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2.
J Cardiothorac Surg ; 19(1): 198, 2024 Apr 10.
Artigo em Inglês | MEDLINE | ID: mdl-38600577

RESUMO

This case report details a rare thymic basaloid carcinoma initially misinterpreted as a mediastinal teratoma, underscoring the diagnostic challenges posed by such tumors. A 71-year-old female presented with an asymptomatic anterior mediastinal tumor discovered incidentally during a routine health examination. Surgical intervention, followed by pathological and immunohistochemical analysis including CK-pan, p63, p40, and CD117 molecules, led to a definitive diagnosis of basaloid carcinoma of the thymus. This case highlights the critical importance of differential diagnosis in mediastinal lesions, especially those presenting with multilocular thymic cysts on chest CT. The subxiphoid video-assisted thoracoscopic surgery enabled complete tumor resection with minimal trauma and favorable postoperative outcomes. The patient opted against further radiotherapy or chemotherapy and she has survived for over eight months without recurrence. This case report contributes to the growing understanding of thymic basaloid carcinoma, a rare and potentially aggressive thymic carcinoma subtype. It emphasizes the necessity for precise surgical techniques and enhanced diagnostic acumen among cardiothoracic surgeons and oncologists.


Assuntos
Carcinoma de Células Escamosas , Cisto Mediastínico , Neoplasias do Mediastino , Teratoma , Timoma , Neoplasias do Timo , Feminino , Humanos , Idoso , Neoplasias do Mediastino/diagnóstico , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/cirurgia , Neoplasias do Timo/patologia , Cisto Mediastínico/diagnóstico , Cisto Mediastínico/cirurgia , Cisto Mediastínico/patologia , Timoma/patologia , Teratoma/diagnóstico
3.
J Cardiothorac Surg ; 19(1): 225, 2024 Apr 16.
Artigo em Inglês | MEDLINE | ID: mdl-38627811

RESUMO

BACKGROUND: The purpose of this study was to evaluate the clinicopathological characteristics of patients who underwent surgical resection for thymic neuroendocrine tumors (TNET) or thymic carcinoma. METHODS: In this study, we retrospectively evaluated the clinicopathological characteristics of our surgical patients at Fukuoka University Hospital from January 1995 to December 2018. RESULTS: There were nine cases of TNET and 16 cases of thymic carcinoma. Regarding the pathological type, the TNET group included three atypical carcinoid cases, two large cell neuroendocrine tumor cases, two small cell carcinoma cases, and two other cases. The thymic carcinoma group included 15 squamous carcinoma cases and one case of adenosquamous carcinoma. Based on the Masaoka-Koga staging system, six TNET cases and 11 thymic carcinoma cases were stage III or IV. The complete resection rate was 77% in the TNET group and 81% in the thymic carcinoma group. Additional chemotherapy and/or radiotherapy was performed in five cases of TNET and 11 cases of thymic carcinoma. The five-year survival rate and five-year disease-free survival rate were 87.5% and 75.0% in the TNET group and 58.9% and 57.1% in the thymic carcinoma group, respectively, with no significant difference between the two groups (P = 0.248 and P = 0.894, respectively). In the univariate analysis, complete resection was a statistically significant prognostic factor (P = 0.017). CONCLUSION: In this study, no difference in prognosis was observed between TNET and thymic carcinomas. To understand the characteristics of these tumors, further case accumulation and multicenter clinical studies are needed. (243words).


Assuntos
Neoplasias Pulmonares , Tumores Neuroendócrinos , Timoma , Neoplasias do Timo , Humanos , Timoma/patologia , Tumores Neuroendócrinos/cirurgia , Tumores Neuroendócrinos/patologia , Estudos Retrospectivos , Estadiamento de Neoplasias , Neoplasias do Timo/patologia , Prognóstico , Neoplasias Pulmonares/patologia
4.
Rev Mal Respir ; 41(4): 317-324, 2024 Apr.
Artigo em Francês | MEDLINE | ID: mdl-38461088

RESUMO

Primary thoracic cancers affect a large number of patients, mainly those with lung cancer and to a lesser extent those with pleural mesothelioma and thymic tumours. Given their frequency and associated comorbidities, in patients whose mean age is high, these diseases are associated with multiple complications. This article, the last of a series dedicated to emergencies in onco-haematological patients, aims to present a clinical picture of the severe complications (side effects, immune-related adverse events) associated with systemic treatments, excluding infections and respiratory emergencies, with which general practitioners and specialists can be confronted. New toxicities are to be expected with the implementation of innovative therapeutic approaches, such as CAR-T cells, along with immunomodulators and antibody-drug conjugates.


Assuntos
Neoplasias Pulmonares , Mesotelioma Maligno , Mesotelioma , Neoplasias Pleurais , Timoma , Humanos , Emergências , Mesotelioma/tratamento farmacológico , Timoma/patologia , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/tratamento farmacológico , Neoplasias Pleurais/tratamento farmacológico
5.
Folia Med (Plovdiv) ; 66(1): 142-146, 2024 Feb 29.
Artigo em Inglês | MEDLINE | ID: mdl-38426478

RESUMO

Resection and reconstruction of the superior vena cava (SVC) are required in a selected group of patients with anterior mediastinal tumors and lung neoplasms. We present the case of a 63-year-old woman who underwent invasive type B2 thymoma resection and a rare type of reconstruction of the superior vena cava using a patch of the left brachiocephalic vein (LBV). The various types of reconstruction of the superior vena cava are discussed.


Assuntos
Timoma , Neoplasias do Timo , Feminino , Humanos , Pessoa de Meia-Idade , Veia Cava Superior/diagnóstico por imagem , Veia Cava Superior/cirurgia , Veia Cava Superior/patologia , Veias Braquiocefálicas/diagnóstico por imagem , Veias Braquiocefálicas/cirurgia , Veias Braquiocefálicas/patologia , Mediastino/patologia , Timoma/diagnóstico por imagem , Timoma/cirurgia , Timoma/patologia , Neoplasias do Timo/diagnóstico por imagem , Neoplasias do Timo/cirurgia , Neoplasias do Timo/patologia
6.
Indian J Pathol Microbiol ; 67(1): 141-144, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38358204

RESUMO

Distinguishing T-lymphoblastic leukemia/lymphoma (T-ALL/T-LBL) from thymomas (especially B1 or B2 type) can be challenging particularly in limited trucut biopsy material where appreciating architecture is difficult or the background epithelial component does not provide tangible evidence for definite diagnosis. As a pathologist, it is important to accurately diagnose these neoplasms because they have entirely distinct management protocols. Recent studies have reported that LIM Domain Only 2 (LMO2) is expressed in neoplastic lymphoblasts of T-ALL/T-LBL and is absent in thymocytes of normal thymuses or thymomas. An observational study was done to test the sensitivity and specificity of LMO2 in differentiating neoplastic lymphoblasts from thymocytes of thymomas/normal thymuses. Our study showed that LMO2 had sensitivity of 70% and specificity of 100% in diagnosing LBL. None of the thymomas (B1 or B2 type) showed expression of LMO2 in the neoplastic cells. LMO2 is a reliable marker of transformed T-cell precursors and should be routinely included in immunohistochemical panel when evaluating thymic/mediastinal neoplasms.


Assuntos
Linfoma de Células T , Leucemia-Linfoma Linfoblástico de Células Precursoras , Leucemia-Linfoma Linfoblástico de Células T Precursoras , Timoma , Neoplasias do Timo , Humanos , Timoma/diagnóstico , Timoma/patologia , Imuno-Histoquímica , Leucemia-Linfoma Linfoblástico de Células T Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células T Precursoras/patologia , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/patologia , Proteínas Proto-Oncogênicas , Proteínas Adaptadoras de Transdução de Sinal , Proteínas com Domínio LIM/metabolismo
7.
Int J Mol Sci ; 25(3)2024 Jan 26.
Artigo em Inglês | MEDLINE | ID: mdl-38338833

RESUMO

Thymic epithelial tumors (TETs) are characterized by their extreme rarity and variable clinical presentation, with the inadequacy of the use of histological classification alone to distinguish biologically indolent from aggressive cases. The utilization of Next Generation Sequencing (NGS) to unravel the intricate genetic landscape of TETs could offer us a comprehensive understanding that is crucial for precise diagnoses, prognoses, and potential therapeutic strategies. Despite the low tumor mutational burden of TETS, NGS allows for exploration of specific genetic signatures contributing to TET onset and progression. Thymomas exhibit a limited mutational load, with prevalent GTF2I and HRAS mutations. On the other hand, thymic carcinomas (TCs) exhibit an elevated mutational burden, marked by frequent mutations in TP53 and genes associated with epigenetic regulation. Moreover, signaling pathway analyses highlight dysregulation in crucial cellular functions and pathways. Targeted therapies, and ongoing clinical trials show promising results, addressing challenges rooted in the scarcity of actionable mutations and limited genomic understanding. International collaborations and data-sharing initiatives are crucial for breakthroughs in TETs research.


Assuntos
Neoplasias Epiteliais e Glandulares , Timoma , Neoplasias do Timo , Humanos , Epigênese Genética , Neoplasias do Timo/genética , Neoplasias do Timo/patologia , Timoma/genética , Timoma/patologia , Neoplasias Epiteliais e Glandulares/genética
8.
Sci Rep ; 14(1): 2674, 2024 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-38302676

RESUMO

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.


Assuntos
Subpopulações de Linfócitos B , Miastenia Gravis , Timoma , Neoplasias do Timo , Humanos , Timoma/complicações , Timoma/patologia , Subpopulações de Linfócitos B/patologia , Neoplasias do Timo/complicações , Neoplasias do Timo/patologia , Linfócitos B/patologia , Miastenia Gravis/complicações , Microambiente Tumoral
9.
BMC Cancer ; 24(1): 92, 2024 Jan 17.
Artigo em Inglês | MEDLINE | ID: mdl-38233754

RESUMO

OBJECTIVE: The efficacy and safety of subxiphoid thoracoscopic thymectomy (SVATS) for early thymoma are unknown. The purposes of this meta-analysis were to evaluate the effectiveness and safety of SVATS for early thymoma, to compare it with unilateral intercostal approach video thoracoscopic surgery (IVATS) thymectomy, and to investigate the clinical efficacy of modified subxiphoid thoracoscopic thymectomy (MSVATS) for early anterior mediastinal thymoma. METHODS: Original articles describing subxiphoid and unilateral intercostal approaches for thoracoscopic thymectomy to treat early thymoma published up to March 2023 were searched from PubMed, Embase, and the Cochrane Library. Standardized mean differences (SMDs) and 95% confidence intervals (CIs) were calculated and analyzed for heterogeneity. Clinical data were retrospectively collected from all Masaoka stage I and II thymoma patients who underwent modified subxiphoid and unilateral intercostal approach thoracoscopic thymectomies between September 2020 and March 2023. The operative time, intraoperative bleeding, postoperative drainage, extubation time, postoperative hospital stay, postoperative visual analog pain score (VAS), and postoperative complications were compared, and the clinical advantages of the modified subxiphoid approach for early-stage anterior mediastinal thymoma were analyzed. RESULTS: A total of 1607 cases were included in the seven studies in this paper. Of these, 591 cases underwent SVATS thymectomies, and 1016 cases underwent IVATS thymectomies. SVATS thymectomy was compared with IVATS thymectomy in terms of age (SMD = - 0.09, 95% CI: -0.20 to - 0.03, I2 = 20%, p = 0.13), body mass index (BMI; SMD = - 0.10, 95% CI: -0.21 to - 0.01, I2 = 0%, p = 0.08), thymoma size (SMD = - 0.01, 95% CI: -0.01, I2 = 0%, p = 0.08), operative time (SMD = - 0.70, 95% CI: -1.43-0.03, I2 = 97%, p = 0.06), intraoperative bleeding (SMD = - 0.30. 95% CI: -0.66-0.06, I2 = 89%, p = 0.10), time to extubation (SMD = - 0.34, 95%CI: -0.73-0.05, I2 = 91%, p = 0.09), postoperative hospital stay (SMD = - 0.40, 95% CI: -0.93-0.12, I2 = 93%, p = 0.13), and postoperative complications (odds ratio [OR] = 0.94, 95% CI: 0.42-2.12, I2 = 57%, p = 0.88), which were not statistically significantly different between the SVATS and IVATS groups. However, the postoperative drainage in the SVATS group was less than that in the IVATS group (SMD = - 0.43, 95%CI: -0.84 to - 0.02, I2 = 88%, p = 0.04), and the difference was statistically significant. More importantly, the postoperative VAS was lower in the SVATS group on days 1 (SMD = - 1.73, 95%CI: -2.27 to - 1.19, I2 = 93%, p < 0.00001), 3 (SMD = - 1.88, 95%CI: -2.84 to - 0.81, I2 = 97%, p = 0.0005), and 7 (SMD = - 1.18, 95%CI: -2.28 to - 0.08, I2 = 97%, p = 0.04) than in the IVATS group, and these differences were statistically significant. A total of 117 patients undergoing thoracoscopic thymectomy for early thymoma in the Department of Thoracic Surgery of the Second Hospital of Jilin University were retrospectively collected and included in the analysis, for which a modified subxiphoid approach was used in 42 cases and a unilateral intercostal approach was used in 75 cases. The differences between the two groups (MSVATS vs. IVATS) in general clinical characteristics such as age, sex, tumor diameter, Masaoka stage, Word Health Organization (WHO) stage, and intraoperative and postoperative conditions, including operative time, postoperative drainage, extubation time, postoperative hospital stay, and postoperative complication rates, were not statistically significant (p > 0.05), while BMI, intraoperative bleeding, and VAS on postoperative days 1, 3, and 7 were all statistically significant (p < 0.05) in the MSVATS group compared with the IVATS group. CONCLUSION: The meta-analysis showed that the conventional subxiphoid approach was superior in terms of postoperative drainage and postoperative VAS pain scores compared with the unilateral intercostal approach. Moreover, the modified subxiphoid approach had significant advantages in intraoperative bleeding and postoperative VAS pain scores compared with the unilateral intercostal approach. These results indicate that MSVATS can provide more convenient operation conditions, a better pleural cavity view, and a more complete thymectomy in the treatment of early thymoma, indicating that is a safe and feasible minimally invasive surgical method.


Assuntos
Timoma , Neoplasias do Timo , Humanos , Timoma/patologia , Estudos Retrospectivos , Cirurgia Torácica Vídeoassistida/efeitos adversos , Cirurgia Torácica Vídeoassistida/métodos , Neoplasias do Timo/patologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Dor Pós-Operatória/etiologia
10.
Cancer Imaging ; 24(1): 5, 2024 Jan 04.
Artigo em Inglês | MEDLINE | ID: mdl-38178266

RESUMO

OBJECTIVE: To determine whether histogram analysis based on unenhanced CT can play a role in the differential diagnosis of thymoma and lymphoma from thymic hyperplasia and cyst (mean CT attenuation > 10 HU). MATERIALS AND METHODS: This retrospective study included consecutive asymptomatic participants who have prevascular mediastinal lesions incidentally detected by unenhanced CT between December 2013 and August 2020, and with definitive diagnosis by pathology or additional radiologic work-ups. A total of thirteen histogram parameters on enhanced CT were calculated for each lesion, then were compared between tumor (thymoma + lymphoma) and non-tumor (hyperplasia + cyst). Receiver operating characteristic analysis was conducted to investigate the performance of histogram parameter for identifying tumor. RESULTS: The study population included 192 patients (106 men and 86 women) with a mean age of 50.5 years at the time of CT examination. Of them, 94 patients have tumor (87 thymomas and 7 lymphoma) and 98 have non-tumor (48 thymic hyperplasia and 50 cysts). Nine of the thirteen histogram parameters revealed significant difference between the two groups, including median, minimum, range, 10th percentile, 90th percentile, kurtosis, skewness, uniformity and entropy. No significant difference was observed in the mean CT attenuation between groups. Higher median was found to be independent predictors for distinguishing tumor from non-tumor, and can achieve an area under the curve (AUC) of 0.785 (95% confidence interval [95% IC], 0.720-0.841). CONCLUSIONS: Histogram analysis based on unenhanced CT may be able to provide some help in the differential diagnosis of incidental lesions in prevascular mediastinal. GRAND SUPPORT: This study was sponsored by Natural Science Foundation of Shanghai (No. 21ZR1459700).


Assuntos
Cistos , Linfoma , Timoma , Hiperplasia do Timo , Neoplasias do Timo , Masculino , Humanos , Feminino , Pessoa de Meia-Idade , Timoma/diagnóstico por imagem , Timoma/patologia , Estudos Retrospectivos , China , Neoplasias do Timo/diagnóstico por imagem , Neoplasias do Timo/patologia , Linfoma/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Diagnóstico Diferencial
11.
J Thorac Oncol ; 19(1): 52-70, 2024 01.
Artigo em Inglês | MEDLINE | ID: mdl-37774950

RESUMO

INTRODUCTION: Stage classification is an important underpinning of management in patients with cancer and rests on a combination of three components-T for tumor extent, N for nodal involvement, and M for distant metastases. This article details the revision of the N and the M components of thymic epithelial tumors for the ninth edition of the TNM classification of malignant tumors proposed by the Thymic Domain of the International Association for the Study of Lung Cancer Staging and Prognostic Factors Committee. METHODS: The N and M components of the eighth edition staging system were verified by a large international collaborative data source through a data-driven analysis. A total of 9147 cases were included for analysis, including 7662 thymomas, 1345 thymic carcinomas, and 140 neuroendocrine thymic tumors. RESULTS: Lymph node involvement rates were 1.5% in thymomas and 17.6% and 27.7% in thymic carcinomas and neuroendocrine thymic tumors, respectively. Rates of lymph node metastasis were increasingly higher in tumors with higher T stage and higher-grade histologic type. Survival analysis validated the differences in the N and M categories proposed in the eighth edition staging system. Good discrimination in overall survival was detected among pathologic (p)N and pM categories in patients with thymoma and thymic carcinoma. CONCLUSIONS: No changes are proposed from the eighth edition for the N and M components. The proposed stage classification will provide a useful tool for management of the disease among the global thymic community.


Assuntos
Neoplasias Pulmonares , Neoplasias Epiteliais e Glandulares , Tumores Neuroendócrinos , Timoma , Neoplasias do Timo , Humanos , Estadiamento de Neoplasias , Neoplasias Pulmonares/patologia , Timoma/patologia , Proteínas do Mieloma , Neoplasias do Timo/patologia , Prognóstico , Neoplasias Epiteliais e Glandulares/patologia , Tumores Neuroendócrinos/patologia
12.
Thorac Cancer ; 15(2): 122-130, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38011005

RESUMO

BACKGROUND: Thymic epithelial tumors (TETs) are rare malignant tumors with limited treatment options. No established second-line treatment regimen is available following the preferred first-line chemotherapy, resulting in unsatisfactory efficacy and poor prognosis for patients with advanced TETs. This study aimed to evaluate the efficacy of small molecule multitarget antiangiogenic inhibitors as well as the prognostic factors for advanced TETs. METHODS: A retrospective study was conducted using data from a real-world database. Clinical information and survival follow-up data were collected from 52 patients with advanced TETs who received small molecule multitarget antiangiogenic inhibitors at Zhejiang Cancer Hospital between August 10, 2016 and August 10, 2022. The short-term efficacy of the treatments, survival time of the patients, and relevant prognostic factors of advanced TETs were analyzed. RESULTS: Out of the 52 patients included in this study, 16 had thymoma and 36 had thymic carcinoma. The 52 patients had an overall response rate of 21.1% and a disease control rate of 94.2%. In addition, the median progression-free survival (PFS) was 8.05 months, and the overall survival (OS) was 25.00 months. Apatinib was given to 33 patients, anlotinib to 15 patients, and sunitinib or lenvatinib to four patients. Only seven patients received antiangiogenic inhibitors as their first-line therapy, 27 patients as their second-line therapy, and 18 patients as third-line or subsequent therapy. Meanwhile, 42 patients received monotherapy with an antiangiogenesis inhibitor, while 10 patients received combination therapy. Univariate analysis indicated that the combined treatment was associated with a superior OS (p = 0.044); multivariate analysis indicated that the combined treatment was an independent prognostic factor for PFS (p = 0.014) and OS (p = 0.012). CONCLUSION: The findings suggest that small molecule multitarget antiangiogenic inhibitors are efficacious as second or post-line treatments as a viable alternative treatment option for patients with advanced TETs.


Assuntos
Neoplasias Epiteliais e Glandulares , Timoma , Neoplasias do Timo , Humanos , Estudos Retrospectivos , Neoplasias do Timo/patologia , Timoma/patologia , Neoplasias Epiteliais e Glandulares/tratamento farmacológico
13.
Eur J Cardiothorac Surg ; 65(2)2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38011656

RESUMO

OBJECTIVES: A study of tumour metabolic reprogramming has revealed disease biomarkers and avenues for therapeutic intervention. Metabolic reprogramming in thymoma is currently understudied and largely unknown. This study utilized metabolomics and isotope tracing with 13C-glucose to metabolically investigate thymomas, adjacent thymic tissue and benign thymic lesions. METHODS: From 2017 to 2021, 20 patients with a suspected thymoma were recruited to this prospective Institutional Review Board approved clinical trial. At the time of surgery, 11 patients were infused with 13C-glucose, a stable, non-radioactive tracer which reports the flow of carbon through metabolic pathways. Samples were analysed by mass spectrometry to measure the abundance of >200 metabolites.13C enrichment was measured in patients who received 13C-glucose infusions. RESULTS: Histological analysis showed that 9 patients had thymomas of diverse subtypes and 11 patients had benign cysts. In our metabolomic analysis, thymomas could be distinguished from both adjacent thymus tissue and benign lesions by metabolite abundances. Metabolites in pyrimidine biosynthesis and glycerophospholipid metabolism were differentially expressed across these tissues.13C-glucose infusions revealed differential labelling patterns in thymoma compared to benign cysts and normal thymus tissue. The lactate/3PG labelling ratio, a metabolic marker in aggressive lung tumours correlated with lactate uptake, was increased in thymomas (1.579) compared to normal thymus (0.945) and benign masses (0.807) (thymic tissue versus tumour P = 0.021, tumour versus benign P = 0.013). CONCLUSIONS: We report metabolic biomarkers, including differential 13C labelling of metabolites from central metabolism, that distinguish thymomas from benign tissues. Altered glucose and lactate metabolism warrant further investigation and may provide novel therapeutic targets for thymoma.


Assuntos
Cistos , Timoma , Neoplasias do Timo , Humanos , Timoma/diagnóstico , Timoma/cirurgia , Timoma/patologia , Estudos Prospectivos , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/cirurgia , Neoplasias do Timo/patologia , Biomarcadores , Glucose , Lactatos
14.
J Allergy Clin Immunol Pract ; 12(3): 744-752.e3, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38122866

RESUMO

BACKGROUND: Good's syndrome (GS) is a rare immune deficiency described almost 6 decades ago. Despite numerous published individual case reports and data collected in cross-sectional studies of small cohorts, the natural history and long-term outcomes of this disease remain unknown. OBJECTIVE: We aimed to determine the clinical and laboratory evolution of 8 adults diagnosed with GS and consecutively evaluated between 1983 and 2023. METHODS: In this prospective, longitudinal cohort study, newly diagnosed patients with GS were followed through repeated measures of clinical, immune, and hematologic changes, as well as targeted genetic screening. RESULTS: All patients reported a healthy childhood and adolescence with symptom onset during the third or fourth decade of life. All presented to our center with recurrent bacterial sinopulmonary infections, thymoma, hypogammaglobulinemia, and absence of B cells. The median age of GS diagnosis was 57 years. During follow-up, immunoglobin replacement therapy effectively minimized sinopulmonary infections. However, the majority experienced severe and systemic viral or fungal infections, 3 developed basal cell carcinomas, and 5 had progressive bronchiectasis and persistent splenomegaly. The most notable clinical feature was opportunistic infections and in vitro evidence of cellular immune deficiency, which resulted in the death of 2 individuals. We also report a statistically significant, multidecade progressive decline in lymphocytes, platelets, hemoglobin, and red blood cells in our cohort, suggesting gradual bone marrow failure. CONCLUSIONS: Knowledge of the unique phenotype and temporal evolution of GS has allowed us to develop a more comprehensive diagnostic framework. It can be investigated as part of broader research into disease pathophysiology.


Assuntos
Agamaglobulinemia , Síndromes de Imunodeficiência , Doenças da Imunodeficiência Primária , Timoma , Neoplasias do Timo , Adulto , Humanos , Criança , Pessoa de Meia-Idade , Estudos Prospectivos , Estudos Transversais , Estudos Longitudinais , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/patologia , Timoma/diagnóstico , Timoma/patologia , Agamaglobulinemia/diagnóstico , Agamaglobulinemia/terapia , Doenças da Imunodeficiência Primária/diagnóstico
15.
Histopathology ; 84(1): 196-215, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37994555

RESUMO

The epithelial and lymphoid compartments of the thymus can give rise to a wide variety of tumours, including thymomas, thymic carcinomas, lymphoreticular proliferations, germ cell tumours, and sarcomas. While some of these have close similarity to their counterparts in other organs, both in terms of histology and immunohistochemistry, as well as molecular features, others are unique to the thymus. The epithelial tumours, which can develop in the thymus, will be discussed in this review, with a particular emphasis on resolving differential diagnosis by means of morphology, immunohistochemical profiles, and molecular diagnostics.


Assuntos
Neoplasias Epiteliais e Glandulares , Timoma , Neoplasias do Timo , Humanos , Diagnóstico Diferencial , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/patologia , Timoma/diagnóstico , Timoma/patologia , Neoplasias Epiteliais e Glandulares/diagnóstico
16.
Diagn Cytopathol ; 52(2): 131-135, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38102931

RESUMO

Thymic epithelial tumours show characteristic cytological features on fine-needle aspiration cytology, however the cytological features of thymoma in fluid cytology are not well described. We present the case of a 77 year-old-woman with known pleural dissemination of type B2/B3 thymoma presenting with shortness of breath and orthopnoea due to a pleural effusion. Cytological evaluation of the pleural fluid showed cellular smears composed of numerous small lymphocytes with small numbers of admixed mesothelial cells. There was no epithelial component. On immunohistochemical (IHC) staining the lymphocytes were T cells which were positive for CD3. CD1a and terminal deoxynucleotide transferase (TdT) were also positive, consistent with immature lymphocytes of thymic origin. Despite the lack of an epithelial component, this case was diagnosed as suspicious for recurrent/ metastatic thymoma. This is only the second published case of thymoma identified on pleural fluid cytology, and to our knowledge the first case describing thymoma in pleural fluid with no epithelial component, a potential pitfall with the more common differential diagnosis of a reactive lymphocytic effusion.


Assuntos
Timoma , Neoplasias do Timo , Feminino , Humanos , Idoso , Timoma/diagnóstico , Timoma/patologia , Recidiva Local de Neoplasia/patologia , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/patologia , Timo/patologia , Linfócitos T/patologia
17.
Thorac Cancer ; 15(4): 339-346, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38149471

RESUMO

BACKGROUND: Thymic carcinoma is a rare disease with an incidence of around 0.5 cases per million with a poor prognosis. The aim of this study was to assess patient outcomes with advanced thymic carcinoma receiving first-line chemotherapy. METHODS: In our retrospective cohort study, we included patients who underwent treatment for metastatic thymic carcinoma between January 2013 to December 2019 in our hospital. Overall survival, progression-free survival (PFS), objective response rates (ORR) and chemotherapy regimens were assessed and analyzed. RESULTS: A total of 27 patients were retrospectively analyzed. All patients received a platinum (cisplatin or carboplatin) based regimen as first-line chemotherapy (29.6% received ADOC, 11.1% received PE, 40.7% received CP, 14.8% received CAP). The median PFS on first-line chemotherapy was 199 days. The response rate was 40.7%. Median overall survival (OS) was 585 days. Positive CD5 staining was associated with better PFS. CONCLUSION: We highlight the critical role of platinum-based chemotherapy agents as a primary treatment modality in advanced thymic carcinoma, underscoring the efficacy of platinum as a first-line option for recurrent disease, even in cases previously treated with platinum. Additionally, our findings indicate that CD5 positivity could be associated with improved PFS, suggesting its potential as a prognostic marker.


Assuntos
Antineoplásicos , Timoma , Neoplasias do Timo , Humanos , Timoma/tratamento farmacológico , Timoma/patologia , Estudos Retrospectivos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Antineoplásicos/uso terapêutico , Cisplatino/uso terapêutico , Platina/uso terapêutico , Neoplasias do Timo/tratamento farmacológico , Neoplasias do Timo/patologia , Resultado do Tratamento
18.
Int J Mol Sci ; 24(23)2023 Dec 02.
Artigo em Inglês | MEDLINE | ID: mdl-38069386

RESUMO

Angiogenesis significantly influences the carcinogenesis of thymic epithelial tumors (TET). Both thymomas and thymic carcinoma (TC) overexpress VEGF-A and VEGFR-1 and -2. This review aims to provide an appraisal of the use of anti-angiogenics in the treatment of TET. The literature research identified 16 studies that were deemed eligible for further analysis. Seven studies assessed the clinical efficacy of sunitinib and five studies the use of apatinib and/or anlotinib. The multicenter Japanese phase II REMORA trial investigated the efficacy of lenvatinib, which is a multi-targeted inhibitor of VEGFR, FGFR, RET, c-Kit, and other kinases. The objective response rate was 38% (25.6-52%), which is the highest documented in TET that progressed after first-line chemotherapy. Anti-angiogenic agents may be useful in the treatment of TET, which are not amenable to curative treatment. Their toxicity profile seems to be acceptable. However, angiogenesis inhibitors do not appear to have a major influence on either thymomas or TC, although multikinase inhibitors may have some effect on TC. The current evidence suggests that the most active agent is lenvatinib, whereas sunitinib could be proposed as an acceptable second-line therapy for TC. Further research concerning the combination of immune checkpoint inhibitors with anti-angiogenic drugs is warranted.


Assuntos
Neoplasias Epiteliais e Glandulares , Timoma , Neoplasias do Timo , Humanos , Timoma/tratamento farmacológico , Timoma/patologia , Inibidores da Angiogênese/farmacologia , Inibidores da Angiogênese/uso terapêutico , Sunitinibe/uso terapêutico , Neoplasias do Timo/tratamento farmacológico , Neoplasias do Timo/patologia , Neoplasias Epiteliais e Glandulares/tratamento farmacológico , Estudos Multicêntricos como Assunto
19.
Oncol Res Treat ; 46(12): 520-529, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37963448

RESUMO

INTRODUCTION: Thymoma is a common mediastinal tumor, but few studies have been performed in thymoma patients 80 years or older. This study aimed to analyze the clinical features, treatment modalities, and survival outcomes of thymoma patients at least 80 years old and compare these features to those of patients younger than 80 years old. METHOD: Data from thymoma patients in the Surveillance, Epidemiology and End Results database between 2000 and 2019 were selected. Clinical features, treatment modalities of the two age groups were compared. Survival rates were calculated by the Kaplan-Meier method and the log-rank test was used to compare survival rates between two groups. Propensity score matching was used based on whether surgery was performed. Univariate and multivariate Cox proportional-hazards regression analyses were performed to identify independent prognostic factors. RESULTS: Compared with the younger patients, the patients aged 80 years or older had a similar distribution of Masaoka-Koga tumor stage, a higher proportion of type A thymoma, and a lower recurrence rate in the early stage. In elderly patients after propensity score matching, the overall survival and cancer-specific survival were better in the surgery group with complete resection and compared with patients of different ages, elderly patients showed similar benefit from surgery as younger patients were observed. CONCLUSION: In thymoma patients aged 80 years or older, surgery still plays an important role in survival outcome. Compared with younger patients, older patients have unique clinical features.


Assuntos
Timoma , Neoplasias do Timo , Idoso , Humanos , Idoso de 80 Anos ou mais , Timoma/terapia , Timoma/patologia , Estadiamento de Neoplasias , Neoplasias do Timo/terapia , Neoplasias do Timo/patologia , Radioterapia Adjuvante , Pontuação de Propensão , Prognóstico , Estudos Retrospectivos
20.
Am J Case Rep ; 24: e941792, 2023 Nov 25.
Artigo em Inglês | MEDLINE | ID: mdl-38006204

RESUMO

BACKGROUND Myasthenia gravis is a neuromuscular disorder that is strongly associated with thymoma. Although the presence of myasthenia gravis with other tumors is uncommon, approximately 50% of patients with thymoma have myasthenia gravis. Thymic Hodgkin lymphoma should be considered due to the multiple reported cases of patients with myasthenia gravis and Hodgkin lymphoma. In this report, we present the case of 24-year-old woman with myasthenia gravis who was incidentally found to have coexisting thymoma with thymic Hodgkin lymphoma. CASE REPORT A 24-year-old woman with a known case of vitiligo presented with a 2-year history of diplopia and incidental anterior mediastinal mass. Following investigations, myasthenia gravis was diagnosed and managed by pyridostigmine, prednisolone, and azathioprine. Regarding the anterior mediastinal mass, thymoma was suspected based on the presence of myasthenia gravis and radiological findings. She underwent extended transsternal thymectomy. The final histopathological report of the dissected thymus disclosed Hodgkin lymphoma pathology coexisting with thymoma. After the diagnosis of Hodgkin lymphoma nodular sclerosis type IIA was confirmed, 6 cycles of chemotherapy were administered. Four years of follow-up revealed no evidence of Hodgkin lymphoma. However, her symptoms of myasthenia gravis persisted despite Hodgkin lymphoma remission. CONCLUSIONS There is an unclear association between myasthenia gravies and Hodgkin lymphoma. Prior reports revealed regression of myasthenia gravies following Hodgkin lymphoma management, which suggests that myasthenia could be a complication of Hodgkin lymphoma. However, in our case, myasthenia gravis persisted after Hodgkin lymphoma management; therefore, further studies are needed to explore this association.


Assuntos
Doença de Hodgkin , Miastenia Gravis , Timoma , Neoplasias do Timo , Feminino , Humanos , Adulto Jovem , Doença de Hodgkin/complicações , Doença de Hodgkin/diagnóstico , Miastenia Gravis/complicações , Miastenia Gravis/diagnóstico , Brometo de Piridostigmina/uso terapêutico , Timoma/complicações , Timoma/diagnóstico , Timoma/patologia , Neoplasias do Timo/complicações , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/patologia
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