Thymoma and Thymic Carcinoma
The thymus is a small organ located beneath the breastbone, it is part of the immune system and produces T-lymphocytes - cells which kill viruses and signal B-lymphocytes to make antibodies to fight infection. About 90 percent of tumors that start in the thymus are thymomas. Thyomas begin in the cells which line the thymus (epithelial cells) and look similar to normal thymus cells under the microscope. Thymomas range from slow growing tumors that rarely spread outside the thymus, to more aggressive tumors, which can potentially spread to nearby organs in the chest, such as the lining of the lung (the pleura). Thymic Carcinoma also starts in the cells which line the thymus, but looks different to normal thymus cells under the microscope. These are more likely to spread to other parts of the body. Thymic Carcinoids are a rarer type of tumour which start in hormone-producing cells. Thymic carcinoids are sometimes associated with a rare genetic disorder: multiple endocrine neoplasia type 1 (MEN-1) syndrome. Other rare types include Thymolipoma and primary thymic lymphoma.
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MeSH term: Thymoma
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An Unusual Case of Chest Pain in an Adolescent Male: Important Cues to Differential Diagnosis.
Adv Emerg Nurs J. 2017 Jan/Mar; 39(1):10-17 [PubMed] Related Publications
Acute pancreatitis after thoracic duct ligation for iatrogenic chylothorax. A case report.
BMC Surg. 2017; 17(1):9 [PubMed] Free Access to Full Article Related Publications
CASE PRESENTATION: A 57-year-old woman operated for thymoma presented a iatrogenic chylothorax. After thoracic duct ligation, she presented an acute pancreatitis which resolved after conservative treatment. The chylothorax disappeared within 4 days of thoracic duct ligation.
CONCLUSIONS: This is the first report of acute pancreatitis following thoracic duct ligation. The pancreas and digestive tract should be assessed in symptomatic patients after thoracic duct ligation.
Nephrotic syndrome associated with metastatic thymoma treated with chemotherapy.
Medicine (Baltimore). 2017; 96(1):e5408 [PubMed] Free Access to Full Article Related Publications
PATIENT CONCERNS AND DIAGNOSES: We report a 32-year-old female with nephrotic syndrome that was first diagnosed along with invasive thymoma and treated by means of cisplatin-based chemotherapy for the thymoma. The patient initially presented with dyspnea and generalized edema. Chest radiography and computed tomography scans revealed right pleural effusion and a mass in the right middle lung field, which were confirmed by a percutaneous lung biopsy as metastatic invasive thymoma. Severe hypoalbuminemia, heavy proteinuria, hyponatremia, and hypercholesterolemia were features of the nephrotic syndrome. A kidney needle biopsy suggested focal segmental glomerulosclerosis.
INTERVENTIONS AND OUTCOMES: All of the symptoms of nephrotic syndrome were resolved simultaneously during the first 2 cycles of chemotherapy. The patient was on regular follow-up with no specific treatment for nephrotic syndrome and underwent successful resection of the left pleura and anterior thymoma. The patient has shown no evidence of recurrence for 2 years.
LESSONS: We conclude that chemotherapy for invasive thymoma is an effective treatment for nephrotic syndrome accompanying the thymoma.
Bilateral single-port thoracoscopic extended thymectomy for management of thymoma and myasthenia gravis: case report.
J Cardiothorac Surg. 2016; 11(1):153 [PubMed] Free Access to Full Article Related Publications
CASE PRESENTATION: A 74 years old woman with myasthenia gravis was referred to our attention for management of a 3.5 cm, well capsulate, thymoma. All laboratory and cardio-pulmonary tests were within normal; thus, she was scheduled for thymoma resection through an uniportal bilateral thoracoscopic approach. Under general anaesthesia and selective intubation, the patient was placed in a 60° right lateral decubitus. A 3 cm skin incision was performed in the fourth right intercostal space and, through that a 30° video-camera and working instruments were inserted without rib spreading. After complete dissection of the thymus and mediastinal fat, the contralateral pleura was opened, and, through that the specimen was pushed into the left pleural cavity. Then, the patient was placed in the left lateral decubitus. Similarly to the right side procedure, a 3-cm incision was performed in the fourth left intercostal space to complete thymic dissection and retrieve the specimen. No intraoperative and post-operative complications were found. The patient was discharged four days later. Pathological examination revealed a type A thymoma (Masaoka stage I). No recurrence was found at 18 months of follow-up CONCLUSIONS: Bilateral single-port thoracoscopy is an available procedure for management of thymoma associated with myasthenia gravis. The less post-operative pain, the reduction of hospital stay and the better esthetic results are all potential advantages of this approach over traditional technique. Obviously, our impression should be validated by larger studies in terms of long-term oncological outcomes.
miR-20b Inhibits T Cell Proliferation and Activation via NFAT Signaling Pathway in Thymoma-Associated Myasthenia Gravis.
Biomed Res Int. 2016; 2016:9595718 [PubMed] Free Access to Full Article Related Publications
Invasive thymoma presenting as classic superior vena cava syndrome: a case of venous spread metastasis.
BMJ Case Rep. 2016; 2016 [PubMed] Related Publications
Autoimmune hepatitis: an uncommon presentation of thymoma.
Tumori. 2016; 102(Suppl. 2) [PubMed] Related Publications
METHODS: We report the case of a 31-year-old man affected by autoimmune hepatitis associated with myasthenia gravis and thymoma, successfully treated with extended thymectomy.
RESULTS: The patient is free from neoplastic and hepatic disease 4 years after surgery. Eighteen months after thymectomy, an exacerbation of hepatitis was successfully treated with steroids.
CONCLUSIONS: To the authors' knowledge, only 7 cases of myasthenia gravis associated with thymoma and autoimmune hepatitis have been reported in the English-language literature. The exact role of thymoma in immune-mediated hepatitis is unclear. It seems likely that thymoma-associated T-cell abnormalities, due to the presence of thymoma, may have a role in the development of this rare clinical triad of autoimmune hepatitis, thymoma and myasthenia gravis.
Postoperative Recurrence of Invasive Thymoma with Cold Agglutinin Disease and Autoimmune Hemolytic Anemia.
Intern Med. 2016; 55(18):2685-9 [PubMed] Related Publications
A case of late-onset, thymoma-associated myasthenia gravis with ryanodine receptor and titin antibodies and concomitant granulomatous myositis.
BMC Neurol. 2016; 16(1):172 [PubMed] Free Access to Full Article Related Publications
CASE PRESENTATION: This report presents a case-vignette of a 72-year-old woman with progressive proximal muscle weakness and myalgias, diagnosed with thymoma-associated myasthenia and bioptically verified granulomatous myositis, with positive autoantibody status for ryanodine receptor and titin antibodies.
CONCLUSIONS: The diagnosis of concurrent myositis and myasthenia gravis, especially in the presence of ryanodine receptor and titin antibodies, should lead neurologists to adopt different treatment strategies compared to those applied in myasthenia or myositis alone. Moreover, further evidence is warranted that titin and, particularly, ryanodine receptor antibodies may co-occur or be pathophysiologically involved in myasthenia-myositis cases.
Occasional detection of thymic epithelial tumor 4 years after diagnosis of adult onset Still disease: A challenging case report and immuno-oncological considerations coming from pertinent literature review.
Medicine (Baltimore). 2016; 95(36):e4357 [PubMed] Free Access to Full Article Related Publications
METHODS: Herein, we report, to the best of our knowledge, the first description of an association between thymoma and adult onset Still disease (AOSD) in a 47-year-old man. The first one was occasionally detected 4 years later the diagnosis of AOSD, and surgically removed via right lateral thoracotomy. Histology confirmed an encapsulated thymic tumor (type AB sec. WHO-classification).
RESULTS: The AOSD was particularly resistant to the therapy, requiring a combination of immunosuppressant followed by anti-IL1R, that was the only steroids-sparing treatment capable to induce and maintain the remission. The differential diagnosis was particularly challenging because of the severe myasthenic-like symptoms that, with normal laboratory tests, were initially misinterpreted as fibromyalgia. The pathogenic link of this association could be a thymus escape of autoreactive T lymphocytes causing autoimmunity.
CONCLUSION: Clinicians should be always include the possibility of a thymoma in the differential diagnosis of an unusual new onset of weakness and normal laboratories data, in particular once autoimmune disease is present in the medical history.
The clinical features, diagnosis and management of recurrent thymoma.
J Cardiothorac Surg. 2016; 11(1):140 [PubMed] Free Access to Full Article Related Publications
Bone metastasis of malignant thymomas associated with peripheral T-cell lymphocytosis.
BMC Surg. 2016; 16(1):58 [PubMed] Free Access to Full Article Related Publications
CASE PRESENTATION: A 47-year-old female patient was admitted to our hospital for ptosis. A diagnosis of malignant thymoma was made based on the thymectomy and pathological result. Peripheral T-cell lymphocytosis and bone metastasis were found later. T-cell lymphocytosis was relived after surgical and radiation therapy to the metastasis.
CONCLUSION: Peripheral T-cell lymphocytosis is a rare paraneoplastic phenomenon associated with thymomas. This report is the first to describe an invasive thymoma with late bone metastasis accompanied with T-cell lymphocytosis. We should be aware of peripheral T-cell lymphocytosis in thymomas and it may contribute to a better understanding of the complex physiology and pathogenesis of thymoma.
Clinicopathologic and prognostic factors of thymoma in Tunisia.
Tunis Med. 2016; 94(2):118-22 [PubMed] Related Publications
AIM: the aim of this study is to present clinicopathological features of thymomas in Tunisia and analyse the prognostic factors.
METHODS: From 1993 to 2004, clinical data of 40 cases of thymomas were compiled retrospectively. Microscopic slides were reviewed and reclassified according to the WHO classification of thymic tumors 2004. Clinical staging adopted was Masaoka system. Analysis of survival was determined by Kaplan-Meier method and log-rank test was used to compare survival curves. These statistical analyses were performed by SPSS.
RESULTS: they were 23 women and 17 males of ages ranging from 14 to 76 years (mean age 51 years). The distribution of histological WHO types was: 1 type A, 7 type AB, 6 type B1, 17 type B2, 6 type B3, 2 cases of micronodular thymoma with lymphoid stroma and 1 case of metaplastic thymoma. According to Masaoka stage, 10 patients were in stage I, 11 stage II, 9 stage IIIa, 4 stage IIIb, 5 stage Iva and 1 stage IVb. The average overall survival was 56 months. Univariate analyses showed that Masaoka stage, completeness surgical resection and age were prognostic factors whereas in multivariate analysis, age was the only prognostic factor. Neither myasthenia gravis nor histological WHO subtypes had effect in survival.
CONCLUSION: masaoka stage, completeness surgical resection and age are the prognostic factors predicting survival in our series.
Microthymoma in elderly-onset myasthenia gravis detected preoperatively.
Asian Cardiovasc Thorac Ann. 2016; 24(8):818-821 [PubMed] Related Publications
Induction Therapy for Thymoma.
Thorac Surg Clin. 2016; 26(3):325-32 [PubMed] Related Publications
Video-assisted thoracoscopic surgery thymectomy versus open thymectomy in patients with myasthenia gravis: a meta-analysis.
Acta Chir Belg. 2016; 116(5):282-288 [PubMed] Related Publications
METHODS: We searched PubMed, Embase, ScienceDirect, Web of Science, and CNKI for related articles using combinations of the search terms video-assisted thoracoscopic thymectomy, transsternal thymectomy, and MG. The inter-study heterogeneity was assessed by χ(2)-based Q statistics, and the extent of inconsistency was generated by I(2) statistics.
RESULTS: A total of 12 studies with 1173 patients were included, and there was no difference in the operation time (p = 0.08) and ICU time (p = 0.14) between the two groups, but VATS thymectomy was associated with less intra-operation blood loss and hospital time (p < 0.00001). VATS was also associated with lower rates of total complication (OR =0.59; 95% CI, 0.37-0.94; p = 0.03) and myasthenic crisis (OR = 0.51; 95% CI, 0.28-0.92; p = 0.03), but the rates of pneumonia (OR = 0.59; 95% CI, 0.29-1.32; p = 0.21) and complete remission rate (CSR) (OR = 0.64; 95% CI, 0.38-1.09; p = 0.10) had no obvious differences between the VATS and OT groups.
CONCLUSION: Patients with MG undergoing VATS thymectomy achieved better surgical outcomes and fewer complications than those who received OT.
Tumor-related gene expression levels in thymic carcinoma and Type B3 thymoma.
J Cardiothorac Surg. 2016; 11(1):85 [PubMed] Free Access to Full Article Related Publications
METHODS: TC and B3 specimens resected from 1999 through 2012 were investigated. Tumor segments were collected from the specimens by micro-dissection to extract mRNA, then RT-PCR was performed according to Dannenberg's tumor profile method for semi-quantitation of tumor-related gene mRNA. To compare with other types of cancer, data from lung cancer (LC) cases in our database were also examined.
RESULTS: The gene expression levels of thymidylate synthase were significantly higher in TC and B3 as compared to LC specimens (p < 0.02), while no difference were observed between TC and B3 tumors. The ratio of folypolyglutamyl synthase (FPGS) to gamma-glutamyl hydrolase (GGH) mRNA was significantly lower in TC than in B3 (p < 0.05), with lower FPGS/GGH in those tumors related to overall survival. Also, the gene expression of vascular endothelial growth factor (VEGF) was significantly higher in TC as compared to B3 (p = 0.04), with higher VEGF gene expression in TC and B3 specimens related to overall survival of affected patients. Epidermal growth factor receptor (EGFR) expression was significantly higher in B3 as compared to both TC and LC specimens (p < 0.01). However, there were no EGFR gene mutations detected in any of the specimens.
CONCLUSIONS: These results indicate that elevated expressions of the tumor-related genes FPGS/GGH and VEGF are correlated with malignancy of TC and B3 tumors. Additional examinations will be necessary to investigate their chemosensitivity.
Thymomas With Extensive Clear Cell Component: A Clinicopathologic and Immunohistochemical Study of Nine Cases.
Am J Clin Pathol. 2016; 146(1):132-6 [PubMed] Related Publications
METHODS: The patients were six men and three women aged between 45 and 62 years (mean, 52 years). Presenting symptoms included shortness of breath, chest pain, and cough. Diagnostic imaging revealed anterior mediastinal masses in all patients, and all underwent thymectomy.
RESULTS: Grossly, the tumors varied from 3 to 9 cm in the greatest dimension. Four cases were invasive and five encapsulated. Histologically, the tumors were characterized by an epithelial cell component with extensive clear cell change, admixed with lymphocytes in varying proportions. Immunohistochemically, the tumors demonstrated the typical immunophenotype of thymomas characterized by positive staining of the epithelial cells for pancytokeratin and cytokeratin 5/6, absent expression of CD5 and c-kit, and reactivity of the lymphocytes for terminal deoxynucleotidyl transferase. Clinical follow-up available for six patients showed that all were alive and well 12 to 24 months after surgical resection.
CONCLUSIONS: The cases herein presented highlight an unusual feature in thymoma that has more commonly been ascribed to thymic carcinoma. They also emphasize the importance of correct diagnosis to determine the appropriate treatment strategy and to accurately predict prognosis.
Good's syndrome: Is thymectomy the solution? Case report and literature review.
Asian Cardiovasc Thorac Ann. 2016; 24(7):712-4 [PubMed] Related Publications
The spectrum of ectopic thymomas.
Virchows Arch. 2016; 469(3):245-54 [PubMed] Related Publications
Bilateral thoracoscopic extended thymectomy versus sternotomy.
Asian Cardiovasc Thorac Ann. 2016; 24(6):555-61 [PubMed] Related Publications
METHODS: From 2011 to 2014, 43 patients undergoing thymectomy were divided into 2 groups: 23 underwent video-assisted thoracoscopic extended thymectomy, and 20 had thymectomy via sternotomy. The primary outcomes were postoperative pain score (visual analog scale) at 6, 12, 24, 48, and 72 h, and 1-month postoperatively, and morphine consumption in the first 48 h. Secondary outcomes were surgical and clinical results.
RESULTS: There were no significant differences between the 2 groups in terms of demographics and preoperative clinical data. Compared to the sternotomy group, the video-assisted thoracoscopic thymectomy group had lower pain scores and morphine consumption at all time points, significantly less operative blood loss and chest drainage volume, and shorter hospital stay. The rates of improvement in myasthenia gravis were 85% and 86% in the video-assisted thoracoscopic thymectomy and sternotomy groups, respectively. No recurrence of thymoma was found in either group (median follow-up 27 months).
CONCLUSIONS: Our results seem to confirm that in selected cases, video-assisted thoracoscopic thymectomy allows complete resection of thymus and perithymic tissue, similar to sternotomy but with the known advantages of minimally invasive surgery including less pain and a good cosmetic result.
Benign anlage tumour: a very unusual neck mass.
BMJ Case Rep. 2016; 2016 [PubMed] Related Publications
A Case of Good's Syndrome Presenting with Pulmonary Tuberculosis.
Indian J Chest Dis Allied Sci. 2015 Oct-Dec; 57(4):247-50 [PubMed] Related Publications
Importance of thymectomy and prognostic factors in the complex treatment of myasthenia gravis.
Bratisl Lek Listy. 2016; 117(4):195-200 [PubMed] Related Publications
METHODS: 345 patients with MG who underwent an extended thymectomy from April 1990 to December 2010. Patients were separated into the 4 groups: group with a complete stable remission, pharmacological remission group, group of patients with significant improvement and the group with mild improvement of symptoms.
RESULTS: In our study, we observed 345 patients with MG and thymectomy. 137 patients (39.71 %) attained the complete stable remission (CSR) and 92 patients (26.67 %) the pharmacological remission (PR). The significant improvement of MG symptomatology was achieved in 95 patients (27.54 %). The rest of 21 patients from total 345 (6.08 %) have reached only a mild improvement. Patients after thymectomy with CSR were in the clinical stage I and IIA, in accordance to the modified Osserman classification and most benefited from the thymectomy.
CONCLUSIONS: The extended thymectomy combined with immunotherapy is the preferred treatment with an expected satisfactory long-term remission rate. Despite the recent improvements in MG medical therapy, thymectomy plays an important role in the otherwise complex treatment of the disease. Because of early diagnosis with thymectomy performed without a delay, patients can achieve significantly more often favorable outcomes and even stable remissions (Tab. 4, Fig. 7, Ref. 17).
Ectopic thymic carcinoma presenting as an intrathoracic mass.
Asian Cardiovasc Thorac Ann. 2016; 24(5):480-3 [PubMed] Related Publications
Survival Impact of Adjuvant Radiation Therapy in Masaoka Stage II to IV Thymomas: A Systematic Review and Meta-analysis.
Int J Radiat Oncol Biol Phys. 2016; 94(5):1129-36 [PubMed] Related Publications
METHODS AND MATERIALS: A database search was conducted with EMBASE, PubMed, Web of Science, Cochrane Library, and Ovid from inception to August 2015. Thymic carcinomas were excluded, and studies comparing overall survival (OS) with and without PORT in thymomas were included. The hazard ratios (HRs) of OS were extracted, and a random-effects model was used in the pooled analysis.
RESULTS: Seven retrospective series with a total of 1724 patients were included and analyzed. Almost all of the patients underwent macroscopically complete resection, and thymoma histology was confirmed by the World Health Organization criteria. In the overall analysis of stage II to IV thymomas, OS was not altered with the receipt of PORT (HR 0.79, 95% confidence interval [CI] 0.58-1.08). Although PORT was not associated with survival difference in Masaoka stage II disease (HR 1.45, 95% CI 0.83-2.55), improved OS was observed with the addition of PORT in the discrete pooled analysis of stage III to IV (HR 0.63, 95% CI 0.40-0.99). Significant heterogeneity and publication bias were not found in the analyses.
CONCLUSIONS: From the present meta-analysis of sole primary thymomas, we suggest the potential OS benefit of PORT in locally advanced tumors with macroscopically complete resection, but not in stage II disease. Further investigations with sufficient survival data are needed to establish detailed treatment indications.
About thymic carcinomas: challenges in diagnosis and management.
Asian Cardiovasc Thorac Ann. 2016; 24(4):350-4 [PubMed] Related Publications
METHODS: We studied 12 (92%) men and 1 (7.7%) woman with a mean age of 37 years (range 15-60 years). All patients were symptomatic, with chest pain representing the most frequent symptom. Radiology revealed anterior mediastinal masses in all cases, with either infiltration of the adjacent organs or pulmonary parenchymal metastases.
RESULTS: The diagnosis was made on surgical biopsies in 12 cases and a lymph node biopsy in one. Microscopic examination revealed squamous carcinoma in 3 cases, synovial sarcoma in 1, mucinous adenocarcinoma in 1, undifferentiated carcinoma in 2, clear cell carcinoma in 1, lymphoepithelioma-like carcinoma in 2, atypical carcinoid tumor in 2, and sarcomatoid carcinoma in 1. Total surgical resection was possible in one patient after neoadjuvant chemotherapy and radiotherapy. Follow-up was possible in only 6 patients, and the mean survival reached 13 months.
CONCLUSION: In spite of the lack of follow-up information, this study demonstrates the poor outcome associated with these tumors and the need for standardized treatment.
Preoperative Anxiety in Patients With Myasthenia Gravis and Risk for Myasthenic Crisis After Extended Transsternal Thymectomy: A CONSORT Study.
Medicine (Baltimore). 2016; 95(10):e2828 [PubMed] Free Access to Full Article Related Publications
A clinicopathological study of mediastinal masses operated in a tertiary care hospital in Eastern India in 3 years with special reference to thymoma.
Indian J Pathol Microbiol. 2016 Jan-Mar; 59(1):20-4 [PubMed] Related Publications
MATERIALS AND METHODS: This is a retrospective study of cases presenting with mediastinal masses attending the Cardiothoracic Surgery Department of Medical College, Kolkata between May 2011 and April 2014. Detailed history, physical, and radiological findings were noted. Fine needle aspiration cytology (FNAC) was performed when feasible. Following surgery, histopathological, and immunohistochemical (IHC) examinations of the specimens were undertaken.
RESULTS: Of the 22 cases included in our study, ten were anterior, seven middle, and five posterior mediastinal masses. Fifteen cases were male and seven were female. Thymic pathology was detected in seven cases, lymphoma in five, extragonadal germ cell tumor (GCT) in three, schwannoma and pericardial cyst in two cases each and neurofibroma, ganglioneuroma, and retrosternal thyroid in one case each. The age group of the patients for each diagnostic category was found to be of significance. FNAC was done in 15 cases. IHC was required for classification of lymphoma cases (CD45, CD15, CD30, CD20, CD3, Tdt, CD34, and Ki-67).
CONCLUSION: This study reflects the incidence of different mediastinal masses in West Bengal with their clinicopathologic correlation.
Good's Syndrome Accompanied by Agranulocytosis Following a Rapid Clinical Course.
Intern Med. 2016; 55(5):537-40 [PubMed] Related Publications