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Medicine Dec 2023Patients who have myasthenia gravis or dermatomyositis show clinical signs of muscular weakening. Ocular muscle involvement is uncommon, and symmetrical proximal limb...
RATIONALE
Patients who have myasthenia gravis or dermatomyositis show clinical signs of muscular weakening. Ocular muscle involvement is uncommon, and symmetrical proximal limb weakness is the typical presentation of dermatomyositis. However, the earliest and most noticeable sign in those with myasthenia gravis is extraocular muscular paralysis. Dermatomyositis is frequently complicated by malignancy, and the common malignancies associated with dermatomyositis vary by region and ethnicity, while thymoma is relatively rare. About 10% to 15% of people with myasthenia gravis have thymoma, which is involved in the etiology of the disease.
PATIENT CONCERNS
A 68-year-old female presented with ocular muscle weakness for 10 days that manifested as bilateral blepharoptosis with the phenomenon of "light in the morning and heavy in the evening." Imaging examination showed anterior mediastinal thymic tumor with metastasis.
DIAGNOSES
After a thorough physical examination, we discovered bilateral upper limbs with grade IV muscle strength and the typical rash of dermatomyositis. In combination with elevated serum kinase levels and electromyography suggesting myogenic damage, the patient was finally diagnosed as dermatomyositis with multiple metastases of thymoma.
INTERVENTIONS
The patient received oral hydroxychloroquine sulfate, topical corticosteroids, and tacrolimus ointment, but these did not work very well. Subsequently, the patient underwent surgery combined with radiotherapy for the thymoma.
OUTCOMES
Muscle weakness in the patient improved after effective treatment of tumor, and the rash mostly disappeared.
CONCLUSION
Ocular muscle weakness and thymoma are more common in myasthenia gravis, but we cannot ignore the possibility of dermatomyositis. To further establish the diagnosis, a thorough physical examination and laboratory findings are required. Further tumor screening should be performed for patients with dermatomyositis. Early detection and management of possible tumors are essential to the treatment of dermatomyositis linked to malignancies.
Topics: Female; Humans; Aged; Thymoma; Dermatomyositis; Myasthenia Gravis; Thymus Neoplasms; Muscle Weakness; Exanthema
PubMed: 38115296
DOI: 10.1097/MD.0000000000036234 -
Indian Journal of Pathology &... 2022Myasthenia gravis (MG) is a prototypic T-cell-dependent antibody-mediated autoimmune disease that leads to ocular or generalized muscular weakness. The disease is most...
BACKGROUND
Myasthenia gravis (MG) is a prototypic T-cell-dependent antibody-mediated autoimmune disease that leads to ocular or generalized muscular weakness. The disease is most commonly caused by antibodies to the acetylcholine receptors, often with underlying thymic pathology.
AIMS
This study is aimed at analyzing the pathological spectrum of the excised thymuses in patients with myasthenia.
MATERIALS AND METHODS
This was a retrospective 10-year study of 68 thymectomy specimens performed as a part of the treatment of patients with MG.
STATISTICAL ANALYSIS
Nil.
RESULTS
There were 47 males and 21 females (male to female ratio of 2.2:1) with a mean age of 41 years. Only three patients presented with ocular myasthenia. The thymus was normal in 9 patients (13.2%) and atrophic in 17 patients (25%). Follicular hyperplasia and thymomas were seen in 6 and 36 patients, respectively.
CONCLUSION
The thymectomies performed in patients of MG had a fairly variable spectrum on histology; the thymic tumors were predominantly of the cortical phenotype.
Topics: Adolescent; Adult; Aged; Female; Histological Techniques; Humans; Male; Middle Aged; Myasthenia Gravis; Retrospective Studies; Thymectomy; Thymoma; Young Adult
PubMed: 35074977
DOI: 10.4103/IJPM.IJPM_935_20 -
Zhongguo Fei Ai Za Zhi = Chinese... Jan 2021Thymomas are the most common primary malignant tumors of anterior mediastinal. However, there are no specific laboratory indicator for the diagnosis the diagnosis of...
BACKGROUND
Thymomas are the most common primary malignant tumors of anterior mediastinal. However, there are no specific laboratory indicator for the diagnosis the diagnosis of thymoma. The aim of this study was to screen out a tumor marker for diagnosis of thymoma by mRNA microarray analysis and confirmed it.
METHODS
By mRNA microarray analysis of 31 thymomas and peritumoral thymic tissues, we found that the transcription level of neuronal pentraxin 1 (NPTX1) gene was up-regulated more than 4 times in thymomas. To further verify the above results, we detected the transcription and expression level of NPTX1 in 60 thymoma and 30 thymic cyst patients by quantitative Real-Time polymerase chain reaction (PCR), immunohistochemistry and enzyme-linked immunosorbent assay (ELISA). Furthermore, the diagnostic value of NPTX1 in thymoma by receiver operating characteristic curve (ROC) was analyzed.
RESULTS
The transcription level of NPTX1 mRNA in thymoma tissues was significantly higher than that in the thymic tissues of control group [(2.88±1.02) vs (1.35±0.47), P<0.001); The expression level of NPTX1 in thymoma tissues was significantly higher than that in the thymic tissues of control group (2 vs 1, P<0.001); The preoperative serum level of NPTX1 protein in thymoma patients were significantly higher than that in the thymic cyst patients of control group [(1,018.29±209.38) pg/mL vs (759.95±66.02) pg/mL, P<0.001]; At the threshold of 842.22 pg/mL, sensitivity and specificity of NPTX1 as a serologic marker were 85.00% and 93.33%, respectively for thymoma. ROC showed that the area the under curve (AUC) of NPTX1 was 0.902.
CONCLUSIONS
NPTX1 was highly expressed in thymoma patients, and had diagnostic value for thymoma.
Topics: Adult; Aged; Biomarkers, Tumor; C-Reactive Protein; Diagnosis, Differential; Female; Humans; Lung Neoplasms; Male; Middle Aged; Nerve Tissue Proteins; ROC Curve; Thymoma; Young Adult
PubMed: 33478183
DOI: 10.3779/j.issn.1009-3419.2021.102.03 -
Bulletin Du Cancer Apr 1999The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of the French Cancer Centres (FNCLCC), the 20... (Review)
Review
CONTEXT
The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of the French Cancer Centres (FNCLCC), the 20 French Cancer Centres and specialists from French Public Universities, General Hospitals and Private Clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and outcome for cancer patients. The methodology is based on literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery.
OBJECTIVES
To develop clinical practice guidelines according to the definitions of Standards, Options and Recommendations for the clinical care of malignant thymoma in adult.
METHODS
Data have been identified by literature search using Medline (december 1998) and the expert groups personal reference lists. Once the guidelines were defined, the document was submitted for review to national and international independent reviewers, and to the medical committees of the 20 French Cancer Centres.
RESULTS
The main recommendations for malignant thymoma management are that: 1) the clinical diagnosis is based on appropriate clinical and radiological findings; 2) the final diagnosis is pathological and made from a biopsy, except in cases of well-encapsulated tumors which are completely resected. The biopsy, via anterior mediastinostomy, should be performed by the surgeon who will subsequently perform the definitive surgery; 3) surgical resection must be complete including thymus and perithymic fat and performed by an experienced surgeon; 4) the therapeutic strategy for malignant thymoma is based on the three current staging systems and involves surgery with radiotherapy given if the capsule is invaded or penetrated. Radiotherapy should be given in experienced centres. Inclusion of patients in prospective clinical trials is recommended in order to determine the usefulness of neoadjuvant chemotherapy and multimodality approaches; 5) treatment of metastatic malignant thymoma is based on chemotherapy. Secondary surgery may be performed with the aim of achieving complete resection. Inclusion in clinical trials is recommended; 6) at the present time, there are no clear data on which to base guidelines for timing and duration of follow-up studies in this condition. Because of late recurrence, follow-up should be long.
Topics: Adult; Humans; Neoplasm Recurrence, Local; Neoplasm Staging; Paraneoplastic Syndromes; Thymoma; Thymus Neoplasms
PubMed: 10341342
DOI: No ID Found -
Journal of Thoracic Oncology : Official... Oct 2013
Topics: Humans; Prognosis; Prospective Studies; Thymoma; Thymus Neoplasms
PubMed: 24457232
DOI: 10.1097/JTO.0b013e3182a8fd9e -
Journal of Veterinary Diagnostic... Nov 2020Persistent small-cell lymphocytosis in dogs with a concurrent mediastinal mass has been associated with both thymoma and small-cell lymphoma. In thymomas, neoplastic...
Persistent small-cell lymphocytosis in dogs with a concurrent mediastinal mass has been associated with both thymoma and small-cell lymphoma. In thymomas, neoplastic thymic epithelial cells induce overproduction and release of polyclonal lymphocytes, whereas thymic lymphoma results in thymic effacement by a clonal expansion of neoplastic lymphocytes and subsequent leukemic phase of lymphoma. Flow cytometry has been used to differentiate these 2 entities by immunophenotyping mediastinal mass aspirates. It has been reported that cases with mediastinal masses in which ≥ 10% of the associated small-cell lymphocytes were double positive for CD4 and CD8 were thymomas, whereas masses associated with < 10% were suggestive of lymphoma. We report a unique case of thymoma-associated lymphocytosis lacking the classic CD4+CD8+ immunophenotype. Our findings suggest that there may be more diversity in the thymoma-associated lymphocyte immunophenotype than has been identified previously; immunophenotyping alone might not be sufficient to differentiate thymic small-cell lymphoma from thymoma-associated lymphocytosis. In dogs with mediastinal masses and peripheral lymphocytosis, employing a variety of testing modalities to avoid misdiagnosis is prudent. These modalities include cytologic and/or histologic evaluation, immunophenotyping, and clonality assessment.
Topics: Animals; Dog Diseases; Dogs; Female; Flow Cytometry; Immunophenotyping; Lymphocytosis; Lymphoma; Male; T-Lymphocytes; Thymoma; Thymus Neoplasms
PubMed: 32814519
DOI: 10.1177/1040638720948628 -
BMC Pulmonary Medicine Jun 2024The diagnostic complexities that arise in radiographic distinction between ectopic pleural thymoma and other thoracic neoplasms are substantial, with instances of...
BACKGROUND
The diagnostic complexities that arise in radiographic distinction between ectopic pleural thymoma and other thoracic neoplasms are substantial, with instances of co-occurring T-cell lymphocytosis and osseous metastasis being exceedingly rare.
CASE PRESENTATION
A 51-year-old woman was admitted to our hospital with dyspnea and chest pain. Upon imaging examination, she was found to have diffuse and nodular pleural thickening on the left side, collapse of the left lung and a compression in the second thoracic vertebrae. All lesions showed significant F-FDG uptake on F-FDG PET/CT examination. Furthermore, she exhibited T-cell lymphocytosis in her peripheral blood, lymph nodes, and bone marrow. After ruling out malignant pleural mesothelioma (MPM), lung cancer with pleural metastasis, and T-cell lymphoma, the definitive diagnosis asserted was ectopic pleural thymoma with T-cell lymphocytosis and bone metastasis.
CONCLUSION
Physicians need to expand their knowledge of the imaging features of ectopic pleural thymoma. Cases with T-cell lymphocytosis may exhibit increased aggressiveness and prone to bone metastasis.
Topics: Humans; Female; Middle Aged; Thymoma; Lymphocytosis; Pleural Neoplasms; Bone Neoplasms; Positron Emission Tomography Computed Tomography; Thymus Neoplasms; T-Lymphocytes; Fluorodeoxyglucose F18; Diagnosis, Differential; Pleura
PubMed: 38877486
DOI: 10.1186/s12890-024-03090-x -
Journal of Radiology Case Reports Oct 2020Thymoma and paraganglioma are known causes of mediastinal masses, the latter being extremely rare. Thymomas arise from remnant thymic tissue in the anterior mediastinum;...
Thymoma and paraganglioma are known causes of mediastinal masses, the latter being extremely rare. Thymomas arise from remnant thymic tissue in the anterior mediastinum; whereas, thoracic paragangliomas arise from para-aortic or para-vertebral sympathetic chain ganglion (derivatives of embryonic neural crest) in the middle or posterior mediastinum. We report a case of a middle-aged woman with two mediastinal masses, originally believed to be a single tumor or primary malignancy with adjacent metastasis on Computed Tomography (CT) that were further delineated with Magnetic Resonance Imaging (MRI) and [68Ga]-DOTA-(Tyr3)-octreotate (DOTA-TATE) Positron Emission Tomography-Computed Tomography (PET-CT) and surgical pathology as two distinct entities: left epicardial paraganglioma and anterior mediastinal thymoma. A comprehensive discussion of both entities is included.
Topics: Female; Heart Neoplasms; Humans; Magnetic Resonance Imaging; Mediastinal Neoplasms; Middle Aged; Paraganglioma; Positron Emission Tomography Computed Tomography; Thymoma; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 33708339
DOI: 10.3941/jrcr.v14i10.4101 -
Endocrine Journal Nov 2022Intrathyroidal thymic carcinoma (ITTC) is a rare malignancy of the thyroid. It is thought to originate from ectopic thymic tissue or embryonic thymic rest, in, or...
Intrathyroidal thymic carcinoma (ITTC) is a rare malignancy of the thyroid. It is thought to originate from ectopic thymic tissue or embryonic thymic rest, in, or adjacent to, the thyroid. We analyzed the backgrounds, clinicopathological features, and prognosis of 20 patients with ITTC, treated at our hospital. Thirteen of the 15 patients (86%) who underwent ultrasonography were diagnosed as malignant, based on imaging findings. 16 of the 17 patients (93%) who underwent cytology, were diagnosed or suspected to be malignant. Locally curative surgery (thyroidectomy and lymph node dissection) was performed for 19 patients. Large tumor size (>4 cm) was positively related to pathological node metastasis (p = 0.0389). Fourteen patients, including nine Ex-positive patients, underwent adjuvant external beam radiotherapy (EBRT) of the neck after surgery. Two patients showed recurrence of thyroid bed after and neither of them underwent adjuvant EBRT after surgery. Two patients who underwent EBRT showed recurrences of the lateral nodes (level V and level II), but they were easily dissected by re-operation. Ten- and 20-year local recurrence-free survival rates were 84.9% and 60.6%, respectively. To date, four patients showed distant recurrence, and 10- and 20-year distant recurrence-free survival rates were 75.0% and 75.0%, respectively. Our findings indicate that 1) the prognosis of ITTC is generally favorable, and 2) large tumor size is significantly related to lymph node metastasis. Two patients showing recurrence of the central region did not undergo EBRT; thus, further comparative studies are desirable to elucidate whether EBRT can prevent significant local recurrence.
Topics: Humans; Carcinoma, Papillary; Thyroid Neoplasms; Thymoma; Thyroidectomy; Prognosis; Thymus Neoplasms; Neoplasm Recurrence, Local; Neck Dissection; Retrospective Studies
PubMed: 35768280
DOI: 10.1507/endocrj.EJ22-0141 -
PloS One 2013Thymoma represents one of the rarest of all malignancies. Stage and completeness of resection have been used to ascertain postoperative therapeutic strategies albeit...
PURPOSE
Thymoma represents one of the rarest of all malignancies. Stage and completeness of resection have been used to ascertain postoperative therapeutic strategies albeit with limited prognostic accuracy. A molecular classifier would be useful to improve the assessment of metastatic behaviour and optimize patient management.
METHODS
qRT-PCR assay for 23 genes (19 test and four reference genes) was performed on multi-institutional archival primary thymomas (n = 36). Gene expression levels were used to compute a signature, classifying tumors into classes 1 and 2, corresponding to low or high likelihood for metastases. The signature was validated in an independent multi-institutional cohort of patients (n = 75).
RESULTS
A nine-gene signature that can predict metastatic behavior of thymomas was developed and validated. Using radial basis machine modeling in the training set, 5-year and 10-year metastasis-free survival rates were 77% and 26% for predicted low (class 1) and high (class 2) risk of metastasis (P = 0.0047, log-rank), respectively. For the validation set, 5-year metastasis-free survival rates were 97% and 30% for predicted low- and high-risk patients (P = 0.0004, log-rank), respectively. The 5-year metastasis-free survival rates for the validation set were 49% and 41% for Masaoka stages I/II and III/IV (P = 0.0537, log-rank), respectively. In univariate and multivariate Cox models evaluating common prognostic factors for thymoma metastasis, the nine-gene signature was the only independent indicator of metastases (P = 0.036).
CONCLUSION
A nine-gene signature was established and validated which predicts the likelihood of metastasis more accurately than traditional staging. This further underscores the biologic determinants of the clinical course of thymoma and may improve patient management.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Female; Gene Expression Profiling; Humans; Male; Middle Aged; Prognosis; Reverse Transcriptase Polymerase Chain Reaction; Survival Rate; Thymoma; Thymus Neoplasms; Young Adult
PubMed: 23894276
DOI: 10.1371/journal.pone.0066047