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1.
Can Vet J ; 65(7): 682-691, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38952759

ABSTRACT

Objective: Thymoma-associated paraneoplastic syndromes in dogs and cats include myasthenia gravis, hypercalcemia, exfoliative dermatitis, erythema multiforme, T-cell lymphocytosis, myocarditis, anemia, and polymyositis. Paraneoplastic myasthenia gravis (MG) is the most commonly reported paraneoplastic syndrome in dogs with thymic epithelial tumors. The objective of this study was to examine cases of canine thymic-associated MG treated surgically, with the specific objective of providing an updated clinical picture of the preoperative management, postoperative complications, and outcomes of these cases. Animals: Nine dogs with paraneoplastic MG underwent surgical removal of a thymic epithelial tumor. Procedure: Medical records of dogs with MG that received surgical treatment of a thymic epithelial tumor between January 1, 2012 and October 1, 2022 were obtained from 4 veterinary teaching hospitals. Descriptions of perioperative MG management, complications, and outcomes were reported. Results: Six of the 9 dogs received medical therapy for MG, with either a cholinesterase inhibitor (4 dogs) or a cholinesterase inhibitor and immunosuppressive agent (2 dogs), before surgery. The median duration of medical therapy for MG before surgery was 7.5 d (range: 2 to 60 d). Three of 9 dogs experienced immediate postoperative complications and were euthanized. Six of 9 dogs (66.6%) survived to discharge and 3 of 6 dogs that survived to discharge were alive at the time of writing. At the time of writing, 3 of 6 dogs had complete resolution of clinical signs attributable to MG and 2 of 6 had partial resolution. The median time from surgery to resolution of clinical signs of MG in these dogs was 63 d (range: 2 to 515 d). Conclusion: Dogs with thymic epithelial tumors and paraneoplastic MG are at a high risk for perioperative complications. Clinical relevance: The findings of this study corroborate previous literature stating that paraneoplastic MG is a poor prognostic indicator for dogs with thymic epithelial tumors, while also highlighting the variation in approaches to clinical management of thymic-associated MG in veterinary medicine and the lack of established protocols guiding perioperative management.


Prise en charge préopératoire et complications postopératoires chez 9 chiens subissant un traitement chirurgical de la myasthénie grave associée au thymus. Objectif: Les syndromes paranéoplasiques associés au thymome chez le chien et le chat comprennent la myasthénie grave, l'hypercalcémie, la dermatite exfoliative, l'érythème polymorphe, la lymphocytose à cellules T, la myocardite, l'anémie et la polymyosite. La myasthénie paranéoplasique (MG) est le syndrome paranéoplasique le plus fréquemment rapporté chez les chiens atteints de tumeurs épithéliales thymiques. L'objectif de cette étude était d'examiner les cas de MG canine associée au thymus traités chirurgicalement, dans le but spécifique de fournir un tableau clinique actualisé de la prise en charge préopératoire, des complications postopératoires et des résultats de ces cas. Animaux: Neuf chiens atteints de MG paranéoplasique ont subi l'ablation chirurgicale d'une tumeur épithéliale thymique. Procédure: Les dossiers médicaux des chiens atteints de MG ayant reçu un traitement chirurgical d'une tumeur épithéliale thymique entre le 1er janvier 2012 et le 1er octobre 2022 ont été obtenues auprès de 4 hôpitaux universitaires vétérinaires. Des descriptions de la prise en charge péri-opératoire de la MG, des complications et des résultats ont été rapportées. Résultats: Six des 9 chiens ont reçu un traitement médical pour la MG, avec soit un inhibiteur de la cholinestérase (4 chiens), soit un inhibiteur de la cholinestérase et un agent immunosuppresseur (2 chiens), avant la chirurgie. La durée médiane du traitement médical de la MG avant la chirurgie était de 7,5 jours (plage : 2 à 60 jours). Trois des neuf chiens ont présenté des complications postopératoires immédiates et ont été euthanasiés. Six des 9 chiens (66,6 %) ont survécu jusqu'à leur sortie et 3 des 6 chiens qui ont survécu jusqu'à leur sortie étaient en vie au moment de la rédaction. Au moment de la rédaction de cet article, 3 chiens sur 6 présentaient une résolution complète des signes cliniques attribuables à la MG et 2 chiens sur 6 présentaient une résolution partielle. Le délai médian entre l'intervention chirurgicale et la résolution des signes cliniques de MG chez ces chiens était de 63 jours (plage : 2 à 515 jours). Conclusion: Les chiens atteints de tumeurs épithéliales thymiques et de MG paranéoplasique présentent un risque élevé de complications périopératoires. Pertinence clinique: Les résultats de cette étude corroborent la littérature antérieure indiquant que la MG paranéoplasique est un indicateur de mauvais pronostic pour les chiens atteints de tumeurs épithéliales thymiques, tout en soulignant également la variation des approches de prise en charge clinique de la MG associée au thymus en médecine vétérinaire et le manque de protocoles établis de gestion guidant les interventions périopératoires.(Traduit par Dr Serge Messier).


Subject(s)
Dog Diseases , Myasthenia Gravis , Postoperative Complications , Thymus Neoplasms , Animals , Dogs , Dog Diseases/surgery , Myasthenia Gravis/veterinary , Myasthenia Gravis/surgery , Thymus Neoplasms/veterinary , Thymus Neoplasms/surgery , Thymus Neoplasms/complications , Postoperative Complications/veterinary , Male , Female , Cholinesterase Inhibitors/therapeutic use , Preoperative Care/veterinary , Immunosuppressive Agents/therapeutic use , Neoplasms, Glandular and Epithelial/veterinary , Neoplasms, Glandular and Epithelial/surgery , Thymoma/veterinary , Thymoma/surgery , Thymoma/complications
2.
J Clin Immunol ; 44(7): 156, 2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38954150

ABSTRACT

BACKGROUND: Thymoma presents with several autoimmune manifestations and is associated with secondary autoimmune regulator (AIRE) deficiency. Pneumonitis has recently been described as an autoimmune manifestation associated with thymoma presenting with similar clinical, radiographic, histological, and autoantibody features as seen in patients with inherited AIRE deficiency who suffer from Autoimmune PolyEndocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED) syndrome. OBJECTIVES: To treat two patients with biopsy-proven thymoma-associated pneumonitis with lymphocyte-directed immunomodulation. METHODS: Two patients with thymoma were enrolled on IRB-approved protocols at the NIH Clinical Center. We performed history and physical examination; laboratory, radiographic, histologic and pulmonary function evaluations; and measurement of the lung-directed autoantibodies KCNRG and BPIFB1 prior to and at 1- and 6-months following initiation of lymphocyte-directed immunomodulation with azathioprine with or without rituximab. RESULTS: Combination T- and B-lymphocyte-directed immunomodulation resulted in improvement of clinical, functional, and radiographic parameters at 6-month follow-up evaluations in both patients with sustained remission up to 12-36 months following treatment initiation. CONCLUSION: Lymphocyte-directed immunomodulation remitted autoimmune pneumonitis in two patients with thymoma.


Subject(s)
Immunomodulation , Thymoma , Humans , Thymoma/immunology , Thymoma/complications , Thymoma/diagnosis , Female , Male , Rituximab/therapeutic use , Autoantibodies/immunology , Middle Aged , Thymus Neoplasms/immunology , Thymus Neoplasms/complications , Thymus Neoplasms/diagnosis , Pneumonia/etiology , Pneumonia/immunology , Pneumonia/diagnosis , Autoimmune Diseases/immunology , Autoimmune Diseases/diagnosis , Autoimmune Diseases/etiology , Adult , Azathioprine/therapeutic use , B-Lymphocytes/immunology , Treatment Outcome , T-Lymphocytes/immunology
3.
BMC Pulm Med ; 24(1): 310, 2024 Jul 02.
Article in English | MEDLINE | ID: mdl-38956567

ABSTRACT

BACKGROUND: Myasthenia gravis (MG) is the most common paraneoplastic disorder associated with thymic neoplasms. MG can develop after thymectomy, and this condition is referred to post-thymectomy myasthenia gravis (PTMG). Diffuse panbronchiolitis (DPB), is a rare form of bronchiolitis and is largely restricted to East Asia, has been reported in association with thymic neoplasms. Only three cases of combined MG and DPB have been reported in the literature. CASE PRESENTATION: A 45-year-old Taiwanese woman presented to our hospital with productive cough, rhinorrhea, anosmia, ear fullness, shortness of breath, and weight loss. She had a history of thymoma, and she underwent thymectomy with adjuvant radiotherapy 7 years ago. Chest computed tomography scan revealed diffuse bronchitis and bronchiolitis. DPB was confirmed after video-assisted thoracoscopic surgery lung biopsy, and repeated sputum cultures grew Pseudomonas aeruginosa. She has been on long-term oral azithromycin therapy thereafter. Intravenous antipseudomonal antibiotics, inhaled amikacin, as well as oral levofloxacin were administered. Three months after DPB diagnosis, she developed ptosis, muscle weakness, and hypercapnia requiring the use of noninvasive positive pressure ventilation. MG was diagnosed based on the acetylcholine receptor antibody and repetitive stimulation test results. Her muscle weakness gradually improved after pyridostigmine and corticosteroid therapies. Oral corticosteroids could be tapered off ten months after the diagnosis of MG. She is currently maintained on azithromycin, pyridostigmine, and inhaled amikacin therapies, with intravenous antibiotics administered occasionally during hospitalizations for respiratory infections. CONCLUSIONS: To our knowledge, this might be the first case report of sequential development of DPB followed by PTMG. The coexistence of these two disorders poses a therapeutic challenge for balancing infection control for DPB and immunosuppressant therapies for MG.


Subject(s)
Bronchiolitis , Myasthenia Gravis , Thymectomy , Thymus Neoplasms , Humans , Female , Myasthenia Gravis/etiology , Middle Aged , Bronchiolitis/etiology , Thymectomy/adverse effects , Thymus Neoplasms/surgery , Thymus Neoplasms/complications , Tomography, X-Ray Computed , Haemophilus Infections/etiology , Haemophilus Infections/diagnosis , Thymoma/surgery , Anti-Bacterial Agents/therapeutic use , Taiwan
4.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao ; 46(3): 462-465, 2024 Jun.
Article in Chinese | MEDLINE | ID: mdl-38953272

ABSTRACT

Intraspinal metastasis from malignant carcinomas in other body parts is rarely reported.Intraspinal metastases are often epidural,with primary tumors mostly from the lung and prostate.The extramedullary subdural metastasis of thymic carcinoma is particularly rare and prone to misdiagnosis due to overlapping imaging features with primary intraspinal tumors.This article reports one case of intraspinal metastasis of thymic carcinoma,with the main diagnostic clues including a history of thymic carcinoma,fast growth rate,and irregular shape.


Subject(s)
Thymoma , Thymus Neoplasms , Humans , Thymus Neoplasms/pathology , Thymus Neoplasms/diagnostic imaging , Male , Thymoma/pathology , Thymoma/diagnostic imaging , Thymoma/secondary , Spinal Neoplasms/secondary , Spinal Neoplasms/diagnostic imaging , Middle Aged
5.
BMJ Case Rep ; 17(6)2024 Jun 11.
Article in English | MEDLINE | ID: mdl-38862190

ABSTRACT

A young woman in her early 30s presented with a right thyroid mass and progressive hoarseness due to a right vocal cord palsy. The preoperative fine-needle aspiration cytology was classified as Bethesda V and she underwent a total thyroidectomy and neck dissection. Intraoperatively, the thyroid mass was adherent to the oesophagus, trachea and encasing the right recurrent laryngeal nerve which was sacrificed. Final histopathology diagnosed a rare subtype of thyroid cancer known as intrathyroidal thymic carcinoma (ITC). She was then sent for adjuvant radiotherapy after a multidisciplinary tumour board discussion. This case report highlights the difficulty in preoperative diagnosis of ITC and the importance of immunohistochemical staining in clinching the diagnosis. In view of its rarity, there have been no published consensus on the treatment of ITC, hence we would like to share some learning points through a comprehensive literature review.


Subject(s)
Thymus Neoplasms , Thyroid Neoplasms , Thyroidectomy , Humans , Female , Thyroid Neoplasms/pathology , Thyroid Neoplasms/diagnosis , Thyroid Neoplasms/surgery , Thymus Neoplasms/surgery , Thymus Neoplasms/diagnosis , Thymus Neoplasms/complications , Thymus Neoplasms/diagnostic imaging , Thymus Neoplasms/pathology , Adult , Biopsy, Fine-Needle , Thymoma/pathology , Thymoma/surgery , Thymoma/diagnostic imaging , Thymoma/diagnosis , Thymoma/complications , Neck Dissection , Radiotherapy, Adjuvant , Diagnosis, Differential , Hoarseness/etiology
6.
BMC Pulm Med ; 24(1): 280, 2024 Jun 14.
Article in English | MEDLINE | ID: mdl-38877486

ABSTRACT

BACKGROUND: The diagnostic complexities that arise in radiographic distinction between ectopic pleural thymoma and other thoracic neoplasms are substantial, with instances of co-occurring T-cell lymphocytosis and osseous metastasis being exceedingly rare. CASE PRESENTATION: A 51-year-old woman was admitted to our hospital with dyspnea and chest pain. Upon imaging examination, she was found to have diffuse and nodular pleural thickening on the left side, collapse of the left lung and a compression in the second thoracic vertebrae. All lesions showed significant 18F-FDG uptake on 18F-FDG PET/CT examination. Furthermore, she exhibited T-cell lymphocytosis in her peripheral blood, lymph nodes, and bone marrow. After ruling out malignant pleural mesothelioma (MPM), lung cancer with pleural metastasis, and T-cell lymphoma, the definitive diagnosis asserted was ectopic pleural thymoma with T-cell lymphocytosis and bone metastasis. CONCLUSION: Physicians need to expand their knowledge of the imaging features of ectopic pleural thymoma. Cases with T-cell lymphocytosis may exhibit increased aggressiveness and prone to bone metastasis.


Subject(s)
Bone Neoplasms , Lymphocytosis , Pleural Neoplasms , Thymoma , Humans , Female , Middle Aged , Thymoma/pathology , Thymoma/diagnostic imaging , Thymoma/complications , Thymoma/diagnosis , Lymphocytosis/pathology , Lymphocytosis/diagnosis , Pleural Neoplasms/secondary , Pleural Neoplasms/pathology , Pleural Neoplasms/complications , Pleural Neoplasms/diagnosis , Bone Neoplasms/secondary , Positron Emission Tomography Computed Tomography , Thymus Neoplasms/pathology , Thymus Neoplasms/complications , Thymus Neoplasms/diagnosis , T-Lymphocytes/pathology , Fluorodeoxyglucose F18 , Diagnosis, Differential , Pleura/pathology , Pleura/diagnostic imaging
7.
J Cardiothorac Surg ; 19(1): 318, 2024 Jun 04.
Article in English | MEDLINE | ID: mdl-38835049

ABSTRACT

Thymoma is a rare malignancy with usual location in the antero-superior mediastinum. Ectopic cervical thymoma (ECT) is an extremely rare tumor that originates from ectopic tissue, and is caused by the aberrant migration of the embryonic thymus. Our patient was a 56-year-old man who had a nodular lesion in the neck for several years. Computed tomography and Enhanced magnetic resonance imaging were performed. He underwent surgery, and a histological examination resulted in a diagnosis of type AB thymoma.


Subject(s)
Choristoma , Magnetic Resonance Imaging , Thymoma , Thymus Neoplasms , Tomography, X-Ray Computed , Humans , Male , Middle Aged , Thymoma/surgery , Thymoma/diagnosis , Thymoma/diagnostic imaging , Thymoma/pathology , Thymus Neoplasms/surgery , Thymus Neoplasms/diagnosis , Thymus Neoplasms/diagnostic imaging , Thymus Neoplasms/pathology , Choristoma/surgery , Choristoma/diagnosis , Choristoma/pathology , Choristoma/diagnostic imaging , Neck/diagnostic imaging , Head and Neck Neoplasms/surgery , Head and Neck Neoplasms/diagnosis , Head and Neck Neoplasms/pathology , Head and Neck Neoplasms/diagnostic imaging
8.
Kyobu Geka ; 77(5): 394-398, 2024 May.
Article in Japanese | MEDLINE | ID: mdl-38720610

ABSTRACT

A 73-year-old woman presented with left anterior chest pain and back pain. Computed tomography (CT) scan showed an anterior mediastinal tumor. It also showed partial anomalous pulmonary venous drainage (left superior pulmonary vein draining into the left brachiocephalic vein), and the tumor was located near the left brachiocephalic vein. The operation was performed through a median sternotomy to resect the thymus and tumor with partial resection of the left upper lobe due to the tumor's adhesion to the left upper lobe. One of the vascular anomalies encountered in adult thoracic surgery is partial anomalous pulmonary venous drainage. It is important to recognize the presence of such an anomaly on imaging and to anticipate the surgical procedure with a preoperative surgical technique.


Subject(s)
Pulmonary Veins , Thymoma , Thymus Neoplasms , Tomography, X-Ray Computed , Humans , Female , Aged , Pulmonary Veins/abnormalities , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/surgery , Thymoma/surgery , Thymoma/diagnostic imaging , Thymoma/complications , Thymus Neoplasms/surgery , Thymus Neoplasms/diagnostic imaging , Thymus Neoplasms/complications
9.
Indian J Pathol Microbiol ; 67(2): 422-424, 2024 Apr 01.
Article in English | MEDLINE | ID: mdl-38769812

ABSTRACT

ABSTRACT: Pyrexia of unknown origin can be caused due to numerous infective and noninfective causes. It poses a diagnostic dilemma to the clinicians and requires a myriad of investigations for the confirmation of diagnosis. Thymomas are rare mediastinal tumors that present as anterior mediastinal mass; however, thymomas presenting as pyrexia of unknown origin has rarely been reported in the literature. We report an interesting case of a middle-aged male who presented as pyrexia of unknown origin due to thymoma.


Subject(s)
Fever of Unknown Origin , Thymoma , Thymus Neoplasms , Humans , Thymoma/complications , Thymoma/diagnosis , Thymoma/pathology , Male , Fever of Unknown Origin/etiology , Thymus Neoplasms/complications , Thymus Neoplasms/diagnosis , Thymus Neoplasms/pathology , Middle Aged , Tomography, X-Ray Computed
10.
J Neuroinflammation ; 21(1): 126, 2024 May 11.
Article in English | MEDLINE | ID: mdl-38734662

ABSTRACT

Myasthenia gravis (MG) is an immune-mediated disease frequently associated with thymic changes. Increased T helper 17 (Th17) cell activity and dysfunctional regulatory T (Treg) cells have been demonstrated in subgroups of MG. On the other hand, hypoxia-inducible factor 1 (HIF-1) has been shown to regulate the Th17/Treg balance by inducing Th17 differentiation while attenuating Treg development. To identify the underlying mechanisms of different thymic pathologies in MG development, we evaluated thymic samples from thymoma-associated myasthenia gravis (TAMG), MG with hyperplasia (TFH-MG) and thymoma without MG (TOMA) patients. Differential gene expression analysis revealed that TAMG and TFH-MG cells are associated with different functional pathways. A higher RORC/FOXP3 ratio provided evidence for Th17/Treg imbalance in TAMG potentially related to increased HIF1A. The hypoxic microenvironment in thymoma may be a driver of TAMG by increasing HIF1A. These findings may lead to new therapeutic approaches targeting HIF1A in the development of TAMG.


Subject(s)
Hypoxia-Inducible Factor 1, alpha Subunit , Myasthenia Gravis , Th17 Cells , Thymoma , Female , Humans , Male , Hypoxia-Inducible Factor 1, alpha Subunit/genetics , Hypoxia-Inducible Factor 1, alpha Subunit/metabolism , Myasthenia Gravis/genetics , Myasthenia Gravis/immunology , Myasthenia Gravis/pathology , T-Lymphocytes, Regulatory/metabolism , T-Lymphocytes, Regulatory/immunology , Th17 Cells/metabolism , Th17 Cells/immunology , Thymoma/complications , Thymoma/genetics , Thymoma/immunology , Thymus Gland/pathology , Thymus Neoplasms/complications , Thymus Neoplasms/genetics
11.
Anticancer Res ; 44(6): 2511-2515, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38821590

ABSTRACT

BACKGROUND/AIM: Thymic carcinoma is a rare cancer type with limited treatment options. Our previous study demonstrated that statins, which inhibit 3-hydroxy-3-methylglutaryl coenzyme A reductase, can prevent thymic carcinoma. However, the mechanisms through which statins affect intracellular events in cancer cells are not well understood. The aim of the study was to determine how thymic carcinoma modulates the intracellular signals in response to statin administration. MATERIALS AND METHODS: We analyzed statin-induced protein phosphorylation in Ty82 human thymic carcinoma cells, which were cultured with fluvastatin, and protein phosphorylation was examined using western blotting. RESULTS: Treating Ty82 with fluvastatin led to ERK5 phosphorylation via protein prenylation attenuation. The antitumor effects of fluvastatin on thymic carcinoma were enhanced when combined with an ERK5 inhibitor. CONCLUSION: Statin therapy in combination with ERK5 inhibition may be a promising therapeutic approach for treating thymic carcinoma.


Subject(s)
Fatty Acids, Monounsaturated , Fluvastatin , Indoles , Mitogen-Activated Protein Kinase 7 , Thymus Neoplasms , Fluvastatin/pharmacology , Humans , Thymus Neoplasms/drug therapy , Thymus Neoplasms/pathology , Thymus Neoplasms/metabolism , Cell Line, Tumor , Mitogen-Activated Protein Kinase 7/metabolism , Mitogen-Activated Protein Kinase 7/antagonists & inhibitors , Phosphorylation/drug effects , Indoles/pharmacology , Fatty Acids, Monounsaturated/pharmacology , Thymoma/drug therapy , Thymoma/pathology , Hydroxymethylglutaryl-CoA Reductase Inhibitors/pharmacology , Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use , Animals
12.
Autoimmunity ; 57(1): 2347379, 2024 Apr 27.
Article in English | MEDLINE | ID: mdl-38723105

ABSTRACT

Thymoma is closely associated with myasthenia gravis (MG). However, due to the heterogeneity of thymoma and the intricate pathogenesis of MG, it remains unclear why some patients with thymoma develop MG and others do not. In this study, we conducted a comparative phenotype analysis of thymocytes in type B thymomas in patients with MG (MG (+) thymomas) and without MG (MG (-) thymomas) via fluorescence-activated cell sorting (FACS). Our results show that the developmental stages defined by the expression of CD3, CD4, and CD8 were largely maintained in both MG (+) and MG (-) thymomas, with CD4+CD8+ cells constituting the majority of thymocytes in type B thymoma, and no significant difference between this cell population was observed in MG (+) and MG (-) thymomas.We discovered that CD4+CD8+ thymocytes in MG (+) thymomas expressed low levels of αß TCR and high levels of IL-7 receptor α (IL-7Rα), whereas in MG (-) thymomas, CD4+CD8+ thymocytes exhibited the opposite pattern of αß TCR and IL-7Rα expression. These results suggest that the positive and negative selection processes of CD4+CD8+ thymocytes might differ between MG (+) thymomas and MG (-) thymomas. The expression of the Helios transcription factor is induced during negative selection and marks a group of T cells that have undergone negative selection and are likely to be deleted due to strong TCR binding with self-peptides/MHC ligands. We observed that the percentage of Helios-positive CD4SP T cells was greater in MG (-) than in MG (+) thymomas. Thus, the differentially regulated selection process of CD4+CD8+ thymocytes, which involves TCR and IL-7/IL-7Rα signaling, is associated with the presence of MG in type B thymomas.


Subject(s)
Myasthenia Gravis , Receptors, Antigen, T-Cell, alpha-beta , Thymocytes , Thymoma , Humans , Thymoma/immunology , Thymoma/pathology , Thymoma/metabolism , Myasthenia Gravis/immunology , Myasthenia Gravis/pathology , Myasthenia Gravis/metabolism , Receptors, Antigen, T-Cell, alpha-beta/metabolism , Male , Thymocytes/immunology , Thymocytes/metabolism , Female , Middle Aged , Receptors, Interleukin-7/metabolism , Receptors, Interleukin-7/immunology , Adult , Aged , Thymus Neoplasms/immunology , Thymus Neoplasms/pathology , Thymus Neoplasms/metabolism , CD4-Positive T-Lymphocytes/immunology , CD4-Positive T-Lymphocytes/metabolism , CD8-Positive T-Lymphocytes/immunology , CD8-Positive T-Lymphocytes/metabolism , Immunophenotyping
13.
Int J Rheum Dis ; 27(5): e15187, 2024 May.
Article in English | MEDLINE | ID: mdl-38742741

ABSTRACT

Thymic tumors are very rare neoplasms in children and account for less than 1% of mediastinal tumors in pediatric patients. One-third of the pediatric patients present with symptoms related to the compression of the tumor mass on the surrounding anatomic structures, and paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, acquired hypogammaglobulinemia, and connective tissue disorders, which rarely occur in children with thymic tumors. Herein, we report a case of thymic carcinoma mimicking the symptoms of a connective tissue disease with symmetrical polyarthritis accompanying myositis, fever, weight loss, and malaise in a 15-year-old male patient. To our knowledge, this is the first case pediatric thymic carcinoma accompany with severe polyarthritis and myopathy, thus we have reviewed the current literature regarding the cases of thymic malignancies coexisting with paraneoplastic syndromes in children.


Subject(s)
Arthritis , Myositis , Paraneoplastic Syndromes , Thymoma , Thymus Neoplasms , Humans , Male , Myositis/diagnosis , Myositis/complications , Paraneoplastic Syndromes/diagnosis , Paraneoplastic Syndromes/etiology , Thymus Neoplasms/complications , Thymus Neoplasms/diagnosis , Adolescent , Arthritis/diagnosis , Arthritis/etiology , Thymoma/complications , Thymoma/diagnosis , Treatment Outcome , Thymectomy , Biopsy
14.
Clin Immunol ; 263: 110233, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38697554

ABSTRACT

Ataxia-telangiectasia (A-T) is a rare disorder caused by genetic defects of A-T mutated (ATM) kinase, a key regulator of stress response, and characterized by neurodegeneration, immunodeficiency, and high incidence of cancer. Here we investigated NK cells in a mouse model of A-T (Atm-/-) showing that they are strongly impaired at killing tumor cells due to a block of early signaling events. On the other hand, in Atm-/- littermates with thymic lymphoma NK cell cytotoxicity is enhanced as compared with ATM-proficient mice, possibly via tumor-produced TNF-α. Results also suggest that expansion of exhausted NKG2D+ NK cells in Atm-/- mice is driven by low-level expression of stress-inducible NKG2D ligands, whereas development of thymoma expressing the high-affinity MULT1 ligand is associated with NKG2D down-regulation on NK cells. These results expand our understanding of immunodeficiency in A-T and encourage exploring NK cell biology in A-T patients in the attempt to identify cancer predictive biomarkers and novel therapeutic targets.


Subject(s)
Ataxia Telangiectasia Mutated Proteins , Killer Cells, Natural , NK Cell Lectin-Like Receptor Subfamily K , Animals , Killer Cells, Natural/immunology , Ataxia Telangiectasia Mutated Proteins/genetics , Ataxia Telangiectasia Mutated Proteins/metabolism , NK Cell Lectin-Like Receptor Subfamily K/genetics , NK Cell Lectin-Like Receptor Subfamily K/metabolism , Mice , Ataxia Telangiectasia/genetics , Ataxia Telangiectasia/immunology , Mice, Knockout , Mice, Inbred C57BL , Thymoma/immunology , Thymoma/genetics , Tumor Necrosis Factor-alpha/metabolism , Tumor Necrosis Factor-alpha/immunology , Cytotoxicity, Immunologic , Thymus Neoplasms/immunology , Thymus Neoplasms/genetics , Signal Transduction , Membrane Proteins , Histocompatibility Antigens Class I
15.
Rozhl Chir ; 103(2): 40-47, 2024.
Article in English | MEDLINE | ID: mdl-38697812

ABSTRACT

Thoracic surgery is increasingly influenced by the development of minimally invasive approaches which have also influenced surgery in the area of the anterior mediastinum. The previously standard approach to the thymus via partial sternotomy was gradually replaced by the videothoracoscopic approach in most cases. In recent years, robotically assisted surgery has been gaining ground worldwide in this area, as well. The aim of our paper is to provide a comprehensive overview of procedures in the field of the thymus, including their indications, and to share our first experience with robot-assisted thymus surgery. At the 3rd Department of Surgery, since the start of the robot-assisted thymus surgery program, 23 thymectomies have been performed using this approach, of which 17 were performed for thymoma, 3 for myasthenia gravis, and 3 for parathyroid adenoma localized in thymus tissue. From our experience and the available data, it follows that the length of hospitalization, the rate of complications and the resulting effect of robot-assisted procedures is comparable to VTS procedures; however, the robot-assisted surgery also allows for mini-invasive treatment even in significantly obese patients and in patients with advanced thymic tumors who would otherwise be indicated for open thymectomy.


Subject(s)
Myasthenia Gravis , Robotic Surgical Procedures , Thymectomy , Thymoma , Thymus Neoplasms , Humans , Robotic Surgical Procedures/methods , Thymectomy/methods , Thymus Neoplasms/surgery , Thymoma/surgery , Myasthenia Gravis/surgery , Parathyroid Neoplasms/surgery , Thymus Gland/surgery , Male
16.
BMC Cancer ; 24(1): 651, 2024 May 28.
Article in English | MEDLINE | ID: mdl-38807039

ABSTRACT

OBJECTIVES: This study aims to develop an innovative, deep model for thymoma risk stratification using preoperative CT images. Current algorithms predominantly focus on radiomic features or 2D deep features and require manual tumor segmentation by radiologists, limiting their practical applicability. METHODS: The deep model was trained and tested on a dataset comprising CT images from 147 patients (82 female; mean age, 54 years ± 10) who underwent surgical resection and received subsequent pathological confirmation. The eligible participants were divided into a training cohort (117 patients) and a testing cohort (30 patients) based on the CT scan time. The model consists of two stages: 3D tumor segmentation and risk stratification. The radiomic model and deep model (2D) were constructed for comparative analysis. Model performance was evaluated through dice coefficient, area under the curve (AUC), and accuracy. RESULTS: In both the training and testing cohorts, the deep model demonstrated better performance in differentiating thymoma risk, boasting AUCs of 0.998 and 0.893 respectively. This was compared to the radiomic model (AUCs of 0.773 and 0.769) and deep model (2D) (AUCs of 0.981 and 0.760). Notably, the deep model was capable of simultaneously identifying lesions, segmenting the region of interest (ROI), and differentiating the risk of thymoma on arterial phase CT images. Its diagnostic prowess outperformed that of the baseline model. CONCLUSIONS: The deep model has the potential to serve as an innovative decision-making tool, assisting on clinical prognosis evaluation and the discernment of suitable treatments for different thymoma pathological subtypes. KEY POINTS: • This study incorporated both tumor segmentation and risk stratification. • The deep model, using clinical and 3D deep features, effectively predicted thymoma risk. • The deep model improved AUCs by 16.1pt and 17.5pt compared to radiomic model and deep model (2D) respectively.


Subject(s)
Deep Learning , Thymoma , Thymus Neoplasms , Tomography, X-Ray Computed , Humans , Female , Thymoma/diagnostic imaging , Thymoma/pathology , Middle Aged , Male , Tomography, X-Ray Computed/methods , Risk Assessment/methods , Thymus Neoplasms/pathology , Thymus Neoplasms/diagnostic imaging , Adult , Aged , Retrospective Studies
17.
Medicine (Baltimore) ; 103(19): e37956, 2024 May 10.
Article in English | MEDLINE | ID: mdl-38728510

ABSTRACT

This study, based on a population, explored the prognostic value of postoperative radiotherapy (PORT) for Masaoka-Koga IIB stage thymomas. Patients diagnosed with thymoma from 2004 to 2017 in the Surveillance, Epidemiology, and End Results (SEER) database were included in the retrospective study. Through propensity score matching, the baseline characteristics of the patients were successfully matched to mitigate the selection bias of PORT. Survival rates and survival curves were compared between the PORT and non-PORT groups, with potential confounding factors addressed using a multivariate Cox regression model. In this study, 785 cases of IIB stage thymoma were included from the SEER database, and 303 patients were successfully matched between PORT and non-PORT groups through propensity score matching, with no significant differences in baseline characteristics. In the PORT and non-PORT groups, 10-year overall survival rates were 65.2% versus 59.6%, and cancer-specific survival rates were 87.0% vs. 84.4%, PORT did not yield statistically significant improvements in overall survival (P = .275) or cancer-specific survival (P = .336) for stage IIB thymomas. Based on the SEER database, the results of our study indicated that PORT does not confer a significant survival benefit for IIB stage thymomas.


Subject(s)
Neoplasm Staging , Propensity Score , SEER Program , Thymoma , Thymus Neoplasms , Humans , Thymoma/radiotherapy , Thymoma/mortality , Thymoma/surgery , Thymoma/pathology , Female , Male , Middle Aged , Retrospective Studies , Thymus Neoplasms/radiotherapy , Thymus Neoplasms/mortality , Thymus Neoplasms/pathology , Thymus Neoplasms/surgery , Aged , Adult , Radiotherapy, Adjuvant , Survival Rate , Prognosis
18.
BMC Cardiovasc Disord ; 24(1): 269, 2024 May 22.
Article in English | MEDLINE | ID: mdl-38778265

ABSTRACT

INTRODUCTION: Surgery remains the primary treatment modality for thymic carcinoma, with adjuvant radiotherapy being recommended to effectively mitigate local recurrence and metastasis rates subsequent to incomplete or complete resection. Chemoradiotherapy has the potential to induce coronary artery occlusion, thereby potentially impacting patients' long-term survival rates. The existing literature currently lacks comprehensive research on the lesion characteristics of coronary artery injury resulting from chemoradiotherapy. CASE PRESENTATION: The male patient, aged 55, was admitted to the hospital due to recurrent chest tightness and pain persisting for one week. Notably, the patient had previously undergone curative resection surgery for thymic carcinoma seven years ago. After the surgical procedure, the patient underwent a course of adjuvant chemotherapy comprising docetaxel and platinum. 11 months later, imaging examination diagnosed tumor recurrence, and concurrent chemoradiotherapy was administered at a total dose of 62 Gy/31F for planning gross target volume (PGTV) and 54 Gy/31F for planning target volume (PTV) with 2 cycles of paclitaxel and cisplatin. Re-admission of the patient occurred after a 7-year interval subsequent to the completion of concurrent chemoradiotherapy, leading to a subsequent diagnosis of acute non-ST segment elevation myocardial infarction. Following administration of antiplatelet, anticoagulant, and anti-myocardial ischemia therapy, coronary angiography revealed the presence of a bifurcation lesion at the distal end of the left main trunk. Intravascular ultrasound (IVUS) examination demonstrated significant negative remodeling of both the main trunk and its branches at the bifurcation site, characterized by minimal atherosclerotic plaque components. CONCLUSIONS: Chemoradiotherapy may induce damage to endothelial cells, resulting in an inflammatory response. Negative remodeling of blood vessels is likely to occur, primarily characterized by vasoconstriction but with less atherosclerotic plaque burden. Routine stent implantation in negatively remodeled areas may lead to vascular rupture, necessitating intravascular imaging examination.


Subject(s)
Thymoma , Thymus Neoplasms , Humans , Male , Thymus Neoplasms/therapy , Thymus Neoplasms/diagnostic imaging , Middle Aged , Treatment Outcome , Time Factors , Thymoma/therapy , Thymoma/diagnostic imaging , Coronary Angiography , Vascular System Injuries/etiology , Vascular System Injuries/diagnostic imaging , Vascular System Injuries/therapy , Coronary Vessels/diagnostic imaging , Coronary Vessels/injuries , Coronary Vessels/drug effects , Chemoradiotherapy/adverse effects
19.
BMC Neurol ; 24(1): 173, 2024 May 23.
Article in English | MEDLINE | ID: mdl-38783232

ABSTRACT

BACKGROUND: Ectopic cervical thymoma (ECT) is an extremely rare tumor, especially in association with myasthenia gravis (MG). CASE PRESENTATION: We report a case of myasthenia gravis with an ectopic thymoma in the neck, whose myasthenic symptoms significantly improved after complete removal of the mass. A 55-year-old woman with generalized myasthenia gravis (MG) experienced worsening neuromuscular weakness after abruptly discontinuing pyridostigmine. Testing revealed acetylcholine receptor-antibody (AChR-Ab) positivity and a cervical mass initially thought to be thyroid or parathyroid was identified as a thymoma, type A. Post-surgery and radiation therapy, her myasthenic symptoms improved significantly with less prednisone and pyridostigmine requirements over time and no need for additional immunotherapies. CONCLUSIONS: Diagnosing ECTs is challenging due to rarity, atypical locations, and inconclusive fine needle aspiration cytology (FNAC) results, often misinterpreted as thyroid or parathyroid lesions. As proper management of patients with MG, including thymectomy, offers favorable clinical outcomes such as significant improvement in myasthenic complaints and reduced immunosuppressive medication requirements, clinicians should be vigilant of the ectopic locations of thymomas to ensure timely diagnosis and intervention.


Subject(s)
Myasthenia Gravis , Thymoma , Humans , Female , Myasthenia Gravis/complications , Myasthenia Gravis/diagnosis , Middle Aged , Thymoma/complications , Thymoma/diagnosis , Thymus Neoplasms/complications , Thymus Neoplasms/diagnosis , Choristoma/complications , Choristoma/pathology
20.
Neurology ; 102(12): e209482, 2024 Jun 25.
Article in English | MEDLINE | ID: mdl-38781559

ABSTRACT

The role of immunosenescence, particularly the natural process of thymic involution during aging, is increasingly acknowledged as a factor contributing to the development of autoimmune diseases and cancer. Recently, a concern has been raised about deleterious consequences of the surgical removal of thymic tissue, including for patients who undergo thymectomy for myasthenia gravis (MG) or resection of a thymoma. This review adopts a multidisciplinary approach to scrutinize the evidence concerning the long-term risks of cancer and autoimmunity postthymectomy. We conclude that for patients with acetylcholine receptor antibody-positive MG and those diagnosed with thymoma, the removal of the thymus offers prominent benefits that well outweigh the potential risks. However, incidental removal of thymic tissue during other thoracic surgeries should be minimized whenever feasible.


Subject(s)
Myasthenia Gravis , Thymectomy , Thymoma , Thymus Gland , Thymus Neoplasms , Humans , Thymectomy/adverse effects , Thymectomy/methods , Myasthenia Gravis/surgery , Thymus Gland/surgery , Thymus Neoplasms/surgery , Thymus Neoplasms/complications , Thymoma/surgery , Thymoma/complications , Postoperative Complications/etiology , Autoimmune Diseases/surgery
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