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BMJ Case Reports Jul 2012Paraneoplastic neurological syndromes are conditions that manifest as the remote effects of cancer. These are very rare, occurring in 1/10000 patients with a malignancy,...
Paraneoplastic neurological syndromes are conditions that manifest as the remote effects of cancer. These are very rare, occurring in 1/10000 patients with a malignancy, and include Lambert-Eaton myasthenic syndrome, limbic encephalitis, subacute cerebellar ataxia, opsoclonus-myoclonus, Stiff-Person Syndrome, retinopathies, chronic gastrointestinal pseudo-obstruction and sensory neuropathy. This report describes a case of 41-year-old man who presented with elements of multiple paraneoplastic syndromes, including chronic gastrointestinal pseudo-obstruction, myasthenia gravis-Lambert-Eaton overlap syndrome and polymyositis, and who was subsequently found to have a malignant thymoma. There are only three reported cases in the literature describing cases of Lambert-Eaton myasthenic syndrome in association with a thymoma, and only one case of a myasthenia gravis-Lambert-Eaton overlap syndrome in a patient with thymoma. However, there are no documented cases in the literature of this constellation of syndromes in a patient with a malignant thymoma.
Topics: Adult; Humans; Intestinal Pseudo-Obstruction; Lambert-Eaton Myasthenic Syndrome; Male; Myasthenia Gravis; Polymyositis; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 22761203
DOI: 10.1136/bcr.02.2012.5790 -
American Journal of Surgery Jul 1978The histopathology, clinical features, treatment, and results are reported in twenty-nine patients with thymoma. Benign and malignant thymomas are differentiated by...
The histopathology, clinical features, treatment, and results are reported in twenty-nine patients with thymoma. Benign and malignant thymomas are differentiated by their invasive characteristics. Surgical excision is recommended for benign lesions. Surgical excision and postoperative irradiation are recommended for malignant thymomas irrespective of the predominant cell type. The recommended treatment for thymoma and the symptomatic results in myasthenia gravis are unpredictable.
Topics: Adult; Female; Humans; Infant; Male; Middle Aged; Myasthenia Gravis; Thymoma; Thymus Neoplasms
PubMed: 677390
DOI: 10.1016/0002-9610(78)90199-x -
Interactive Cardiovascular and Thoracic... Sep 2012A best evidence topic in cardiothoracic surgery was written according to a structured protocol. The question addressed was 'Does surgical debulking for advanced stages... (Review)
Review
A best evidence topic in cardiothoracic surgery was written according to a structured protocol. The question addressed was 'Does surgical debulking for advanced stages of thymoma improve survival?' Altogether, only 17 papers were found using the reported search, of which only 10 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated; these studies have mainly reported the survival and recurrence rates after total vs subtotal resection of thymic tumours in patients receiving or not receiving adjuvant chemoradiotherapy. These studies confirmed that complete resection is the best prognostic factor in thymomas. With regard to subtotal tumour resection/debulking, we did not find any randomized controlled trials. The evidence on this topic is scarce and these 10 reported were retrospective reviews of the operative, histology and survival data of patients with thymoma who had subtotal vs partial resection for advanced stages of thymoma. Although debulking surgery for thymoma had positively affected survival, in six studies, the difference failed to reach statistical significance. Three of the studies, on the other hand, showed a higher survival rate in thymomas in which maximum debulking was performed and the treatment was followed by high-dose irradiation. None of these studies showed any benefit in debulking surgery for thymic carcinoma. Besides histology and tumour cell-type, other factors influencing survival included the tumour stage and the presence of symptoms such as myasthenia gravis as a warning sign at an early stage. Current evidence in the literature on the survival after debulking surgery for thymoma is contradictory, and most of the studies do not show any survival benefit after debulking for thymoma. However, debulking surgery minimizes the tumour size and area for irradiation postoperatively, hence it can result in less damage to the adjacent tissue during radiotherapy and may be considered for patients in advanced stages of thymoma in whom extensive radiotherapy will be required. In these cases, however, the risks of surgery followed by radiotherapy or radiotherapy alone should carefully be assessed prior to the initiation of treatment.
Topics: Adult; Female; Humans; Neoplasm Staging; Survival Rate; Thymectomy; Thymoma; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 22700684
DOI: 10.1093/icvts/ivs263 -
Lung Cancer (Amsterdam, Netherlands) Apr 2023The aim of this study was to differentiate benign from malignant tumors in the anterior mediastinum based on computed tomography (CT) imaging characteristics, which...
OBJECTIVES
The aim of this study was to differentiate benign from malignant tumors in the anterior mediastinum based on computed tomography (CT) imaging characteristics, which could be useful in preoperative planning. Additionally, our secondary aim was to differentiate thymoma from thymic carcinoma, which could guide the use of neoadjuvant therapy.
MATERIALS AND METHODS
Patients referred for thymectomy were retrospectively selected from our database. Twenty-five conventional characteristics were evaluated by visual analysis, and 101 radiomic features were extracted from each CT. In the step of model training, we applied support vector machines to train classification models. Model performance was assessed using the area under the receiver operating curves (AUC).
RESULTS
Our final study sample comprised 239 patients, 59 (24.7 %) with benign mediastinal lesions and 180 (75.3 %) with malignant thymic tumors. Among the malignant masses, there were 140 (58.6 %) thymomas, 23 (9.6 %) thymic carcinomas, and 17 (7.1 %) non-thymic lesions. For the benign versus malignant differentiation, the model that integrated both conventional and radiomic features achieved the highest diagnostic performance (AUC = 0.715), in comparison to the conventional (AUC = 0.605) and radiomic-only (AUC = 0.678) models. Similarly, regarding thymoma versus thymic carcinoma differentiation, the model that integrated both conventional and radiomic features also achieved the highest diagnostic performance (AUC = 0.810), in comparison to the conventional (AUC = 0.558) and radiomic-only (AUC = 0.774) models.
CONCLUSION
CT-based conventional and radiomic features with machine learning analysis could be useful for predicting pathologic diagnoses of anterior mediastinal masses. The diagnostic performance was moderate for differentiating benign from malignant lesions and good for differentiating thymomas from thymic carcinomas. The best diagnostic performance was achieved when both conventional and radiomic features were integrated in the machine learning algorithms.
Topics: Humans; Thymoma; Retrospective Studies; Lung Neoplasms; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 36871345
DOI: 10.1016/j.lungcan.2023.02.014 -
Journal of Thoracic Oncology : Official... Oct 2010Thymomas and thymic carcinomas (TC) are rare epithelial tumors of the thymus. Although most thymomas have organotypic features (i.e., resemble the normal thymus), TC are... (Review)
Review
Thymomas and thymic carcinomas (TC) are rare epithelial tumors of the thymus. Although most thymomas have organotypic features (i.e., resemble the normal thymus), TC are morphologically undistinguishable from carcinomas in other organs. Apart from their different morphology, TC and thymomas differ also in functional terms (TC, in contrast to thymomas, have lost the capacity to promote the maturation of intratumorous lymphocytes), have different genetic features (discussed in this review), a different immunoprofile (most TC overexpress c-KIT, whereas thymomas are consistently negative), and different clinical features (TC, in contrast to thymomas, are not associated with paraneoplastic myasthenia gravis). Thus, although all the data suggest that the biology of thymomas and TC is different, in clinical practice, their therapeutic management up to now is identical. In the age of personalized medicine, the time may have come to think this over. We will briefly review the molecular genetics of malignant thymic tumors, summarize the current status of targeted therapies with an emphasis on the multitargeted kinase inhibitors sunitinib and sorafenib, and try to outline some future directions.
Topics: Adult; Female; Humans; Liver Neoplasms; Middle Aged; Neoplasm Proteins; Prognosis; Thoracic Wall; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 20859121
DOI: 10.1097/JTO.0b013e3181f209a8 -
Current Treatment Options in Oncology Jun 2019The thymus is a key organ involved in establishing central immune tolerance. Thymic epithelial tumors (TETs) include thymomas and thymic carcinomas. Thymomas, which are... (Review)
Review
The thymus is a key organ involved in establishing central immune tolerance. Thymic epithelial tumors (TETs) include thymomas and thymic carcinomas. Thymomas, which are histologically distinct from thymic carcinomas, lead to dysregulated thymopoiesis via decreased thymic epithelial expression of AIRE and MHC Class II, as well as via alterations in thymic architecture, thereby resulting in autoimmune complications that manifest as paraneoplastic disorders (PNDs). Although progress has been made in elucidating the mechanisms underlying thymoma-associated PNDs, there remains a great need to further define the underlying mechanisms and to identify additional immune biomarkers, such as novel antibodies (in "seronegative" cases) to facilitate diagnosis and monitoring of patients. In addition, a better understanding of the pathogenesis of PNDs could lead to improved treatment strategies for both thymomas and their immune complications. In advanced, refractory cases of TETs (both thymoma and thymic carcinoma), additional therapeutic approaches are needed. Immune checkpoint inhibitors have revolutionized the treatment of several malignancies and hold promise in the treatment of TETs; however, the risks for immune-related adverse events (especially for inducing PNDs as well as in the setting of pre-existing PNDs) underscore the need to optimize patient selection and improve clinical management before there can be widespread acceptance of checkpoint inhibitor therapy in patients with TETs.
Topics: Antineoplastic Agents, Immunological; Biomarkers, Tumor; Humans; Paraneoplastic Syndromes; Thymoma; Thymus Neoplasms
PubMed: 31227926
DOI: 10.1007/s11864-019-0661-2 -
Presse Medicale (Paris, France : 1983) Oct 1984Three cases of metastatic malignant thymoma are reported. In all three cases the tumour was invasive and excision was only partial or impossible. Histologically, these...
Three cases of metastatic malignant thymoma are reported. In all three cases the tumour was invasive and excision was only partial or impossible. Histologically, these tumours were of epithelial origin with a variable lymphocytic component. Metastases were initially present in one case and in the other two developed within the first year. Partial remission was obtained with different drug combinations in only one case, and it was of short duration. The authors emphasize the increased frequency and poor prognosis of these tumours and the need for multiple chemotherapy and loco-regional treatment in the management of invasive thymomas.
Topics: Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Female; Humans; Male; Prognosis; Thymoma; Thymus Neoplasms
PubMed: 6239155
DOI: No ID Found -
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 -
Seminars in Thoracic and Cardiovascular... Jan 1992
Review
Topics: Humans; Myasthenia Gravis; Prognosis; Thymoma; Thymus Neoplasms
PubMed: 1550889
DOI: No ID Found -
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