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BMC Cancer Feb 2023The classification of thymomas is based on the morphology of epithelial tumor cells and the proportion of lymphocytes. Type A thymomas are composed of the spindle or...
BACKGROUND
The classification of thymomas is based on the morphology of epithelial tumor cells and the proportion of lymphocytes. Type A thymomas are composed of the spindle or oval tumor epithelial cells. Tumor cells of B thymomas are epithelioid-shaped with increasing atypia. Type AB thymomas have the features of epithelial tumor cells of A and B thymomas. The diagnosis can be difficult because of the complex morphology. Some novel thymic epithelial markers have been reported in several preclinical studies, but they have not been applied to clinical practice. Here, we investigated the expression of 3 cortical and 3 medullary markers, which are thymoproteasome-specific subunit β5t (β5t), thymus-specific serine protease 16 (PRSS16), cathepsin V, autoimmune regulator (AIRE), CD40 and claudin-4.
METHODS
Immunohistochemistry was used to analyze 53 cases of thymomas and thymic squamous cell carcinomas (TSCC), aiming to explore the expression of cortical and medullary epithelial markers and their correlation with histological classification, Masaoka-Koga stage, and prognosis.
RESULTS
Our results found that for cortical epithelial markers the expression of β5t, PRSS16, and cathepsin V was higher in type AB and B thymomas than in micronodular thymoma with lymphoid stroma (MNT), and we observed a dramatic increase of β5t and PRSS16 expression in type AB compared to type A thymomas. In medullary epithelial markers, the expression of AIRE was higher in type A than in B3 thymomas. CD40 and β5t expression were associated with the Masaoka-Koga stage. High cathepsin V expression was related to a good prognosis and a longer progression-free survival.
CONCLUSION
This is the first comprehensive analysis of the role of thymic cortical and medullary epithelial markers as biomarkers for differential diagnosis and prognosis in thymic tumors. Thymic medullary epithelial immunophenotype was found to exhibit in type A, MNT, and TSCC. Type B thymomas primarily exhibited a cortical epithelial immunophenotype. Type AB thymomas showed cortical, medullary, or mixed corticomedullary epithelial immunophenotype. Our results demonstrated that thymic cortical and medullary epithelial markers including β5t, PRSS16, cathepsin V, and AIRE could be used as ancillary markers in the diagnosis and prognosis of thymic epithelial tumors.
Topics: Humans; Thymoma; Thymus Neoplasms; Carcinoma, Squamous Cell; Neoplasms, Glandular and Epithelial; CD40 Antigens; Cathepsins
PubMed: 36797681
DOI: 10.1186/s12885-023-10619-6 -
The Indian Journal of Medical Research Nov 2020
Topics: Agammaglobulinemia; Humans; Thymoma; Thymus Neoplasms
PubMed: 35345211
DOI: 10.4103/ijmr.IJMR_2330_19 -
Asian Journal of Surgery Dec 2022The efficacy of radiotherapy for treating thymomas is unclear. The goal of this study was to analyze overall survival (OS) and disease-free survival (DFS) among thymoma...
PURPOSE
The efficacy of radiotherapy for treating thymomas is unclear. The goal of this study was to analyze overall survival (OS) and disease-free survival (DFS) among thymoma patients to determine the impact of postoperative radiotherapy (PORT) on thymoma outcomes.
METHODS
Recorded cases of thymoma at Xinqiao Hospital were retrospectively analyzed from 1991 to 2019. Data on stage II and III thymomas were extracted from medical records. This study evaluated OS and DFS and compared outcomes between surgery and surgery-plus-radiation groups. The Kaplan-Meier method and Cox regression analysis were used to compare DFS and OS for these groups.
RESULTS
Of the 205 patients included in the current study, 142 (69.3%) presented with stage II disease and 63 (30.7%) presented with stage III disease. The median follow-up was 84.3 months. PORT did not statistically significantly improve OS (P = 0.613) and DFS (P = 0.445) in stage II thymoma patients (compared with surgery alone). However, our subgroup analysis showed a statistically significant difference in DFS in patients with stage III thymoma (P = 0.044).
CONCLUSION
Although the routine use of postoperative radiotherapy in patients with thymoma does not appear warranted, patients with stage III thymoma may benefit from adjuvant radiation. These findings, if confirmed, will provide valuable information to guide medical decision-making for thymoma treatment.
Topics: Humans; Thymoma; Retrospective Studies; Neoplasm Staging; Survival Rate; Thymus Neoplasms
PubMed: 35227565
DOI: 10.1016/j.asjsur.2022.01.128 -
The Annals of Thoracic Surgery Jan 2021Thymomas are rare tumors, with limited data regarding treatment of advanced stage disease. Although surgical resection is the mainstay of treatment, the role of... (Comparative Study)
Comparative Study
BACKGROUND
Thymomas are rare tumors, with limited data regarding treatment of advanced stage disease. Although surgical resection is the mainstay of treatment, the role of additional therapy remains controversial. Our objectives were to describe treatment strategies for stage III/IV thymoma in the United States and compare survival outcomes among treatment approaches.
METHODS
We identified Masaoka stage III/IV thymoma reported in the National Cancer Database between 2004 and 2016. Frequencies of treatment with surgery, chemotherapy, radiation, and combinations were calculated. Five-year overall survival was compared using the Kaplan-Meier method and log-rank test. Risk-adjusted proportional hazards modeling compared mortality between treatment regimens.
RESULTS
A total of 1849 patients were identified (1108 stage III, 741 stage IV). Among stage III patients, 83.8% underwent resection (± other modalities) compared with 60.2% of stage IV. Surgery plus radiation was the most common regimen for stage III (32.6%), and nonsurgical treatment (definitive chemotherapy and/or radiation) was the most common for stage IV (36.4%). Overall 5-year survival was 70.3% for stage III and 58.5% for stage IV. In risk-adjusted analysis, surgery plus radiation had the lowest mortality (hazard ratio 0.41, 95% confidence interval 0.30-0.55). Patient age, tumor size, metastases, and non-academic treating hospital were associated with mortality.
CONCLUSIONS
Current treatment regimens for advanced stage thymoma vary significantly. Regimens that include surgical resection are most common and are associated with superior outcomes. Patients selected to have surgery as primary treatment had the best survival. Adjuvant radiation treatment is associated with better survival and should be considered in patients who undergo resection.
Topics: Adolescent; Adult; Aged; Female; Humans; Male; Middle Aged; Neoplasm Staging; Retrospective Studies; Thymoma; Thymus Neoplasms; Time Factors; Treatment Outcome; United States; Young Adult
PubMed: 32659263
DOI: 10.1016/j.athoracsur.2020.05.088 -
Asian Journal of Surgery Jan 2021To investigate clinical and histopathological characteristics of Vietnamese patients with thymoma and myasthenia gravis (MG), and the outcomes of surgical management...
OBJECTIVES
To investigate clinical and histopathological characteristics of Vietnamese patients with thymoma and myasthenia gravis (MG), and the outcomes of surgical management using video-assisted thoracoscopic surgery (VATS) thymectomy.
METHODS
A prospective study was carried out on 61 patients undergoing VATS thymectomy for MG class I, IIA with thymoma in the period from 10/2013 to 5/2019. The WHO histopathological classification, Masaoka's stages and MG grading using the guidelines of the Medical Scientific Advisory Board of the Myasthenia gravis foundation of America (MGFA) were used. All patients were followed up at 1 month, 6 months and over 1 year postoperatively.
RESULTS
The average patient age was 47.3 ± 10.8 years (21-70). The female/male ratio was 0.91.80.3% of patients had MG class IIA. Most of the patients were at Masaoka's stage I and stage II (75.4%). Only 1 patient (1.7%) had highly malignant type B3 thymoma. Conversion to open surgery was required in 8 patients. The surgical time was 91.8 ± 49.9 min and blood loss was 37.3 ± 31.5 ml. Most patients (68.9%) were extubated in the operating room. The postoperative hospital stay was 9.8 ± 5.9 days (5-37 days). 22.6% of patients relapsed after one-year. Refractory MG declined to 5.7% after surgical treatment.
CONCLUSION
VATS thymectomy for MG with thymoma was safe and effective, with a lower rate of intraoperative complications, shorter hospitalization, and better long-term outcomes. This approach could be applicable for patients of all age groups with thymomas at early Masaoka's stages.
Topics: Adult; Aged; Female; Follow-Up Studies; Humans; Intraoperative Complications; Length of Stay; Male; Middle Aged; Myasthenia Gravis; Neoplasm Staging; Prospective Studies; Safety; Thoracic Surgery, Video-Assisted; Thymectomy; Thymoma; Thymus Neoplasms; Time Factors; Treatment Outcome; Vietnam; Young Adult
PubMed: 33172689
DOI: 10.1016/j.asjsur.2020.10.006 -
World Journal of Surgical Oncology Feb 2015Malignant thymomas are rare epithelial neoplasms of the anterior superior mediastinum that are typically invasive in nature and have a higher risk of relapse that may...
Malignant thymomas are rare epithelial neoplasms of the anterior superior mediastinum that are typically invasive in nature and have a higher risk of relapse that may ultimately lead to death. Here we report a case of an advanced malignant thymoma that was successfully treated with neoadjuvant chemotherapy followed by surgical resection and subsequently with advanced and novel radiation therapy techniques. A 65-year-old male was diagnosed with a stage IV malignant thymoma with multiple metastatic lesions involving the left peripheral lung and pericardium. Initial neoadjuvant chemotherapy with a cisplatin-based regimen resulted in a partial response allowing the inoperable tumor to become operable. Following surgical resection of the residual disease, the tumor recurred within a year. The patient then underwent a course of targeted three-dimensional intensity modulated radiation therapy (IMRT) and image-guided radiation therapy (IGRT). Five years after radiation therapy, the localized soft tissue thickening at the left upper lung anterior pleural space had resolved. Seven years after radiation therapy the tumor mass had completely resolved. No recurrences were seen and the patient is well even 8 years after IMRT/IGRT with a favorable outcome. Chemotherapy with targeted three-dimensional IMRT/IGRT should be considered the primary modality for the management of advanced malignant thymoma patients.
Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Chemoradiotherapy; Disease Management; Humans; Lung Neoplasms; Male; Pericardium; Prognosis; Thymoma; Thymus Neoplasms
PubMed: 25889080
DOI: 10.1186/s12957-014-0427-z -
Journal of Thoracic Oncology : Official... Oct 2010Thymomas and thymic carcinomas (TC) are rare epithelial tumors of the thymus. Although most thymomas have organotypic features (i.e., resemble the normal thymus), TC are... (Review)
Review
Thymomas and thymic carcinomas (TC) are rare epithelial tumors of the thymus. Although most thymomas have organotypic features (i.e., resemble the normal thymus), TC are morphologically undistinguishable from carcinomas in other organs. Apart from their different morphology, TC and thymomas differ also in functional terms (TC, in contrast to thymomas, have lost the capacity to promote the maturation of intratumorous lymphocytes), have different genetic features (discussed in this review), a different immunoprofile (most TC overexpress c-KIT, whereas thymomas are consistently negative), and different clinical features (TC, in contrast to thymomas, are not associated with paraneoplastic myasthenia gravis). Thus, although all the data suggest that the biology of thymomas and TC is different, in clinical practice, their therapeutic management up to now is identical. In the age of personalized medicine, the time may have come to think this over. We will briefly review the molecular genetics of malignant thymic tumors, summarize the current status of targeted therapies with an emphasis on the multitargeted kinase inhibitors sunitinib and sorafenib, and try to outline some future directions.
Topics: Adult; Female; Humans; Liver Neoplasms; Middle Aged; Neoplasm Proteins; Prognosis; Thoracic Wall; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 20859121
DOI: 10.1097/JTO.0b013e3181f209a8 -
Ear, Nose, & Throat Journal Sep 2023Intrathyroidal thymic carcinoma (ITC) is a rare low-grade malignant thyroid tumor. There is neither sufficient understanding of this tumor nor its clinical treatment....
OBJECTIVE
Intrathyroidal thymic carcinoma (ITC) is a rare low-grade malignant thyroid tumor. There is neither sufficient understanding of this tumor nor its clinical treatment. This study is to explore the clinicopathological features, treatment, and prognosis of ITC and thereby provide a reference for the diagnosis and treatment of the disease.
METHODS
The clinical, pathological, therapeutic, and prognostic data of 13 patients with ITC were retrospectively analyzed.
RESULTS
The case series comprised 7 males and 6 females, with an average age of 51.9 ± 10.1 years. After surgical resection, all patients received post-operative neck radiotherapy at dosages of 60-66 Gy. Five patients with level VI lymph node metastasis additionally received 6 courses of cisplatin chemotherapy. All patients were followed-up for 21-132 months (median = 66 months), and all of them survived without recurrence or metastasis.
CONCLUSIONS
The diagnosis of ITC depends mainly on pathological and immunohistochemical results, particularly CD5 positive staining. Surgical resection is the preferred primary treatment modality which can be supplemented with radiotherapy and chemotherapy to reduce the risk of recurrence and metastasis.
Topics: Male; Female; Humans; Adult; Middle Aged; Thymoma; Retrospective Studies; Prognosis; Cisplatin; Thymus Neoplasms
PubMed: 36408572
DOI: 10.1177/01455613221141225 -
Journal of Thoracic Oncology : Official... Jul 2009Thymoma is a rare tumor for which there is little randomized evidence to guide treatment. Because of the lack of high-quality evidence, a formal consensus-based approach... (Review)
Review
INTRODUCTION
Thymoma is a rare tumor for which there is little randomized evidence to guide treatment. Because of the lack of high-quality evidence, a formal consensus-based approach was used to develop recommendations on treatment.
METHODS
A systematic refview of the literature was performed. Recommendations were formed from available evidence and developed through a two-round modified Delphi consensus approach.
RESULTS
The treatment recommendations are summarized as follows: Stage I--complete resection of the entire thymus without neoadjuvant or adjuvant therapy. Stage II--complete resection of the entire thymus with consideration of adjuvant radiation for high-risk tumors. Stage IIIA--surgery either initially or after neoadjuvant therapy, or surgery followed by adjuvant therapy. Stage IIIB--treatment may include a combination of chemotherapy, radiation, and/or surgery, or if technically possible, surgery in combination with chemoradiotherapy (concurrent cisplatin based). For bulky tumors, consideration should be given to sequential chemotherapy followed by radiation. Stage IVA--as per stage III, with surgery only if metastases can be resected. Stage IVB--treatment on an individual case basis (no generic recommendations). Recurrent disease--consider surgery, radiation, and/or chemoradiation. Chemoradiation should be considered in all medically inoperable and technically inoperable patients.
CONCLUSION
Consensus was achieved on these recommendations, which serve to provide practical guidance to the physician treating this rare disease.
Topics: Combined Modality Therapy; Delphi Technique; Humans; Neoplasm Metastasis; Neoplasm Staging; Thymoma; Thymus Neoplasms
PubMed: 19557895
DOI: 10.1097/jto.0b013e3181a4b8e0 -
European Journal of Cardio-thoracic... Jun 2014In thymomas, the roles of Masaoka-Koga stage, histology and the presence of myasthenia gravis (MG) have been considered fundamental for patient management and outcomes....
OBJECTIVES
In thymomas, the roles of Masaoka-Koga stage, histology and the presence of myasthenia gravis (MG) have been considered fundamental for patient management and outcomes. In this study, we retrospectively evaluated several clinical variables, with the aim of outlining their relationships and clinical/prognostic significance in resected thymoma patients.
METHODS
A retrospective search of our surgical database for patients operated on for thymoma in six Italian high-volume thoracic surgery centres between 2000 and 2011 was conducted. The following clinical variables were evaluated: Masaoka-Koga Stage, tumour histology, the presence of MG, other autoimmune syndromes or second tumours, the completeness of tumour resection and the development of recurrences.
RESULTS
Five hundred and thirty-seven (273 males-51%) were retrospectively included in this study. Our results indicate that: (i) MG correlates with early Masaoka-Koga stage and B-type thymoma; (ii) Stage III-IVa tumours correlate with B-type tumour; (iii) autoimmune paraneoplastic syndromes correlate with Stage I-II thymoma; (iv) second malignancies correlate with the absence of paraneoplastic disorders and weakly with B-type tumour and (v) overall survival was influenced by Masaoka-Koga stage and completeness of surgical resection.
CONCLUSIONS
In thymomas, Masaoka-Koga stage, histology, MG, other autoimmune syndromes and second malignancies are inter-related, but only Masaoka-Koga tumour stage, amid these clinical variables, has been demonstrated to be a strong prognostic indicator of survival.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Female; Humans; Italy; Kaplan-Meier Estimate; Male; Middle Aged; Myasthenia Gravis; Retrospective Studies; Thymoma; Thymus Neoplasms; Young Adult
PubMed: 24459215
DOI: 10.1093/ejcts/ezt567