-
The Journal of Thoracic and... Apr 2021
Topics: Humans; Neoplasm Staging; Thymoma; Thymus Neoplasms
PubMed: 33293061
DOI: 10.1016/j.jtcvs.2020.11.009 -
Oncology (Williston Park, N.Y.) Mar 2022Thymoma is an uncommon intrathoracic malignant tumor and has a long natural history, with overall survival (OS) in these patients lasting decades. It is uncertain...
BACKGROUND
Thymoma is an uncommon intrathoracic malignant tumor and has a long natural history, with overall survival (OS) in these patients lasting decades. It is uncertain whether the survival of a patient with thymoma is affected by their prior cancer history (PCH). Finding out the impact of PCH on thymoma survival has important implications for both decision-making and research.
METHODS
The Surveillance, Epidemiology, and End Results database was queried for patients with thymoma diagnosed between 1975 and 2015. Kaplan-Meier methods and the Cox proportional hazards model were used to analyze OS across a variety of stages, ages, and treatment methods, in patients both with and without PCH.
RESULTS
A total of 3604 patients with thymoma were identified, including 507 (14.1%) with a PCH. The 10-year survival rate of patients with a PCH (53.8%) was worse than that of those without a PCH (40.32%; 95% CI, 35.24%-45.33%; P < .0001). However, adjusted analyses showed that the impact of a PCH was heterogenous across ages and treatment methods. In subset analyses, PCH was associated with worse survival among patients who were treated with chemoradiotherapy (HR, 2.80; 95% CI, 1.51-5.20; P = .001) and among those who were ≤ 65 years (HR, 1.33; 95% CI, 1.02-1.73; P = .036).
CONCLUSIONS
PCH provides an inferior OS for patients with thymoma. However, it does not worsen the survival in some subgroups, and these patients with thymoma may be eligible for study.
Topics: Databases, Factual; Humans; Neoplasm Staging; Prognosis; Survival Rate; Thymoma; Thymus Neoplasms
PubMed: 35298120
DOI: 10.46883/25920953 -
Scientific Reports Mar 2020To evaluate the role of conventional contrast-enhanced CT (CECT) imaging and dual-energy spectral CT (DECT) perfusion imaging in differentiating the WHO histological... (Comparative Study)
Comparative Study
To evaluate the role of conventional contrast-enhanced CT (CECT) imaging and dual-energy spectral CT (DECT) perfusion imaging in differentiating the WHO histological subtypes of thymic epithelial tumours (TETs). Eighty-eight patients with TETs who underwent DECT perfusion scans (n = 51) and conventional CT enhancement scans (n = 37) using a GE Discovery CT750 HD scanner were enrolled in this study. The mean maximal contrast-enhanced range (mean CEmax) and the perfusion and spectral parameters of the lesions were analysed. Among the six WHO subtypes (Type A, AB, B1, B2, and B3 thymoma and thymic carcinoma), the mean CEmax values and most of the perfusion and spectral parameter values of Type A and Type AB were significantly higher than those of the other subtypes (all P < 0.05), and there was no difference among Type B1, B2 and B3 (all P > 0.05). The mean CEmax value was not different between Type B (including Type B1, B2, and B3) and thymic carcinoma (P = 1.000). The PS, IC, NIC and λ values in the optimal venous phase of thymic carcinoma were higher than those of Type B (all P < 0.05). The parameters of conventional CECT imaging and DECT perfusion imaging can help identify the subtype of TETs, especially those of DECT perfusion imaging in type B thymomas and thymic carcinomas.
Topics: Adult; Aged; Contrast Media; Female; Humans; Male; Middle Aged; Neoplasms, Glandular and Epithelial; Perfusion Imaging; ROC Curve; Retrospective Studies; Thymoma; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 32218504
DOI: 10.1038/s41598-020-62466-1 -
Annals of Surgical Oncology Jul 2023
Topics: Humans; Thymoma; Prognosis; Thymus Neoplasms; Neoplasm Staging; Retrospective Studies
PubMed: 37067743
DOI: 10.1245/s10434-023-13445-z -
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 -
Medicine Dec 2022Thymic epithelial tumors (TETs), including thymomas and thymic carcinomas. Thymomas are rare tumors, but are one of the most common mediastinal neoplasms in adults. The...
INTRODUCTION
Thymic epithelial tumors (TETs), including thymomas and thymic carcinomas. Thymomas are rare tumors, but are one of the most common mediastinal neoplasms in adults. The pathology of thymoma with Systemic Lupus Erythematosus (SLE) and severe pancytopenia is rarely reported. Thus, the current study demonstrates a case of thymoma with SLE and immune-related anemia with unique clinical manifestation.
PATIENT CONCERNS
A 27-year-old woman suffered from abdominal pain, arthralgia, intermittent high fever for a long time.
DIAGNOSIS
Based on the clinical and histopathological manifestations, diagnosis of thymoma with SLE and immune-related anemia was established.
INTERVENTIONS
Patient was treated with methylprednisolone and a complete thymectomy and thymomectomy, the CAP regimen was given 4 times of adjuvant chemotherapy after the operation.
OUTCOMES
After inter-disciplinary consultation as well as extensive discussion and steroid pulse therapy underwent surgery, the patient's blood count and immune function gradually entry sent back to normal.
CONCLUSION
we present the diagnosis and treatment of a case of thymoma with SLE and immune-related anemia, and provides references for the clinical diagnosis and treatment of thymoma combined with SLE, and attempts to explain that SLE patients with thymoma may contribute to the clinical remission of SLE after thymoma resection. It should arouse the attention of clinicians when diagnosing and treating related diseases.
Topics: Adult; Female; Humans; Thymoma; Thymus Neoplasms; Lupus Erythematosus, Systemic; Methylprednisolone; Anemia
PubMed: 36626535
DOI: 10.1097/MD.0000000000032077 -
The Annals of Thoracic Surgery Aug 2014This meta-analysis was designed to determine the effect of surgical and nonsurgical approaches on 5-year and 10-year overall survival (OS) in patients with recurrent... (Comparative Study)
Comparative Study Meta-Analysis Review
This meta-analysis was designed to determine the effect of surgical and nonsurgical approaches on 5-year and 10-year overall survival (OS) in patients with recurrent thymoma. PubMed, Scopus, and the Journal of Japanese Association for Chest Surgery were queried, and 11 studies were eligible. Our meta-analysis using a random-effect model revealed significant differences in the rates of 5- and 10-year OS after thymectomy and in 5-year OS after recurrence, favoring surgically managed patients. Surgical resection may be associated with improved long-term survival and should be considered for patients with recurrent thymoma.
Topics: Checklist; Humans; Survival Rate; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 24980604
DOI: 10.1016/j.athoracsur.2014.04.028 -
Journal of Oncology Pharmacy Practice :... Oct 2019Thymic carcinoma is a rare entity and can be distinguished from benign thymomas by their aggressive nature and poor prognosis. The National Comprehensive Cancer Network... (Review)
Review
Thymic carcinoma is a rare entity and can be distinguished from benign thymomas by their aggressive nature and poor prognosis. The National Comprehensive Cancer Network guidelines recommend resection followed by adjuvant platinum-based chemotherapy for resectable tumors. However, the outcomes for metastatic or relapsed thymic carcinomas are poor with no regimen showing a consistent benefit. Moreover, the relative rarity of these tumors makes clinical trials difficult. Molecular analysis of thymomas shows a high incidence of genetic mutations and targeted therapy holds promise. We will briefly outline and review the current role of targeted therapy in thymic cancer.
Topics: Chemotherapy, Adjuvant; Combined Modality Therapy; Humans; Molecular Targeted Therapy; Mutation; Thymoma; Thymus Neoplasms
PubMed: 31159657
DOI: 10.1177/1078155219852758 -
Histopathology Nov 2023Metaplastic thymoma is a rare thymic tumour characterized by Yes Associated Protein 1 (YAP1) and Mastermind Like Transcriptional Coactivator 2 (MAML2) gene fusions...
AIMS
Metaplastic thymoma is a rare thymic tumour characterized by Yes Associated Protein 1 (YAP1) and Mastermind Like Transcriptional Coactivator 2 (MAML2) gene fusions resulting from an intrachromosomal inversion of chromosome 11. Immunohistochemistry with an antibody directed against the C-terminus of YAP1 has shown loss of expression in YAP1-rearranged vascular neoplasms, poromas, and porocarcinomas. This study aimed to validate an anti-YAP1 C-terminal antibody as an ancillary immunohistochemical marker for the diagnosis of metaplastic thymoma.
MATERIALS AND METHODS
Ten metaplastic thymomas were selected for the current study. Fluorescence in situ hybridization (FISH), next-generation sequencing (NGS), and reverse transcription-polymerase chain reaction (RT-PCR) analyses were performed to detect YAP1::MAML2 fusions. We then performed immunohistochemistry to detect YAP1 C-terminus expression in 10 metaplastic thymomas, 50 conventional thymomas (10 each of type A thymoma, type AB thymoma, type B1 thymoma, type B2 thymoma, and type B3 thymoma) and seven thymic carcinomas.
RESULTS
All 10 cases showed narrow split signals with a distance of nearly two signal diameters and sometimes had false-negative results in YAP1 and MAML2 break-apart FISH (BA-FISH). Abnormal colocalized signals of the YAP1::MAML2 fusion were observed in all 10 cases using fusion FISH (F-FISH) assays. Eight of 10 cases with adequate nucleic acids were successfully sequenced and all showed YAP1::MAML2 fusions; in two cases the fusions were detected by both DNA and RNA sequencing and in six cases by RNA sequencing only. YAP1::MAML2 fusion transcripts were identified in four cases by RT-PCR. Metaplastic thymoma showed loss of YAP1 C-terminus expression in all 10 (100%) cases. All other thymic neoplasms showed retained YAP1 C-terminus expression.
CONCLUSION
YAP1 C-terminus immunohistochemistry is a highly sensitive and specific ancillary marker that distinguishes metaplastic thymoma from its mimics. BA-FISH assays could not effectively detect YAP1::MAML2 fusions due to the proximity of the two genes. Loss of YAP1 C-terminus expression is a reliable surrogate for the detection of YAP1::MAML2 fusions in metaplastic thymoma.
Topics: Humans; Thymoma; In Situ Hybridization, Fluorescence; Transcription Factors; Thymus Neoplasms; Adaptor Proteins, Signal Transducing; Gene Rearrangement; Trans-Activators
PubMed: 37565303
DOI: 10.1111/his.15024 -
European Journal of Cardio-thoracic... Oct 2021The goal of this study was to analyse the outcomes in 53 patients with thymoma, 34 of whom had myasthenia gravis (MG), who were treated with robotic surgery. The...
OBJECTIVES
The goal of this study was to analyse the outcomes in 53 patients with thymoma, 34 of whom had myasthenia gravis (MG), who were treated with robotic surgery. The oncological outcomes of the whole series of patients were analysed. Furthermore, because consistent data are not yet available in the literature, the main focus was the analysis of the neurological results of the patients affected by MG and thymoma.
METHODS
The clinical outcomes of 53 patients with a diagnosis of thymoma who underwent robotic thymectomy between January 2014 and December 2019 in our institution were collected and evaluated; 34 of these patients had a concomitant diagnosis of MG. The neurological status of the patients was determined from a clinical evaluation according to the Osserman classification and on pre- and post-surgery Myasthenia Gravis Composite scores, whereas neurological clinical outcomes were assessed using the Myasthenia Gravis Foundation of America Post-Intervention Score. Reduction of steroid therapy was also considered. The recurrence rate, adjuvant radiotherapy and overall survival of the patients with a thymoma were evaluated.
RESULTS
Neurological outcomes: improvement of the clinical conditions was obtained in 26 patients (76.5%) following the operation: complete stable remission was observed in 5 patients (14.7%), pharmacological remission in 10 (29.4%) and minimal manifestation in 11 (32.3%). Four patients (11.8%) exhibited no substantial change from the pretreatment clinical manifestations or reduction in MG medication and 4 (11.8%) patients experienced worsening of clinical conditions. In 21 patients (61.7%) a reduction of the dosage of steroid therapy was obtained. Oncological outcomes: at an average follow-up of 36 months, the overall survival was 96%, 4 patients (7.5%) had pleural relapses and 12 patients (22.6%) underwent postoperative radiotherapy, according to their stage. In accordance with Masaoka staging, 34% were in stage I, 56.6% in stage II and 9.4% in stage III.
CONCLUSIONS
Our results suggest that robotic surgical treatment of patients with thymoma and concomitant MG is effective in improving the neurological outcomes. Moreover, the oncological results obtained in this series confirm the efficacy of robotic surgery for the treatment of thymic malignancies, with results in line with those of open surgery. However, due to the indolent growth of thymomas, further observations with longer follow-up are necessary.
Topics: Humans; Myasthenia Gravis; Neoplasm Recurrence, Local; Retrospective Studies; Robotic Surgical Procedures; Thymectomy; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 34263301
DOI: 10.1093/ejcts/ezab253