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1.
Histopathology ; 84(1): 183-195, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37988262

ABSTRACT

Mediastinal tumours represent a heterogeneous group of entities derived from the manifold structures located in or adjacent to the mediastinum. Due to the occurrence of some of these tumours in characteristic mediastinal compartments, an anatomical subdivision of the mediastinum in the prevascular (anterior), visceral (middle), and paravertebral (posterior) is helpful for the differential diagnosis. Benign anterior mediastinal tumours linked to an enlargement of the thymic gland mainly consist of thymic cysts and several types of thymic hyperplasia: true thymic hyperplasia, rebound hyperplasia, lymphofollicular hyperplasia, and so-called thymic hyperplasia with lymphoepithelial sialadenitis (LESA)-like features. Mature teratomas, ectopic (para)thyroid tissue, and benign thymic tumours such as thymolipoma or thymofibrolipoma represent further typical tumours of the anterior mediastinum. Pericardial, bronchogenic, or oesophageal duplication cysts predominate in the middle mediastinum, whereas neurogenic tumours and myelolipomas are characteristic findings in the posterior compartment. Vascular tumours, lipomas, adenomatoid tumours, Castleman disease, or mediastinitis are further examples of less frequent tumours or tumorous lesions affecting the mediastinum. This review focuses on benign mediastinal lesions with an emphasis on benign tumours of the thymus. Besides histology, characteristic epidemiological and clinical aspects prerequisite for the correct diagnosis and patient management are discussed.


Subject(s)
Mediastinal Neoplasms , Thymus Hyperplasia , Thymus Neoplasms , Humans , Mediastinum/pathology , Mediastinal Neoplasms/diagnosis , Mediastinal Neoplasms/pathology , Thymus Hyperplasia/diagnosis , Thymus Hyperplasia/pathology , Hyperplasia/pathology , Thymus Neoplasms/pathology
2.
Rapid Commun Mass Spectrom ; 37(14): e9529, 2023 Jul 30.
Article in English | MEDLINE | ID: mdl-37125446

ABSTRACT

RATIONALE: Thymoma is a rare malignant tumor but it is the most common primary tumor of the anterior mediastinum. The current imaging methods for thymoma screening suffer from false positive rate problems, and thymoma pathogenesis remains elusive. Study of thymoma metabolic characteristics could provide clues for improving the diagnosis and understanding the pathogenesis of thymoma. METHODS: Metabolic profiling of plasma from thymoma and thymic hyperplasia patients was performed using ultrahigh-performance liquid chromatography combined with high-resolution mass spectrometry in both positive and negative ionization modes. After pre- and post-processing, the dataset was divided into three age groups and statistical analysis was performed to select differential metabolites of thymoma. For feature identification, experimental tandem mass spectra were matched to those of databases and available chemical standards, and also manually annotated with plausible chemical structures to ensure high identification confidence. RESULTS: A total of 47 differential metabolites were identified in thymoma. Significantly higher levels of histidine, sphinganine 1-phosphate, lactic acid dimer, phenylacetylglutamine, LPC (18:3) and LPC (16:1), and significantly lower levels of phenylalanine, indole-3-propionic acid (IPA), hippuric acid and mesobilirubinogen were associated with thymoma. Tryptophan level in thymoma-associated myasthenia gravis (TAMG) was significantly lower than that of the MG(-) group. IPA and hippuric acid abundances exhibited increasing trends from indolent to aggressive thymoma. CONCLUSIONS: Our study revealed aberrant aromatic amino acid metabolism and fatty acid oxidation might be associated with thymoma. The identified unique metabolic characteristics of thymoma may provide valuable information for study of the molecular mechanism of thymoma pathogenesis, and improvement of diagnosis and discovery of new therapeutic strategies for thymoma.


Subject(s)
Thymoma , Thymus Hyperplasia , Thymus Neoplasms , Humans , Thymoma/complications , Thymoma/diagnosis , Thymoma/pathology , Thymus Hyperplasia/complications , Thymus Hyperplasia/pathology , Thymus Neoplasms/complications , Thymus Neoplasms/diagnosis , Thymus Neoplasms/pathology , Metabolomics , Mass Spectrometry , Chromatography, Liquid
3.
BMC Surg ; 21(1): 38, 2021 Jan 14.
Article in English | MEDLINE | ID: mdl-33446156

ABSTRACT

BACKGROUND: Chemotherapy can cause thymic atrophy and reduce T-cell output in cancer patients. However, the thymus in young adult patients has regenerative potential after chemotherapy, manifesting as thymic hyperplasia which can be easily mistaken as residual disease or recurrence in patients suffering lymphoma. CASE PRESENTATION: This study reports a case of lymphoma in a young female adult who was initially diagnosed with an anterior mediastinal mass, and was found to have soft tissue occupying the anterior mediastinum repeatedly after chemotherapy, suggesting a lymphoma residue or disease progression. From discussions by a multi-disciplinary team (MDT), the anterior mediastinal mass of the patient was considered unknown and might be thymus tissue or tumor tissue, and it was eventually identified as thymus tissue via histopathology. CONCLUSIONS: The anterior mediastinal mass appearing after chemotherapy in patients with lymphoma can be considered as enlarged thymus, and such phenomenon is frequent in young adult patients who undergo chemotherapy or autologous hematopoietic stem cell transplantation. Additionally, detection of thymic output cells in peripheral blood might be a feasible approach to differentiate thymic hyperplasia from lymphoma.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/adverse effects , Lymphoma/drug therapy , Mediastinal Neoplasms/drug therapy , Thymus Hyperplasia/chemically induced , Adult , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Diagnostic Errors , Disease Progression , Female , Humans , Lymphoma/pathology , Mediastinal Neoplasms/pathology , Mediastinum/diagnostic imaging , Mediastinum/pathology , Neoplasm Recurrence, Local , Thymus Gland/pathology , Thymus Hyperplasia/diagnosis , Thymus Hyperplasia/diagnostic imaging , Thymus Hyperplasia/pathology
4.
BMC Endocr Disord ; 20(1): 97, 2020 Jun 30.
Article in English | MEDLINE | ID: mdl-32605565

ABSTRACT

BACKGROUND: Hyperthyroidism-induced hypercalcemia has been reported previously, but hypercalcemia accompanied by severe osteoporosis and significant thymic enlargement in patients with hyperthyroidism is quite rare. We report the coexistence of hypercalcemia, osteoporosis and thymic enlargement in a patient with Graves' disease. CASE PRESENTATION: A 22-year-old female was diagnosed as Graves' disease with obviously elevated serum calcium and reduced parathyroid hormone levels. Dual-energy x-ray absorptiometry and chest enhanced computer tomography (CT) revealed severe osteoporosis and a significant enlargement of thymus. After the successful control of hyperthyroidism with methimazole, hypercalcemia was corrected, bone mineral density was improved and thymus also shrank obviously. CONCLUSION: This is a very rare case of hypercalcemia accompanied by severe osteoporosis and significant thymic enlargement induced by Graves' disease. In clinical practice, examination of thymus and bone density should be considered when a patient with Graves' disease was present with hypercalcemia.


Subject(s)
Graves Disease/physiopathology , Hypercalcemia/pathology , Osteoporosis/pathology , Thymus Hyperplasia/pathology , Adult , Female , Humans , Hypercalcemia/complications , Osteoporosis/complications , Prognosis , Thymus Hyperplasia/complications , Young Adult
5.
BMC Nephrol ; 21(1): 528, 2020 12 04.
Article in English | MEDLINE | ID: mdl-33276741

ABSTRACT

BACKGROUND: Idiopathic multicentric Castleman disease (iMCD) is an uncommon lymphoproliferative disorder and lacks treatment consensus. Herein, we report a case of iMCD complicated with Sjögren's syndrome (SS) and secondary membranous nephropathy (SMN). CASE PRESENTATION: A 45-year-old female with dry mouth for 3 months and anasarca and proteinuria for 2 months was admitted. She also experienced chest tightness, wheezing, fever, weight loss, moderate proteinuria and hypoalbuminemia. A computed tomography (CT) scan revealed a tissue mass in the thymus area and enlarged multiple lymph nodes. Her symptoms did not improve after resection of the thymus mass. The pathological findings were "reactive hyperplasia of the mediastinal lymph nodes and thymic hyperplasia". Lymph node biopsy findings confirmed iMCD with human herpes virus-8 (HHV-8) negativity. Based on anti-nuclear antibody (ANA) 1:320, anti-SSA and anti-SSB antibody positivity, salivary flow less than 0.1 ml/min and lip biopsy with focal lymphocytic sialadenitis, SS was diagnosed. Kidney biopsy showed secondary membranous nephropathy with endocapillary cell proliferation and infiltration of plasma cells and lymphocytes in the tubulointerstitium. Serum interleukin-6 (IL-6) levels were significantly increased, and therapy with tocilizumab (anti-IL-6 receptor antibody) worked well. The combination of cyclophosphamide (CyS) with methylprednisolone (MP) maintained satisfactory remission. CONCLUSIONS: Our case of iMCD with SS and SMN is rare. There is a need for increased awareness of the disease to avoid unnecessary procedures and misdiagnoses. IL-6 was extremely high, and there was a rapid response to anti-IL-6 receptor agents. The combination of CyS with MP maintained complete remission.


Subject(s)
Castleman Disease/pathology , Glomerulonephritis, Membranous/pathology , Sjogren's Syndrome/immunology , Antibodies, Antinuclear/immunology , Antibodies, Monoclonal, Humanized/therapeutic use , Castleman Disease/complications , Castleman Disease/drug therapy , Cyclophosphamide/therapeutic use , Diagnostic Errors , Female , Glomerulonephritis, Membranous/drug therapy , Glomerulonephritis, Membranous/etiology , Glucocorticoids/therapeutic use , Humans , Immunosuppressive Agents/therapeutic use , Interleukin-6/immunology , Maintenance Chemotherapy , Methylprednisolone/therapeutic use , Middle Aged , Remission Induction , Sjogren's Syndrome/complications , Sjogren's Syndrome/drug therapy , Thymus Hyperplasia/complications , Thymus Hyperplasia/pathology , Thymus Neoplasms/diagnosis
6.
Int J Neurosci ; 129(4): 313-319, 2019 Apr.
Article in English | MEDLINE | ID: mdl-30260722

ABSTRACT

BACKGROUND: A tri-modal distribution of age-at-onset emerged among females patients with myasthenia gravis (MG) in our database. This finding may be indicative of different gender-based disease mechanisms. METHODS: We retrospectively reviewed the files of 127 MG patients for the clinical, serology and thymus pathology according to their age at disease onset: ≤40 years (early-onset, EOMG), 40-70 years (intermediate-onset, IOMG) and >70 years (late-onset, LOMG). RESULTS: EOMG was more common among females, and IOMG was more common among males. Ocular MG was more common among the male MG patients with an IOMG. Patients with EOMG had lower rates of positive anti-acetylcholine receptor (anti-AChR). IOMG females, but not IOMG males, had lower rates of positive anti-AChR. IOMG and EOMG females had high rates of thymic hyperplasia, while EOMG males had high rates of thymoma. Comorbidity with autoimmune diseases was common among females with IOMG and LOMG. CONCLUSIONS: The prevalence of IOMG was the reason for the trend reversal of MG prevalence between genders. The clinical features of patients with IOMG differed between genders in the rates of positive anti-AChR, follicular hyperplasia of the thymus and comorbidity with autoimmune diseases. This may suggest a different gender-based mechanism of immune intolerance towards AChR and other antigens.


Subject(s)
Myasthenia Gravis , Thymus Hyperplasia , Adult , Age of Onset , Aged , Autoantibodies , Female , Humans , Male , Middle Aged , Myasthenia Gravis/complications , Myasthenia Gravis/epidemiology , Myasthenia Gravis/immunology , Myasthenia Gravis/pathology , Receptors, Cholinergic/immunology , Retrospective Studies , Sex Factors , Thymus Hyperplasia/epidemiology , Thymus Hyperplasia/etiology , Thymus Hyperplasia/immunology , Thymus Hyperplasia/pathology
7.
Monaldi Arch Chest Dis ; 88(3): 913, 2018 09 04.
Article in English | MEDLINE | ID: mdl-30183162

ABSTRACT

The familial occurrence of thymic pathology, even though rare, is widely reported in the literature and mainly concerns cases of familial autoimmune myasthenia gravis. Other less frequent cases of familial occurrence of thymoma, thymic carcinoid and thymic hyperplasia have been described. It seems that the familial occurrence is poorly recorded and thus its prevalence is underestimated. We report two families whose members presented different forms of thymic pathology and discuss the necessity of screening programs in family members of patients presenting a thymic lesion.


Subject(s)
Carcinoma/diagnostic imaging , Family , Thymoma/diagnostic imaging , Thymus Hyperplasia/diagnostic imaging , Thymus Neoplasms/diagnostic imaging , Adult , Aged , Carcinoma/pathology , Carcinoma/surgery , Early Detection of Cancer , Female , Fluorodeoxyglucose F18 , Humans , Male , Mass Screening , Middle Aged , Positron-Emission Tomography , Radiopharmaceuticals , Thymoma/pathology , Thymoma/surgery , Thymus Hyperplasia/pathology , Thymus Hyperplasia/surgery , Thymus Neoplasms/pathology , Thymus Neoplasms/surgery
8.
Pathologe ; 38(4): 286-293, 2017 Jul.
Article in German | MEDLINE | ID: mdl-28429039

ABSTRACT

Reactive and neoplastic thymic pathologies are the main considerations in the case of masses in the anterior and middle part of the mediastinum, while neurogenic tumors are predominant in the posterior mediastinum (which are not dealt with here). In neonates and infants, the commonest pathologies in the anterior mediastinum comprise germ cell tumors (mainly teratomas), congenital thymic cysts and true thymic hyperplasia (TTH). In toddlers, teratomas, yolk sac tumors and cysts predominate. In children over 5 years of age, lymphomas are the commonest mass lesions whereas thymomas and thymic carcinomas are rare. In addition, inflammation-linked hyperplasia in myasthenia gravis and rebound thymic hyperplasia after chemotherapy must be considered. Although rare at all ages, sarcomas must be considered in the differential diagnosis from birth onwards and throughout adolescence. Based on the report of a rare case of recurrent TTH, the differential diagnosis of this benign but potentially life-threatening condition is discussed.


Subject(s)
Lymphatic Diseases/diagnosis , Thymus Gland/pathology , Thymus Hyperplasia/diagnosis , Thymus Neoplasms/diagnosis , Adolescent , Child , Child, Preschool , Diagnosis, Differential , Female , Humans , Immunohistochemistry , Infant , Infant, Newborn , Lymphatic Diseases/pathology , Male , Mediastinal Cyst/diagnosis , Mediastinal Cyst/pathology , Myasthenia Gravis/diagnosis , Myasthenia Gravis/pathology , Sarcoma/diagnosis , Sarcoma/pathology , Teratoma/diagnosis , Teratoma/pathology , Thymectomy , Thymoma/diagnosis , Thymoma/pathology , Thymus Hyperplasia/pathology , Thymus Neoplasms/pathology , Tomography, X-Ray Computed
9.
Pathologe ; 37(5): 400-11, 2016 Sep.
Article in German | MEDLINE | ID: mdl-27465275

ABSTRACT

The mediastinum is a complex body region of limited space but containing numerous organs of different embryonic origins. A variety of lesions that are difficult to distinguish from each other can occur here. Non-neoplastic lesions of the mediastinum represent important differential diagnostic pitfalls to mediastinal tumors, clinically, radiologically and histopathologically. It is important to bear these lesions in mind and to adequately verify or exclude them before starting further differential diagnostic considerations on mediastinal neoplasms. The most common non-neoplastic lesions in this region include cysts and lymphadenopathies. Mediastinal cysts result from abnormal events in the branching of the tracheobronchial tree, the pharyngeal pouches, the primary intestines, the pleuropericardial membranes and the brain meninges or are complications of inflammatory and hydrostatic processes. The histogenesis of the lining epithelium and the cyst wall structure are decisive for the exact classification. The histopathologically most prevalent patterns of mediastinal lymphadenopathies are those accompanied by increased histiocytes and Castleman's disease. Sclerosis is a non-specific reaction pattern of the mediastinum and can be associated with many processes; therefore, when establishing the diagnosis of sclerosing mediastinitis, several differential diagnoses have to be excluded. Simple thymic hyperplasia can be accompanied by considerable increase in organ size with severe local symptoms, while follicular thymic hyperplasia is often associated with myasthenia gravis and represents the most common findings in non-thymoma thymectomy specimens.


Subject(s)
Mediastinal Cyst/diagnosis , Mediastinal Diseases/diagnosis , Choristoma/diagnosis , Choristoma/pathology , Diagnosis, Differential , Humans , Lymphadenopathy/diagnosis , Lymphadenopathy/pathology , Mediastinal Cyst/pathology , Mediastinal Diseases/pathology , Mediastinal Neoplasms/diagnosis , Mediastinal Neoplasms/pathology , Mediastinitis/diagnosis , Mediastinitis/pathology , Mediastinum/pathology , Sclerosis/diagnosis , Sclerosis/pathology , Thymus Hyperplasia/diagnosis , Thymus Hyperplasia/pathology
10.
Radiology ; 274(1): 238-49, 2015 Jan.
Article in English | MEDLINE | ID: mdl-25105246

ABSTRACT

PURPOSE: To prospectively evaluate (a) effectiveness and limits of dual-echo chemical-shift magnetic resonance (MR) imaging for distinguishing hyperplastic thymus from anterior mediastinal tumors in adulthood by using chemical-shift ratio ( CSR chemical-shift ratio ) and signal intensity index ( SII signal intensity index ), with proposal of optimal threshold value for each, and (b) whether age affects these indexes. MATERIALS AND METHODS: Study was institutional review board approved, with informed consent obtained. Ninety-two subjects (53 men, 39 women; age range, 18-84 years) were divided into a rebound and lymphoid hyperplasia group (group A, 30 patients) and a tumor group (group B, 62 patients). MR images were assessed; interrater reliability was evaluated. Differences in CSR chemical-shift ratio and SII signal intensity index were tested with the Mann-Whitney U test and the Kruskal-Wallis test. Discrimination abilities of CSR chemical-shift ratio and SII signal intensity index were evaluated with logistic regression models, and optimal cutoff points were proposed. Quantitative parameters were correlated with age by using Pearson correlation coefficients. RESULTS: Interreader agreement was excellent (intraclass correlation coefficient: CSR chemical-shift ratio , 0.893; SII signal intensity index , 0.898). Mean CSR chemical-shift ratio and SII signal intensity index ± standard deviation were 0.545 ± 0.162 and 46.29% ± 18.41 for group A and 1.045 ± 0.094 and -0.06% ± 4.89 for group B, respectively, with significant differences for both indexes between groups (P < .0001). No overlap was found for SII signal intensity index between groups; CSR chemical-shift ratio values overlapped in a few younger adults. Distinguishing hyperplastic thymus from tumors was better with SII signal intensity index than CSR chemical-shift ratio . Respective sensitivity, specificity, and cutoff points were 100%, 100%, and 8.92% for SII signal intensity index and 100%, 96.7%, and 0.849 for CSR chemical-shift ratio . Significant correlation was found for CSR chemical-shift ratio (r = -0.761) and SII signal intensity index (r = 0.821) with age in group A (P < .001). For group B, significant correlation with age was seen for CSR chemical-shift ratio (r = 0.702, P < .001) but not SII signal intensity index (r = -0.196, P = .127). All subjects but one in group A and none in group B had signal intensity decrease at chemical-shift MR imaging. CONCLUSION: With dual-echo chemical-shift MR imaging, SII signal intensity index and CSR chemical-shift ratio have high accuracy to distinguish thymic hyperplasia from tumors, although overlapped CSR chemical-shift ratio values can occur in early adulthood.


Subject(s)
Lymph Nodes/pathology , Lymphoma/diagnosis , Magnetic Resonance Imaging/methods , Mediastinal Neoplasms/diagnosis , Thymoma/diagnosis , Thymus Hyperplasia/diagnosis , Thymus Neoplasms/diagnosis , Adolescent , Adult , Aged , Aged, 80 and over , Diagnosis, Differential , Female , Humans , Lymphoma/pathology , Male , Mediastinal Neoplasms/pathology , Middle Aged , Thymoma/pathology , Thymus Hyperplasia/pathology , Thymus Neoplasms/pathology , Tomography, X-Ray Computed
11.
Can Assoc Radiol J ; 66(1): 5-15, 2015 Feb.
Article in English | MEDLINE | ID: mdl-24736228

ABSTRACT

The thymus is routinely encountered on cross-sectional imaging studies of the chest. It has a variable appearance, undergoes dynamic changes during periods of stress, and demonstrates numerous different pathologic lesions. Understanding the imaging characteristics of these different lesions facilitates accurate radiographic diagnosis and can prevent unnecessary follow-up imaging and intervention. This article will review normal thymic anatomy and development, thymic hyperplasia and associated medical conditions, and the imaging and pathologic features of various benign and malignant thymic lesions.


Subject(s)
Carcinoid Tumor/diagnostic imaging , Lipoma/diagnostic imaging , Neoplasms, Glandular and Epithelial/diagnostic imaging , Thymus Gland/anatomy & histology , Thymus Hyperplasia/diagnostic imaging , Thymus Neoplasms/diagnostic imaging , Thymus Neoplasms/pathology , Humans , Lymphoma/diagnostic imaging , Mediastinal Cyst/diagnostic imaging , Neoplasms, Glandular and Epithelial/pathology , Thymus Hyperplasia/pathology , Tomography, X-Ray Computed
12.
Clin Radiol ; 69(5): e230-45, 2014 May.
Article in English | MEDLINE | ID: mdl-24581970

ABSTRACT

Myasthenia gravis (MG) is an autoimmune disorder often associated with thymic abnormalities. At onset, thymic lymphoid hyperplasia (TLH) and thymoma can be found in up to 65% and 15% of patients, respectively. Diagnostic imaging is crucial in this setting in order to detect the presence and type of the thymic abnormality and in the preoperative planning, when indicated. Chest radiography has a minor role due to its low accuracy. Computed tomography is the imaging modality of choice, although the differentiation between a small thymoma and TLH that appears as a focal soft-tissue mass may be not possible. Magnetic resonance imaging (MRI) is not usually employed, but it is useful in equivocal cases, especially in differentiating focal TLH from thymoma by using chemical-shift sequences for defining the proper management. In addition, diffusion-weighted (DW)-MRI can differentiate lipid-poor normal/hyperplastic thymus from thymoma and could be useful in differentiating non-advanced from advanced thymomas. Positron emission tomography (PET)-CT is not helpful in distinguishing early from advanced thymoma but can be used to differentiate thymic carcinoma from thymoma. Hereby, we discuss the imaging features of thymic abnormalities in MG, even focusing on novel aspects of chemical-shift and DW-MRI.


Subject(s)
Magnetic Resonance Imaging , Myasthenia Gravis/pathology , Positron-Emission Tomography , Thymoma/diagnosis , Thymus Gland/pathology , Thymus Hyperplasia/diagnosis , Thymus Neoplasms/diagnosis , Tomography, X-Ray Computed , Diagnosis, Differential , Female , Humans , Male , Thymoma/pathology , Thymus Hyperplasia/pathology , Thymus Neoplasms/pathology
13.
J Neuroimmunol ; 395: 578441, 2024 Oct 15.
Article in English | MEDLINE | ID: mdl-39216158

ABSTRACT

INTRODUCTION: Dendritic cells (DCs) are crucial to form ectopic germinal centers (GCs) in the hyperplastic thymus (HT), which are typically found in anti-acetylcholine receptor autoantibody-positive myasthenia gravis (MG) patients. However, the characteristics of such DCs in the HT and their roles in thymic hyperplasia formation remain unclear. METHODS: We collected thymic tissue from MG patients and patients who underwent cardiac surgery. The tissues were cut into sections for immunohistochemistry and immunofluorescence or digested into a single cell suspension for flow cytometry. RESULTS: In addition to formation of ectopic GCs, we found that the proportion of the medulla in the thymic parenchyma was higher than that in the cortex (areacortex/areamedulla, 1.279 vs. 0.6576) in the HT of MG patients. The density of conventional dendritic cells (cDCs) in the HT was 131 ± 64.36 per mm2, whereas in normal thymic tissue, the density was 59.17 ± 22.54 per mm2. The more abundant cDCs expressed co-stimulatory molecules (CD80 and CD86) strongly. Moreover, the more abundant subset was mainly CD141+ DCs (cDC1s), accounting for an increase from 15% to 29%. However, these increased cDC1s appeared to be unrelated to Hassall's corpuscles and ectopic GCs. CONCLUSION: Thymic hyperplasia in MG patients is manifested as an increase in the proportion of the thymic medulla accompanied by increases in the density and functional activation as well as changes in the subset composition of cDCs.


Subject(s)
Dendritic Cells , Myasthenia Gravis , Thymus Gland , Thymus Hyperplasia , Humans , Myasthenia Gravis/pathology , Myasthenia Gravis/immunology , Dendritic Cells/pathology , Male , Female , Adult , Middle Aged , Thymus Hyperplasia/pathology , Thymus Gland/pathology , Thymus Gland/immunology , Aged , Young Adult , Hyperplasia/pathology , Adolescent
14.
Eur J Neurol ; 20(9): 1272-6, 2013 Sep.
Article in English | MEDLINE | ID: mdl-23679930

ABSTRACT

BACKGROUND AND PURPOSE: The differences in the characteristics of thymus histology, coexisting autoimmune diseases and related autoantibodies between anti-muscle-specific receptor tyrosine kinase (MuSK)-antibody (Ab)-positive myasthenia gravis (MG) patients, and anti-acetylcholine receptor (AChR)-Ab-positive MG patients are not clearly defined. METHODS: The types of thymus histology, coexisting autoimmune diseases and associated Abs in 83 MuSK-Ab-positive patients nationwide were investigated and were compared with those in AChR-Ab-positive patients followed at our institute (n = 83). As for the autoantibodies associated with thymoma, titin Abs were measured. RESULTS: Thymoma was not present in any of the MuSK-Ab-positive patients but presented in 21 patients (25.3%) amongst the AChR-Ab-positive patients. Titin Abs were absent in MuSK-Ab-positive patients but positive in 25 (30.1%) of the AChR-Ab-positive patients. Concomitant autoimmune diseases were present in eight MuSK-Ab-positive patients (9.6%) amongst whom Hashimoto's thyroiditis and rheumatoid arthritis predominated, whereas 22 AChR-Ab-positive patients (26.5%) had one or more concomitant autoimmune diseases of which Graves' disease predominated. CONCLUSIONS: Differences in frequency of thymoma and thymic hyperplasia, coexisting autoimmune diseases and autoantibody positivity between MuSK-Ab-positive and AChR-Ab-positive MG were indicated, suggesting that, in contrast with AChR-Ab-positive MG, thymus does not seem to be involved in the pathogenic mechanisms of MuSK-Ab-positive MG.


Subject(s)
Autoantibodies/immunology , Autoantigens/immunology , Autoimmune Diseases/complications , Myasthenia Gravis/complications , Receptor Protein-Tyrosine Kinases/immunology , Receptors, Cholinergic/immunology , Thymus Gland/pathology , Adult , Asian People , Autoantibodies/blood , Autoantigens/blood , Connectin/immunology , Female , Humans , Male , Middle Aged , Myasthenia Gravis/immunology , Myasthenia Gravis/pathology , Radioimmunoassay , Thymoma/complications , Thymoma/pathology , Thymus Hyperplasia/complications , Thymus Hyperplasia/pathology , Thymus Neoplasms/complications , Thymus Neoplasms/pathology
15.
Nat Genet ; 23(2): 176-84, 1999 Oct.
Article in English | MEDLINE | ID: mdl-10508513

ABSTRACT

Gadd45a-null mice generated by gene targeting exhibited several of the phenotypes characteristic of p53-deficient mice, including genomic instability, increased radiation carcinogenesis and a low frequency of exencephaly. Genomic instability was exemplified by aneuploidy, chromosome aberrations, gene amplification and centrosome amplification, and was accompanied by abnormalities in mitosis, cytokinesis and growth control. Unequal segregation of chromosomes due to multiple spindle poles during mitosis occurred in several Gadd45a -/- cell lineages and may contribute to the aneuploidy. Our results indicate that Gadd45a is one component of the p53 pathway that contributes to the maintenance of genomic stability.


Subject(s)
Proteins/genetics , Animals , Apoptosis/genetics , Cell Cycle/genetics , Cell Cycle/physiology , Cell Division/genetics , Cell Transformation, Neoplastic/genetics , Cellular Senescence , Centrosome/metabolism , Embryo, Mammalian/cytology , Female , Fibroblasts/cytology , Fibroblasts/physiology , G1 Phase , Gamma Rays/adverse effects , Gene Deletion , Genes, ras/genetics , Intracellular Signaling Peptides and Proteins , Male , Mice , Mice, Inbred C57BL , Mice, Knockout , Neoplasms/etiology , Neoplasms/genetics , Phenotype , Proteins/physiology , Thymus Hyperplasia/genetics , Thymus Hyperplasia/pathology , GADD45 Proteins
16.
Zhonghua Bing Li Xue Za Zhi ; 41(4): 234-8, 2012 Apr.
Article in Zh | MEDLINE | ID: mdl-22800518

ABSTRACT

OBJECTIVE: To study the clinicopathologic features of primary thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). METHODS: The clinical and pathologic findings were evaluated in 3 cases of biopsy confirmed thymic MALT lymphoma. The clincopathologic features, treatment and prognosis were discussed and literatures reviewed. RESULTS: One male and two female patients presented with asymptomatic mediastinal masses with a history of Sjögren syndrome. They were aged 36, 35 and 41 years respectively, and only one patient had B symptoms. Grossly, all three tumors were encapsulated and had multiple variable-sized cysts on cut-surface. Histopathologically, the normal thymic lobular architecture was effaced by abnormal dense lymphoid infiltration. Prominent lymphoepithelial lesions were formed by centrocyte-like cells infiltrating and expanding Hassall's corpuscles and epithelial cyst lining. All cases showed apparent plasmacytic differentiation. Immunohistochemically, the tumor cells were positive for CD20, CD79a, bcl-2 and negative for CD3, CD5, cyclin D1, CD43, CD10, bcl-6, and CD23. The plasma cells showed kappa light chain restriction. Immunoglobulin heavy chain rearrangement in three cases was confirmed by PCR. All patients were at early stage and received routine chemotherapy with or without radiotherapy after surgical removal. All patients achieved complete remission with 24, 18 and 3 months follow-up, respectively. CONCLUSIONS: Primary thymic MALT lymphoma may be a rare distinctive lymphoma. It can be diagnosed by HE and immunohistochemical study and should be differentiated from reactive lymphoid proliferation, other types of lymphoma and mediastinal thymoma.


Subject(s)
Lymphoma, B-Cell, Marginal Zone/pathology , Thymus Neoplasms/pathology , Adult , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antigens, CD20/metabolism , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cyclophosphamide/therapeutic use , Diagnosis, Differential , Doxorubicin/therapeutic use , Female , Follow-Up Studies , Gene Rearrangement, B-Lymphocyte, Heavy Chain , Humans , Immunoglobulin Heavy Chains/genetics , Keratin-19/metabolism , Lymphoma, B-Cell, Marginal Zone/drug therapy , Lymphoma, B-Cell, Marginal Zone/genetics , Lymphoma, B-Cell, Marginal Zone/metabolism , Lymphoma, B-Cell, Marginal Zone/surgery , Male , Prednisone/therapeutic use , Pseudolymphoma/pathology , Rituximab , Thymus Hyperplasia/pathology , Thymus Neoplasms/drug therapy , Thymus Neoplasms/genetics , Thymus Neoplasms/metabolism , Thymus Neoplasms/surgery , Vincristine/therapeutic use
17.
Pan Afr Med J ; 43: 145, 2022.
Article in French | MEDLINE | ID: mdl-36785681

ABSTRACT

Thymic hyperplasia is an anterior mediastinal mass with a variable clinical presentation. It causes differential diagnostic problems in the pediatric age group and there is no consensus on the therapeutic approach. We here report the case of a 1-month-old infant treated for respiratory distress syndrome. Chest CT scan revealed anterior mediastinal mass, which was excised through median sternotomy. Anatomopathological examination showed thymic hyperplasia. Clinical outcome was satisfactory. This encouraging result suggests that, contrary to what some authors propose, it would be more appropriate to opt for an aggressive therapeutic strategy when managing symptomatic thymic hyperplasia. This is even more justified in a socio-economic context characterised by difficult access to care and follow-up measures limited by patients' means.


Subject(s)
Respiratory Distress Syndrome, Newborn , Thymus Hyperplasia , Infant, Newborn , Humans , Infant , Child , Thymus Hyperplasia/diagnosis , Thymus Hyperplasia/complications , Thymus Hyperplasia/pathology , Developing Countries , Thymus Gland/diagnostic imaging , Thymus Gland/pathology , Respiratory Distress Syndrome, Newborn/etiology , Respiratory Distress Syndrome, Newborn/therapy , Tomography, X-Ray Computed
18.
Lung Cancer ; 166: 76-83, 2022 04.
Article in English | MEDLINE | ID: mdl-35227953

ABSTRACT

BACKGROUND: Non-neoplastic thymic lesions are uncommon findings that corresponds to multiple histological and clinical entities that may be difficult to differentiate from thymic malignancies. In this study, our main objective was to describe the clinical, imaging and pathological characteristics of non-neoplastic thymic lesions in a large cohort of patients. We also aimed at understanding the key factors that led to a decision to surgically resect those lesions. METHODS AND MATERIALS: This is an observational, retrospective study. We enrolled both patients with non-neoplastic thymic lesions - normal thymus, thymic lymphoid/non-lymphoid hyperplasia, and thymic cysts - that had been pathologically-confirmed after surgical resection - , and patients with a thymic lesion that was never operated, based on imaging follow-up. RESULTS: A total of 128 patients were included, 88 of whom underwent surgical resection of the lesion (69%), and 40 patients (31%) had follow-up without surgery. Discovery of the lesion was incidental in 69 (54%) cases; thoracic magnetic resonance imaging was performed in 33 (26%) cases, 85% of which showed apparent decrease in the lesion signal intensity in phase opposition at chemical shift sequences. In the 88 operated patients, there were 34 (39%) normal thymuses, 29 (33%) lymphoid hyperplasias, 6 (7%) non-lymphoid thymic hyperplasias, and 19 (22%) thymic cysts. In the 40 non-operated patients, a major driver for the decision of follow-up was the decrease in the lesion signal intensity in phase opposition at chemical shift sequences, observed in 68% of cases; imaging follow-up of these lesions showed sustained regression in the majority of the cases. CONCLUSIONS: The management of benign thymic lesions requires multidisciplinary assessment. A strategy that integrates clinical and imaging features, including chemical-shift sequences at magnetic resonance imaging, as well as follow-up, allows a better selection of the patients for surgery.


Subject(s)
Lung Neoplasms , Mediastinal Cyst , Thymus Hyperplasia , Thymus Neoplasms , Humans , Mediastinal Cyst/pathology , Mediastinal Cyst/surgery , Retrospective Studies , Thymus Hyperplasia/pathology , Thymus Neoplasms/diagnosis , Thymus Neoplasms/pathology , Thymus Neoplasms/surgery
19.
Curr Med Imaging ; 18(1): 99-102, 2022.
Article in English | MEDLINE | ID: mdl-34342259

ABSTRACT

INTRODUCTION: True thymic hyperplasia following chemotherapy has been described mostly in children. There are a few cases of thymus hyperplasia that have been reported in breast cancer patients. Diagnosis of this unusual entity is very crucial to pretend unnecessary surgery or interventional diagnostic procedures. CASE PRESENTATION: We report a case of thymus hyperplasia in a patient who was operated and treated with adjuvant chemotherapy for stage 2 breast cancer two years ago. In the follow-up CT scans, an anterior mediastinal mass was noted. Radiologic evaluation and follow-up revealed thymus enlargement. DISCUSSION: Thymic hyperplasia following chemotherapy has been described in both children and adults, but occurs mostly in children and adolescents treated for lymphoma and several other types of tumors. Few cases are reported in literature describing thymus hyperplasia following chemotherapy in a breast cancer patient. The imaging findings of thymic hyperplasia on CT, MRI and PET CT are discussed. CONCLUSION: Radiologists must be aware of this unusual finding in breast cancer patients treated with chemotherapy to guide the clinicians appropriately in order to avoid unnecessary surgical intervention, additional invasive diagnostic procedures, or chemotherapy.


Subject(s)
Breast Neoplasms , Thymus Hyperplasia , Adolescent , Adult , Breast Neoplasms/diagnostic imaging , Child , Female , Humans , Positron Emission Tomography Computed Tomography , Thymus Hyperplasia/diagnostic imaging , Thymus Hyperplasia/pathology , Tomography, X-Ray Computed
20.
Tumori ; 97(2): 191-5, 2011.
Article in English | MEDLINE | ID: mdl-21617714

ABSTRACT

AIMS AND BACKGROUND: Thymic tumors (thymomas and thymic carcinomas) represent 50% of all mediastinal tumors. Thymomas usually express high levels of somatostatin receptors, which enable in vivo imaging with 111In-DTPA-octreotide (OctreoScan®). The aim of this study was to further investigate the role of radionuclide techniques in the diagnosis, staging and follow-up of these tumors. METHODS: Eight patients (5 women, 3 men, age range 35-79 years; mean ± SD 56.1 ± 15.8 years) entered the study. In 4 patients, myasthenia gravis was the presenting symptom. 111In-DTPA-octreotide scan was performed within 3 weeks after contrast enhanced CT and/or MRI. Planar and tomographic images were acquired within 24 hours of the injection of 111 MBq OctreoScan. The scintigraphic results were defined in correlation with the histological findings. RESULTS: Histology revealed thymoma in 3 patients, thymic carcinoma in 1, insular carcinoma of presumably thymic origin in 1, thymic carcinoid in 1, and thymic hyperplasia in 2 patients. Two thymomas were at stage I, 1 thymoma and 1 thymic carcinoma at stage II, 1 insular carcinoma of presumably thymic origin at stage IV, and 1 thymic carcinoid at stage IV. OctreoScan consistently accumulated in primary and/or metastatic sites of thymic tumors while no radiotracer uptake was detected in the 2 patients with benign thymic hyperplasia. In 1 patient with a very large mediastinal mass (13 cm in largest diameter) and multiple metastatic deposits in the lungs, OctreoScan scintigraphy showed a large area of pathological uptake in the anterior mediastinum and a small area of focal uptake in the cervical-dorsal region of the right lung corresponding to a lymph node expressing somatostatin receptors. CONCLUSIONS: OctreoScan is avidly taken up by thymic tumors, enabling the diagnosis of these tumors and a better evaluation of their extension. It does not accumulate in thymic hyperplasia, thus allowing the differential diagnosis between these 2 pathological conditions. In patients affected by myasthenia gravis, OctreoScan scintigraphy can play an important role in characterizing thymic masses.


Subject(s)
Somatostatin/analogs & derivatives , Thymoma/diagnostic imaging , Thymus Hyperplasia/diagnostic imaging , Thymus Neoplasms/diagnostic imaging , Tomography, Emission-Computed, Single-Photon/methods , Adult , Aged , Carcinoid Tumor/diagnostic imaging , Diagnosis, Differential , Female , Humans , Male , Middle Aged , Myasthenia Gravis/diagnostic imaging , Neoplasm Staging , Octreotide/analogs & derivatives , Pentetic Acid , Radiopharmaceuticals , Thymoma/pathology , Thymus Hyperplasia/pathology , Thymus Neoplasms/pathology , Tomography, X-Ray Computed
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