemotherapy (either single-agent or combination chemotherapy plus surgery, radiotherapy or not) for thymic carcinoma and/or advanced thymoma. We planned to include all adults (aged 18 years and over) diagnosed with thymic carcinoma and/or with Masaoka stage III or IV thymic tumours. The intended primary outcomes were overall survival (OS) and progression-free survival (PFS).
DATA COLLECTION AND ANALYSIS
Two review authors independently evaluated the search results according to the inclusion and exclusion criteria. There were no studies identified for inclusion and therefore no data extraction was completed.
MAIN RESULTS
No RCTs were eligible for inclusion in this review. We report details of excluded prospective studies in an additional table and try to provide some useful evidence regarding current practice.
AUTHORS' CONCLUSIONS
There were no RCTs eligible for inclusion in this review. In current practice the most common regimen for adult patients with thymic carcinoma or advanced thymoma is cisplatin-based chemotherapy. Considering the condition is rare, it is suggested that an international group is set up to organise and evaluate prospective collection of data from cohorts of patients to inform current clinical practice.
Topics: Adult; Antineoplastic Agents; Humans; Prospective Studies; Thymoma; Thymus Neoplasms
PubMed: 23970462
DOI: 10.1002/14651858.CD008588.pub2 -
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 -
Lung Cancer (Amsterdam, Netherlands) Feb 2023Thymic carcinoma (TC) is a rare cancer and patients failing initial chemotherapy (relapse/refractory) face limited therapeutic options given no approved options or... (Meta-Analysis)
Meta-Analysis Review
Thymic carcinoma (TC) is a rare cancer and patients failing initial chemotherapy (relapse/refractory) face limited therapeutic options given no approved options or consensus standard of care. This study aimed to identify and summarize clinical outcomes of all regimens evaluated in clinical trials of relapsed or refractory patients. Interventional trials enrolling advanced TC patients who failed first-line chemotherapy and reported outcomes in this group were eligible for inclusion in our systemic literature review (SLR). Between-study heterogeneity was assessed to determine the feasibility of pooling specific studies and treatments. Objective response rate (ORR), overall survival (OS), progression-free survival (PFS), and duration of response (DOR) endpoints were of interest for meta-analysis. Nineteen trials were identified in the SLR. Three trials with one or two TC patients were removed from our assessment to reduce publication bias. Response rates among studies with at least ten TC patients varied from 9 % to 38 %. Pooled ORRs in patients receiving S-1 (46 patients), sunitinib (46 patients), or pembrolizumab (66 patients) were 28 %, 24 %, and 21 %, respectively. Prolonged duration of response with pembrolizumab was observed with a pooled median of 23.8 months (95 % confidence interval [CI]: 12, not reached). Median PFS of five months or greater was reported in patients treated with sunitinib, lenvatinib, pembrolizumab, capecitabine + gemcitabine, everolimus, or S-1. Median OS of 20 months or greater was reported in trials evaluating S-1 or pembrolizumab; this endpoint was not reached in trials evaluating lenvatinib, regorafenib, or sunitinib. Generalizability of treatment effects is challenging in the research of rare diseases and meta-analysis of clinical outcomes may help to increase precision and relevance of results to the larger TC population. Our study found limited treatment options upon relapse, demonstrating a need for further investigations into novel therapeutics and well-powered clinical trials to better inform on optimal treatments.
Topics: Humans; Sunitinib; Thymoma; Platinum; Neoplasm Recurrence, Local; Lung Neoplasms; Thymus Neoplasms; Antineoplastic Combined Chemotherapy Protocols
PubMed: 36638588
DOI: 10.1016/j.lungcan.2023.01.003 -
Chinese Medical Journal Jul 2017Good's syndrome (GS) is a rare disease characterized by thymoma, hypogammaglobulinemia, low or absent B-cells, decreased T-cells, an inverted CD4+/CD8+ T-cell ratio and... (Review)
Review
BACKGROUND
Good's syndrome (GS) is a rare disease characterized by thymoma, hypogammaglobulinemia, low or absent B-cells, decreased T-cells, an inverted CD4+/CD8+ T-cell ratio and reduced T-cell mitogen proliferative responses. GS is difficult to diagnose preoperatively due to its rarity and lack of typical symptoms, the characteristics of Chinese GS patients are still lacking. This study aimed to systematically review all the clinical, laboratory, and immunologic findings of reported cases of Chinese patients with GS.
METHODS
We searched for case reports and articles up to January 2017 using PubMed, China National Knowledge Infrastructure, Wangfang database and China Science and Technology Journal Database with the following words in combinations as key words: "thymoma," "hypogammaglobulinemia," and "Good's syndrome." The text words and MeSH terms were entered depending on the databases characteristics. The reference lists from retrieved articles were also screened for additional applicable studies. The authors were restricted to Chinese. There was no language restriction.
RESULTS
Forty-seven patients were reported in 27 studies. We found that GS has a nationwide distribution and that most cases (83%) have been described on the mainland of China. The initial clinical presentation is varied, ranging from symptoms related to the thymoma to infections resulting from immunodeficiency. Type AB (50%) is the most common histologic type of thymomas in Chinese GS patients according to the World Health Organization classification of thymomas. With respect to infection, sinopulmonary infection (74%) is the most common type, followed by skin infection (10%) and intestinal tract infection (10%). Diarrhea was presented in 36% of patients, and autoimmune manifestations were presented in 36% of patients.
CONCLUSIONS
GS is a rare association of thymoma and immunodeficiency with a poor prognosis. Astute clinical acumen and increased awareness of the clinical and immunological profile of GS are needed to increase early diagnosis, that would benefit improved therapeutic effects.
Topics: Agammaglobulinemia; Animals; China; Humans; Immunologic Deficiency Syndromes; Rare Diseases; Thymoma; Thymus Neoplasms
PubMed: 28639577
DOI: 10.4103/0366-6999.208234 -
The Annals of Thoracic Surgery Aug 2008The literature describing multimodal treatment of thymomas consists almost exclusively of retrospective case series. We have used a systematic review to investigate the... (Review)
Review
The literature describing multimodal treatment of thymomas consists almost exclusively of retrospective case series. We have used a systematic review to investigate the role of surgery in the management of thymomas. The use of surgery as the sole therapeutic maneuver in thymoma depends on the stage considered. Subtotal resection followed by adjuvant treatment may prolong survival, but studies are equivocal. Some data supports re-resection of recurrent thymoma in the belief that survival will be prolonged. Approaches to thymectomy other than sternotomy in early stage thymoma are technically sound, but long-term outcome data are lacking.
Topics: Humans; Neoplasm Staging; Thoracic Surgery, Video-Assisted; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 18640366
DOI: 10.1016/j.athoracsur.2008.03.055