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Journal of Thoracic Oncology : Official... Oct 2010Thymomas are relatively uncommon. Nevertheless, an accumulation of studies (mostly retrospective, single-institution series) have made it important to approach this... (Review)
Review
Thymomas are relatively uncommon. Nevertheless, an accumulation of studies (mostly retrospective, single-institution series) have made it important to approach this disease in a knowledgeable, evidence-based fashion. This begins with the approach to evaluation of a patient with an anterior mediastinal mass, in whom a reliable clinical diagnosis is usually possible in experienced centers. Surgical resection is the mainstay of treatment, and every effort must be made to achieve a complete resection.
Topics: Humans; Neoplasm Staging; Prognosis; Thymoma; Thymus Neoplasms
PubMed: 20859126
DOI: 10.1097/JTO.0b013e3181f20dab -
Journal of Thoracic Oncology : Official... Dec 2023A TNM-based system for all types of thymic epithelial tumors was introduced in the eighth edition of the TNM classification of thoracic malignancies. The Thymic Domain...
The International Association for the Study of Lung Cancer Thymic Epithelial Tumors Staging Project: Proposal for a Stage Classification for the Forthcoming (Ninth) Edition of the TNM Classification of Malignant Tumors.
INTRODUCTION
A TNM-based system for all types of thymic epithelial tumors was introduced in the eighth edition of the TNM classification of thoracic malignancies. The Thymic Domain of the Staging and Prognostic Factors Committee of the International Association for the Study of Lung Cancer, composed of multispecialty international experts, was charged to develop proposals for the ninth edition. This article outlines the proposed definitions for the T, the N, and the M components and their combination into stage groups.
METHODS
A large central database of 11,347 patients with thymic epithelial tumors was assembled thanks to the contribution of the major thymic organizations worldwide and analyses were carried out for the T, the N, and the M components and the stage groups. Overall survival was the outcome measure for patients with completely and incompletely resected tumors, and recurrence for those with complete resection. When the number of patients was sufficient, analyses were performed separately for thymomas, thymic carcinomas, and neuroendocrine thymic tumors.
RESULTS
Tumor size is included in the T1 category as T1a (≤5cm) and T1b (>5 cm); the mediastinal pleura is dropped as a T descriptor; invasion of the lung or phrenic nerve is reclassified as T2 (instead of T3). No changes are proposed for the N and the M components from the eighth edition. The stage groups remain the same.
CONCLUSIONS
The proposed changes for the ninth edition of the TNM classification set the stage for further progress in the future for these rare tumors.
Topics: Humans; Neoplasm Staging; Lung Neoplasms; Prognosis; Myeloma Proteins; Thymus Neoplasms; Thymoma; Neuroendocrine Tumors; Neoplasms, Glandular and Epithelial
PubMed: 37689391
DOI: 10.1016/j.jtho.2023.09.002 -
Current Treatment Options in Oncology Dec 2008Although thymic epithelial tumors are rare, they are relatively common among neoplasms of the anterior superior mediastinum. They usually exhibit indolent behavior, but... (Review)
Review
Although thymic epithelial tumors are rare, they are relatively common among neoplasms of the anterior superior mediastinum. They usually exhibit indolent behavior, but do have the capacity to invade surrounding structures and metastasize to distant sites. Thymic carcinomas are rare, but are highly aggressive tumors that are associated with a poor prognosis. The mainstay of therapy is complete surgical resection. Locally advanced thymoma and thymic carcinoma require a multimodality treatment approach with a combination of surgery, chemotherapy, and radiation therapy to decrease the chances of recurrence and improve survival. The risk of disease recurrence lasts for a number of years after completion of primary therapy. A majority of cases of recurrent disease present as pleural recurrences. Once again, surgical resection of recurrent disease represents the cornerstone of successful therapy and is critical to long-term survival. In recent years, a better understanding of the biologic basis of thymic epithelial tumors has led to the emergence of targeted therapy directed against this malignancy.
Topics: Adult; Aged; Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Humans; Middle Aged; Neoplasm Staging; Survival Rate; Survivors; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 19381821
DOI: 10.1007/s11864-009-0083-7 -
Journal of Thoracic Oncology : Official... Jan 2009Thymomas are rare intrathoracic malignant tumors. Commonly used staging system is the Masaoka classification, based on peroperative and histopathological findings.... (Review)
Review
Thymomas are rare intrathoracic malignant tumors. Commonly used staging system is the Masaoka classification, based on peroperative and histopathological findings. Surgery is the cornerstone of the management of thymomas, initially being useful for precise histopathological diagnosis and staging, and in most cases ensuring the first step of the therapeutics simultaneously. After tumor stage, complete resection is the most constant and significant prognostic factor for progression-free and overall survival. Postoperative radiotherapy is recommended in incompletely resected thymomas. Completely resected stage II and III tumors may also benefit from adjuvant radiotherapy to reduce local recurrence rates but without impact on survival. In primary unresectable thymomas, multimodal strategy nowadays includes neoadjuvant chemotherapy, extensive surgery, adjuvant radiotherapy, and in some cases, adjuvant chemotherapy. The most popular chemotherapy regimens combine cisplatin, adriamycin, etoposide, cyclophophamide, or ifosfamide.The management of thymomas is a paradigm of cooperation between clinicians, surgeons, and pathologists from establishing the diagnosis to organizing the therapeutic strategy and evaluating the prognosis. As a consequence of their rarity, no prospective randomized trials are available and collaborative studies are warranted to evaluate and improve current therapeutic standards, taking into account recent improvements in techniques, such as robotic surgery, radiotherapy, and supportive treatments.
Topics: Humans; Thymoma; Thymus Neoplasms
PubMed: 19096319
DOI: 10.1097/JTO.0b013e31818e105c -
Thoracic Cancer May 2021Thymomas and thymic carcinomas are the most common tumor types among anterior mediastinal lesions. However, the relationship between molecular aberrations and thymoma...
BACKGROUND
Thymomas and thymic carcinomas are the most common tumor types among anterior mediastinal lesions. However, the relationship between molecular aberrations and thymoma patients are poorly understood, especially abnormal changes in the expression profiles of circRNAs. The purpose of the present study was to investigate the expression profiles of circRNAs in thymoma patients and their possible roles in the pathogenesis of thymoma.
METHODS
Diseased tissues and surrounding normal thymic tissues in two thymoma patients were collected for circRNA sequencing. The top four upregulated circRNAs were selected as candidates and further validated with RT-PCR in 20 thymoma patients. Gene ontology and signal transduction network analyses of circRNA-related mRNAs were performed to analyze the functional properties. Survival analysis of their parental genes were also carried out to evaluate the clinical value of differentially expressed circRNA.
RESULTS
A total of 73 circRNAs were differentially expressed in thymoma tissues using high-throughput sequencing. Among these circRNAs, hsa_circ_0001173, hsa_circ_0007291, hsa_circ_0003550, and hsa_circ_0001947 were significantly upregulated in thymoma tissues compared with normal thymic tissues. We identified that these four circRNA-related mRNAs were involved in cell-cell adhesion, MAPK pathways, and TNF pathway, which may contribute to the pathological immune disorder in thymoma. Finally, we also found that SCAP (hsa_circ_0007291 parental gene) and AFF2 (hsa_circ_0001947 parental gene) were all significantly related with progression-free survival (PFS) of thymoma patients in a Kaplan-Meier plot (p-value <0.05).
CONCLUSIONS
The expression levels of hsa_circ_0001173, hsa_circ_0007291, hsa_circ_0003550, and hsa_circ_0001947 were significantly upregulated and positively correlated with immune imbalance in thymoma patients.
Topics: Humans; RNA, Circular; Survival Analysis; Thymoma
PubMed: 33704915
DOI: 10.1111/1759-7714.13873 -
BMC Neurology Mar 2022Isaacs' syndrome is a peripheral nerve hyperexcitability (PNH) syndrome due to peripheral motor nerve instability. Acquired Isaacs' syndrome is recognized as a...
BACKGROUND
Isaacs' syndrome is a peripheral nerve hyperexcitability (PNH) syndrome due to peripheral motor nerve instability. Acquired Isaacs' syndrome is recognized as a paraneoplastic autoimmune disease with possible pathogenic voltage-gated potassium channel (VGKC) complex antibodies. However, the longitudinal correlation between clinical symptoms, VGKC antibodies level, and drug response is still unclear.
CASE PRESENTATION
A 45-year-old man had progressive four limbs soreness, muscle twitching, cramps, and pain 4 months before admission. Electromyography (EMG) studies showed myokymic discharges, neuromyotonia, and an incremental response in the high-rate (50 Hz) repetitive nerve stimulation (RNS) test. Isaacs' syndrome was diagnosed based on clinical presentations and EMG reports. Serum studies showed positive VGKC complex antibodies, including leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) antibodies. The acetylcholine receptor antibody was negative. Whole-body computed tomography (CT) and positron emission tomography revealed a mediastinal tumor with the great vessels encasement, right pleura, and diaphragm seeding. Biopsy confirmed a World Health Organization type B2 thymoma, with Masaoka stage IVa. His symptoms gradually improved and both LGI1 and CASPR2 antibodies titer became undetectable after concurrent chemoradiotherapy (CCRT) and high dose steroid treatment. However, his Isaacs' syndrome recurred after the steroid was reduced 5 months later. Follow-up chest CT showed probable thymoma progression. LGI1 antibody turned positive again while CASPR2 antibody remained undetectable.
CONCLUSIONS
Our patient demonstrates that Isaacs' syndrome could be the initial and only neuromuscular manifestation of malignant thymoma. His Isaacs' syndrome is correlated well with the LGI1 antibody level. With an unresectable thymoma, long-term immunosuppressant therapy may be necessary for the management of Isaacs' syndrome in addition to CCRT for thymoma.
Topics: Autoantibodies; Humans; Isaacs Syndrome; Male; Middle Aged; Neoplasm Recurrence, Local; Potassium Channels, Voltage-Gated; Thymoma; Thymus Neoplasms
PubMed: 35246046
DOI: 10.1186/s12883-022-02584-7 -
Journal of Thoracic Oncology : Official... Oct 2010Thymomas are rare epithelial tumors that display significant heterogeneity. Thymomas are usually indolent; however, thymic carcinomas are typically invasive with a high... (Review)
Review
Thymomas are rare epithelial tumors that display significant heterogeneity. Thymomas are usually indolent; however, thymic carcinomas are typically invasive with a high risk of relapse and death. Current treatment approaches are primarily based on clinical stage. Surgery is the mainstay for treatment of early stage disease, whereas multimodality therapy is required for advanced disease. The most important prognostic factors are stage and histology; however, increasing recognition of disease heterogeneity has led to recent exploration of underlying molecular mechanisms. Molecular characterization of thymic tumors may offer strategies to improve diagnosis, therapy, and prognosis. This article describes recently identified molecular characteristics of thymoma and thymic carcinoma that may potentially impact disease classification, targeted therapeutic decision making, and design of future clinical trials.
Topics: Antineoplastic Agents; Clinical Trials as Topic; Combined Modality Therapy; Humans; Neoplasm Proteins; Thymoma; Thymus Neoplasms
PubMed: 20859125
DOI: 10.1097/JTO.0b013e3181f20d9a -
Journal of Medical Case Reports Oct 2014Approximately ten to fifteen percent of patients with myasthenia gravis are found to have a thymoma, and twenty to twenty-five percent of patients with thymoma have... (Review)
Review
INTRODUCTION
Approximately ten to fifteen percent of patients with myasthenia gravis are found to have a thymoma, and twenty to twenty-five percent of patients with thymoma have myasthenia gravis. Thymomatous myasthenia gravis tends to have a difficult clinical course and poor prognosis.
CASE PRESENTATION
We report two cases (one patient of Asian ethnicity and the other of Caucasian ethnicity) of atypical presentations of myasthenia gravis associated with invasive malignant thymoma. Both patients were diagnosed at a young age, in their 20s. They presented with a turbulent course of myasthenia gravis and recurrent thymoma, but obtained good outcome after aggressive treatment involving multiple different specialists.
CONCLUSIONS
Although thymomatous myasthenia gravis tends to have a difficult clinical course and poor prognosis, early and aggressive treatment along with multidisciplinary management may improve the outcome of these patients.
Topics: Adult; Female; Humans; Male; Myasthenia Gravis; Thymoma; Thymus Neoplasms
PubMed: 25312448
DOI: 10.1186/1752-1947-8-340 -
Medical Science Monitor : International... Jul 2021BACKGROUND Thymoma is the most common tumor of the anterior mediastinum, and can be caused by infrequent malignancies arising from the epithelial cells of the thymus....
BACKGROUND Thymoma is the most common tumor of the anterior mediastinum, and can be caused by infrequent malignancies arising from the epithelial cells of the thymus. Unfortunately, blood-based diagnostic markers are not currently available. High-throughput sequencing technologies, such as RNA-seq with next-generation sequencing, have facilitated the detection and characterization of both coding and non-coding RNAs (ncRNAs), which play significant roles in genomic regulation, transcriptional and post-transcriptional regulation, and imprinting and epigenetic modification. The knowledge about fusion genes and ncRNAs in thymomas is scarce. MATERIAL AND METHODS For this study, we gathered large-scale RNA-seq data belonging to samples from 25 thymomas and 25 healthy thymus specimens and analyzed them to identify fusion genes, lncRNAs, and miRNAs. RESULTS We found 21 fusion genes, including KMT2A-MAML2, HADHB-REEP1, COQ3-CGA, MCM4-SNTB1, and IFT140-ACTN4, as the most frequent and significant in thymomas. We also detected 65 differentially-expressed lncRNAs in thymomas, including AFAP1-AS1, LINC00324, ADAMTS9-AS1, VLDLR-AS1, LINC00968, and NEAT1, that have been validated with the TCGA database. Moreover, we identified 1695 miRNAs from small RNA-seq data that were overexpressed in thymomas. Our network analysis of the lncRNA-mRNA-miRNA regulation axes identified a cluster of miRNAs upregulated in thymomas, that can trigger the expression of target protein-coding genes, and lead to the disruption of several biological pathways, including the PI3K-Akt signaling pathway, FoxO signaling pathway, and HIF-1 signaling pathway. CONCLUSIONS Our results show that overexpression of this miRNA cluster activates PI3K-Akt, FoxO, HIF-1, and Rap-1 signaling pathways, suggesting pathway inhibitors may be therapeutic candidates against thymoma.
Topics: Adult; Aged; Biomarkers, Tumor; Female; Humans; Male; Middle Aged; RNA, Long Noncoding; Thymoma; Thymus Neoplasms
PubMed: 34219124
DOI: 10.12659/MSM.929727 -
European Journal of Cardio-thoracic... Apr 2015Observational studies on long-term outcomes following debulking surgery or surgical biopsy for unresectable thymoma showed various results. This meta-analysis was... (Meta-Analysis)
Meta-Analysis Review
Observational studies on long-term outcomes following debulking surgery or surgical biopsy for unresectable thymoma showed various results. This meta-analysis was designed to determine the effect of debulking surgery against surgical biopsy on overall survival in patients with unresectable thymoma. The PubMed database was queried for studies published in the English language on unresectable thymoma and overall survival. We compared overall survival following surgery in patients undergoing debulking surgery and patients undergoing surgical biopsy for unresectable thymoma. Meta-analysis was performed using the Mantel-Haenszel method, and potential publication bias was evaluated with a funnel plot of precision. No randomized trials on this topic were identified. Thirteen retrospective observational studies containing a sum of 314 patients with information on the number of deaths and person-years were suitable for analysis. Information on Masaoka stages, World Health Organization histology and indications for debulking surgery versus surgical biopsy was lacking in most studies. Diversity of neoadjuvant and adjuvant treatments was noted among the eligible studies. One hundred and seventy-two (54.8%) patients underwent debulking surgery and 142 (45.2%) underwent surgical biopsy. The pooled hazard ratio was 0.451 (95% confidence interval: 0.336-0.605, P < 0.001), favouring patients undergoing debulking surgery compared with patients undergoing surgical biopsy. The funnel plot of precision demonstrated no important publication bias. Our results suggest that debulking surgery for unresectable thymoma may be associated with improved overall survival and be considered for patients with unresectable thymoma.
Topics: Biopsy; Humans; Postoperative Complications; Survival Analysis; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 25015950
DOI: 10.1093/ejcts/ezu277