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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 -
Internal Medicine (Tokyo, Japan) Nov 2002We report a patient with KL-6-producing invasive thymoma. A 58-year-old man was admitted complaining of dyspnea and fatigability. Computed tomography of the chest...
We report a patient with KL-6-producing invasive thymoma. A 58-year-old man was admitted complaining of dyspnea and fatigability. Computed tomography of the chest revealed interstitial pneumonia and an anterior mediastinal tumor. The tumor was surgically extirpated and diagnosed as invasive thymoma. Serum KL-6 levels later increased further and another tumor was found in the liver. That liver tumor was resected and histologically diagnosed as a metastasis of thymoma. Following resection, the serum KL-6 level decreased. Tumor cells of both primary and metastatic lesions exhibited positive reactivity to immunohistochemical staining for KL-6. A review of this case is presented.
Topics: Humans; Male; Middle Aged; Mucins; Neoplasm Invasiveness; Thymoma; Thymus Neoplasms
PubMed: 12487172
DOI: 10.2169/internalmedicine.41.979 -
Journal of Thoracic Oncology : Official... Sep 2010The potential benefits and long-term complications of radiotherapy treatment for malignant thymoma are unclear. This is a retrospective analysis of outcome in patients...
INTRODUCTION
The potential benefits and long-term complications of radiotherapy treatment for malignant thymoma are unclear. This is a retrospective analysis of outcome in patients with malignant thymoma from the Surveillance, Epidemiology, and End Results database between 1973 and 2005.
METHODS
Of the 1987 patients identified, 1334 were analyzed. Patients were categorized according to the Masaoka staging system as stage I to IIA, IIB or III to IV. The primary end points were overall survival, cardiac mortality, and the development of secondary malignancies.
RESULTS
Patients received surgery and radiation (50%), surgery alone (26%), radiation alone (12%), or no treatment (12%). The median follow-up time for survivors was 65 months (range, 1-361 months). There was no significant increase in the 12-year cumulative incidence rate of death from heart disease (10.2% radiation versus 7.5% no radiation, p = 0.83) or incidence of secondary malignancies (11.7% versus 12.4%, p = 0.70) with radiation. Compared with surgery alone, adjuvant radiation significantly improved overall survival in patients with stage III to IV disease (p = 0.04) and demonstrated a nonsignificant trend in patients with stage IIB disease (p = 0.09). However, after excluding patients surviving less than 4 months to account for surgical mortality, the benefit with radiation was no longer significant (stage IIB: p = 0.45, stage III-IV: p = 0.44).
CONCLUSIONS
Radiation does not seem to increase the risk of cardiac mortality or secondary malignancy in patients with malignant thymoma. Although the routine use of postoperative radiotherapy in malignant thymoma does not appear warranted, high-risk patients may benefit from adjuvant radiation. This study can help to design prospective trials to further establish the role of radiotherapy in malignant thymoma.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Combined Modality Therapy; Female; Humans; Male; Middle Aged; Neoplasm Staging; Postoperative Period; Radiotherapy, Adjuvant; Retrospective Studies; SEER Program; Survival Rate; Thymectomy; Thymoma; Thymus Neoplasms; Treatment Outcome; Young Adult
PubMed: 20651611
DOI: 10.1097/JTO.0b013e3181e8f345 -
Virchows Archiv : An International... Jan 2021Thymomas exhibit a unique genomic landscape, comprising the lowest on average total mutational burden among adult human cancers; a unique point mutation in the GTF2I... (Review)
Review
Thymomas exhibit a unique genomic landscape, comprising the lowest on average total mutational burden among adult human cancers; a unique point mutation in the GTF2I gene in WHO type A and AB thymomas (and rarely others); almost unique KMT2A-MAML2 translocations in rare WHO type B2 and B3 thymomas; a unique YAP1-MAML2 translocation in almost all metaplastic thymomas; and unique miRNA profiles in relation to GTF2I mutational status and WHO histotypes. While most thymomas can be diagnosed solely on the basis of morphological features, mutational analyses can solve challenging differential diagnostic problems. No molecular biomarkers have been identified that predict the response of unresectable thymomas to chemotherapy or agents with known molecular targets. Despite the common and strong expression of PDL1 in thymomas, immune checkpoint inhibitors are rarely applicable due to the poor predictability of common, life-threatening autoimmune side effects that are related to the unrivaled propensity of thymomas towards autoimmunity.
Topics: Biomarkers, Tumor; DNA Mutational Analysis; Humans; Mutation; Pathology, Molecular; Thymoma; Thymus Neoplasms
PubMed: 33674910
DOI: 10.1007/s00428-021-03068-8 -
Scientific Reports Feb 2024B-cell subsets in peripheral blood (PB) and tumor microenvironment (TME) were evaluated to determine myasthenia gravis (MG) severity in patients with thymoma-associated...
B-cell subsets in peripheral blood (PB) and tumor microenvironment (TME) were evaluated to determine myasthenia gravis (MG) severity in patients with thymoma-associated MG (TMG) and the distribution of B cells in type B TMG. The distribution of mature B cells, including Bm1-Bm5, CD19 and CD20 B cells and non-switched (NSMBCs) and switched (SMBCs) memory B cells, were determined in 79 patients with thymoma or TMG. Quantitative relationships between the T and TMG groups and the TMG-low and TMG-high subgroups were determined. NSMBCs and SMBCs were compared in TME and PB. Type B thymoma was more likely to develop into MG, with types B2 and B3 being especially associated with MG worsening. The percentage of CD19 B cells in PB gradually increased, whereas the percentage of CD20 B cells and the CD19/CD20 ratio were not altered. The (Bm2 + Bm2')/(eBm5 + Bm5) index was significantly higher in the TMG-high than in thymoma group. The difference between SMBC/CD19 and NSMBC/CD19 B cell ratios was significantly lower in the thymoma than TMG group. NSMBCs assembled around tertiary lymphoid tissue in thymomas of patients with TMG. Few NSMBCs were observed in patients with thymoma alone, with these cells being diffusely distributed. MG severity in patients with TMG can be determined by measuring CD19 B cells and Bm1-Bm5 in PB. The CD19/CD20 ratio is a marker of disease severity in TMG patients. Differences between NSMBCs and SMBCs in PB and TME of thymomas can synergistically determine MG severity in patients with TMG.
Topics: Humans; Thymoma; B-Lymphocyte Subsets; Thymus Neoplasms; B-Lymphocytes; Myasthenia Gravis; Tumor Microenvironment
PubMed: 38302676
DOI: 10.1038/s41598-024-53250-6 -
Journal of Thoracic Oncology : Official... Oct 2010Myasthenia gravis is the best known autoimmune disease associated with thymomas, but other conditions can be found in patients with thymic tumors, including some that... (Review)
Review
Myasthenia gravis is the best known autoimmune disease associated with thymomas, but other conditions can be found in patients with thymic tumors, including some that affect the central nervous system (CNS). We have become particularly interested in patients who have acquired neuromyotonia, the rare Morvan disease, or limbic encephalitis. Neuromyotonia mainly involves the peripheral nerves, Morvan disease affects both the peripheral nervous system and CNS, and limbic encephalitis is specific to the CNS. Many of these patients have voltage-gated potassium channel autoantibodies. All three conditions can be associated with thymomas and may respond to surgical removal of the underlying tumor together with immunotherapies and symptomatic treatments. Herein, we review the results of our recent studies that show that voltage-gated potassium channel autoantibodies are not principally directed against the potassium channels themselves but in some patients are directed against a protein that is complexed with potassium channels in both the peripheral nervous system and CNS, contactin-2 associated protein (Caspr2). These antibodies are common in the subgroup of patients with thymic malignancies.
Topics: Autoantibodies; Humans; Membrane Proteins; Nerve Tissue Proteins; Potassium Channels; Thymoma; Thymus Neoplasms
PubMed: 20859119
DOI: 10.1097/JTO.0b013e3181f23f04 -
Internal Medicine (Tokyo, Japan) Sep 2023
Topics: Humans; Cardiac Tamponade; Thymoma; Thymus Neoplasms; Pericardial Effusion
PubMed: 36642518
DOI: 10.2169/internalmedicine.0903-22 -
BMJ Case Reports Mar 2020Malignancies are often associated with autoimmune diseases, which are addressed by treating the underlying cancer. However, there are rare malignancies that can cause...
Malignancies are often associated with autoimmune diseases, which are addressed by treating the underlying cancer. However, there are rare malignancies that can cause autoimmune diseases even after appropriate treatment. Our patient is a 39-year-old Hispanic man with a malignant thymoma recently treated with chemotherapy and radiation who presented with syncope and dyspnoea. He was found to be both anaemic and thrombocytopenic. His labs were consistent with autoimmune haemolytic anaemia (AIHA), except his reticulocyte count was unexpectedly low. Bone marrow biopsy supported a diagnosis of Evans syndrome, a rare autoimmune condition characterised by (AIHA) combined with immune thrombocytopenia. He was also found to have an acute parvovirus B19 infection. He was treated with steroids and RBC transfusion. His blood counts gradually returned to baseline, with improvement in symptoms. This patient's thymoma treatment and active parvovirus B19 infection likely both played a role in the development of Evans syndrome.
Topics: Adult; Anemia, Hemolytic, Autoimmune; Diagnosis, Differential; Erythrocyte Transfusion; Glucocorticoids; Humans; Male; Parvoviridae Infections; Parvovirus B19, Human; Prednisone; Thrombocytopenia; Thymoma; Thymus Neoplasms
PubMed: 32193179
DOI: 10.1136/bcr-2019-233485 -
Journal of Thoracic Oncology : Official... Oct 2010Almost all series reporting on the results of resection in thymic tumors indicate that the performance of a complete resection is probably the most important prognostic... (Review)
Review
Almost all series reporting on the results of resection in thymic tumors indicate that the performance of a complete resection is probably the most important prognostic factor. This issue is not a factor in Masaoka stage I and II tumors that are almost always easily completely resected and have an excellent prognosis. Masaoka stage III tumors that invade the pericardium, lungs, or great vessels have relatively higher incomplete resection rates, significantly higher recurrence rates, and thus a worse prognosis. There are several small reports on the efficacy of resection of the great veins when involved by a thymic malignancy with low morbidity and meaningful long-term survival. Superior vena cava reconstruction is commonly performed by a polytetrafluroethylene, venous, or pericardial graft. These cases can usually be identified preoperatively and, thus, considered for induction therapy. Because these types of cases are almost always of marginal respectability in terms of obtaining a true en bloc resection, there is an increasing enthusiasm for offering induction therapy in an effort to enhance resectability. Preliminary results suggest increased R0 resection rates and improved survival with induction therapy for locally advanced tumors. The optimal induction treatment is unknown. The ultimate extended surgery for advanced thymic tumors is an extrapleural pneumonectomy performed for extensive pleural disease (Masaoka stage IVA). These rarely performed operations are done for IVA disease found at initial presentation and for recurrent disease as a salvage procedure. Again these advanced patients are probably best managed by induction chemotherapy followed by resection.
Topics: Clinical Trials as Topic; Humans; Neoplasm Staging; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 20859130
DOI: 10.1097/JTO.0b013e3181f20eb3 -
Journal of Thoracic Oncology : Official... Sep 2014Normal thymic architecture is essential for the proper development of T-lymphocytes. Immature T-cell progenitors enter the thymus where through interactions with... (Review)
Review
Normal thymic architecture is essential for the proper development of T-lymphocytes. Immature T-cell progenitors enter the thymus where through interactions with cortical and medullary thymic epithelial cells (TECs) they undergo positive and negative selection and become competent cells that do not react with self-antigens. This process requires normal thymic architecture, expression of major histocompatibility complex (MHC) class II, and normal expression of the autoimmune regulator (AIRE) gene. Thymomas are rare neoplasms of the TECs that often generate lymphocytes that mature into CD4+ and CD8+ T-lymphocytes. However, several abnormalities have been described in thymomas that may affect normal T-cell development: the tumor architecture is distorted, neoplastics expresses less MHC class II, most thymomas do not express AIRE, and production of T-regulator cells is decreased. Thymomas are associated with a variety of autoimmune disorders often linked to T-cell-mediated autoimmunity. Myasthenia gravis, the most common autoimmune disorder associated with thymoma patients, is present in 30% of patients with thymoma. Several theories attempt to explain the association of immune disorders with thymomas. These different theories are based on failure of positive and negative selection of T-lymphocytes and on autoimmunizing mechanisms in an AIRE-poor environment in the thymus. The finding that immunosurveillance against cancer may be impaired before the diagnosis of thymoma may challenge current theories and suggest a more complex defect in T-lymphocyte maturation. It is likely that a combination of mechanisms is responsible for immune disorders in patients with thymoma. More investigation is needed to clarify the basic mechanisms responsible for immune disorders in patients with thymoma.
Topics: Humans; Thymoma; Thymus Neoplasms
PubMed: 25396311
DOI: 10.1097/JTO.0000000000000299