A patient with thymoma associated immunodeficiency syndrome (Good's syndrome) and bronchiectasis was retrospectively analyzed. Good's syndrome is a rare condition of immunodeficiency that is characterized by thymoma and hypogammaglobulinemia. It is important to bear in mind that Good's syndrome should be included in the differential diagnosis When patients repeatedly visited for bronchiectasis or infection, we should alert to their immune state and history of thymoma. Early screening of immunological status and aggressive correction of immune deficiency are beneficial to improving the prognosis to patients with Good's syndrome.
Agammaglobulinemia/complications* ; Bronchiectasis/complications* ; Humans ; Retrospective Studies ; Thymoma/complications* ; Thymus Neoplasms/complications*

This paper introduced surgical treatment of malignancy-related superior vena cava syndrome. Typical cases were presented with diagnostic radiology results. Authors focused on the main approach to the malignancy-related superior vena cava syndrome of surgery. In order to make it simple for junior doctors to learn and practice, all 4 operation methods were described in details. The writer hopes it would be helpful for all the young thoracic surgeons.
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Humans ; Neoplasm Metastasis ; Superior Vena Cava Syndrome ; diagnostic imaging ; etiology ; surgery ; Thymoma ; complications ; diagnostic imaging ; surgery ; Thymus Neoplasms ; complications ; diagnostic imaging ; surgery ; Vena Cava, Superior ; diagnostic imaging ; pathology ; surgery

OBJECTIVETo explore the role of TNFα induced protein 3 interacting protein 1 (TNIP1) in the pathogenesis of myasthenia gravis (MG) among patients with thymoma.

METHODSFrom December 2014 to March 2015, 11 patients with MG associated thymoma (MGT) and 11 non-MG thymoma (NMGT) patients receiving thymectomy were selected. Thymus specimens were obtained during surgery, and peripheral venous blood samples were obtained before the surgery. For the MGT cohorts, peripheral venous blood samples were also collected at 3-6 months after the surgery. TNIP1 mRNA was determined with quantitative real-time PCR (qPCR), and its protein expression was evaluated by Western blotting.

RESULTSFor both thymus specimen and peripheral blood samples, the levels of TNIP1 mRNA and protein in the MGT group were both significantly lower than those of the NMGT patients (P<0.05). For the MGT group, the levels of TNIP1 mRNA and protein of the peripheral blood samples have increased following thymus resection compared with before the surgery.

CONCLUSIONReduced TNIP1 expression may have a role in the pathogenesis of MG for patients with thymoma. Thymectomy may help to recover the expression of TNIP1 among such patients.

Blotting, Western ; DNA-Binding Proteins ; genetics ; metabolism ; Female ; Gene Expression ; Genetic Predisposition to Disease ; genetics ; Humans ; Male ; Myasthenia Gravis ; complications ; genetics ; Reverse Transcriptase Polymerase Chain Reaction ; Thymectomy ; Thymoma ; complications ; surgery ; Thymus Neoplasms ; complications ; surgery

OBJECTIVETo evaluate the different pathological and clinical characteristics of thymomas with and without myasthenia gravis (MG) and to determine whether the presence of MG influences the prognosis in thymoma patients.

METHODSThe clinical data from 228 consecutive patients (median sternotomy were used in 153, video-assisted thoracoscopic themectomy were used in 75) operated on from January 1992 to December 2007 was analyzed retrospectively. These thymoma patients had been subdivided into two groups: thymoma with MG (n = 125) and thymoma without MG (n = 103). All thymic epithelial tumors were classified according to the WHO histologic classification and the Masaoka clinical staging system. The result was evaluated according to the Myasthenia Gravis Foundation of America's criterion. The clinical features of the 2 test was compared between the two groups by χ² test, and the survival were compared between the two groups by Cox analysis.

RESULTSThere were no peri-operative deaths. 19 cases were inoperable (6 in the group with MG, 13 without MG (χ² = 4.52, P = 0.035)). The proportions of type A and thymic carcinoma were 0 in the group with MG, 10.5% (11/103) and 11.6% (12/103) respectively in the group without MG. According to the Masaoka's clinical staging, in the group MG, 24.8% (31/125) patients were stage III and IV; in the group without MG, 33.0% (34/103) patients were stage III and IV. There was a significant difference between hyperplastic paraneoplastic thymus coexisting in 28.8% (36/125) patients with MG and only 5.8% (6/103) in patients without MG (χ² = 20.91, P = 0.000) Microthymoma was identified in the paraneoplastic thymus of 3 patients with MG. There were 198 patients followed up, the rate was 86.8% (198/228). There was no recurrence in patients with type A and a few patients with type AB, B1, B2, B3 thymoma and thymic carcinoma recurred. The actuarial 5- and 10-year survival rates were 89.3% and 81.2% for patients with MG respectively, and 90.0% and 78.9% for patients without MG respectively. Within 5 years postoperatively, 6 of 9 patients with MG died of myasthenia crisis, while 6 out of 7 deaths in patients without MG were attributable to inoperable tumors (stage IV) and thymic carcinoma.

CONCLUSIONSThe existence of myasthenia gravis has little influence on the prognosis of thymomas, but it is good for early diagnosis and treatment. Extended thymectomy should be performed to all patients with thymoma, no matter they have myasthenia gravis or not. The main cause of death is myasthenia crisis for thymoma patients with MG and stage IV and (or) thymic carcinoma for patients without MG.

Humans ; Myasthenia Gravis ; complications ; pathology ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Neoplasms, Glandular and Epithelial ; complications ; pathology ; surgery ; Postoperative Period ; Prognosis ; Retrospective Studies ; Sternotomy ; Survival Rate ; Thoracic Surgery, Video-Assisted ; Thymectomy ; Thymoma ; complications ; pathology ; surgery ; Thymus Neoplasms ; complications ; pathology ; surgery

OBJECTIVETo study the effect of spleen lymphocytes on the splenomegaly by hepatocellular carcinoma-bearing mouse model.

METHODSCell counts, cell cycle distribution, the percentage of lymphocytes subsets and the levels of IL-2 were measured, and two-dimensional gel electrophoresis (2-DE) was used to investigate the relationship between spleen lymphocytes and splenomegaly in hepatocellular carcinoma-bearing mice.

RESULTSCompared with the normal group, the thymus was obviously atrophied and the spleen was significantly enlarged in the tumor-bearing group. Correlation study showed that the number of whole spleen cells was positively correlated with the splenic index. The cell diameter and cell-cycle phase distribution of splenocytes in the tumor-bearing group showed no significant difference compared to the normal group. The percentage of CD3+ T lymphocytes and CD8+ T lymphocytes in spleen and peripheral blood of tumor-bearing mice were substantially higher than that in the normal mice. Meanwhile, the IL-2 level was also higher in the tumor-bearing group than in the normal group. Furthermore, two dysregulated protein, β-actin and S100-A9 were identified in spleen lymphocytes from H22-bearing mice, which were closely related to cellular motility.

CONCLUSIONIt is suggested that dysregulated β-actin and S100-A9 can result in recirculating T lymphocytes trapped in the spleen, which may explain the underlying cause of splenomegaly in H22-bearing mice.

Animals ; Carcinoma, Hepatocellular ; complications ; Cell Cycle ; Female ; Liver Neoplasms ; complications ; Lymphocytes ; physiology ; Mice ; Mice, Inbred ICR ; Neoplasms, Experimental ; therapy ; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization ; Spleen ; cytology ; pathology ; Splenomegaly ; etiology ; therapy ; Thymus Gland

The association of thymoma with pure red cell aplasia has been well documented, but amegakaryocytic thrombocytopenia is not a recognized paraneoplastic syndrome complicating thymoma. We report a case of thymoma-complicated pure red cell aplasia and amegakaryocytic thrombocytopenia in a 73-yr-old woman. Pure red cell aplasia was diagnosed seven months after the detection of thymoma. One year after the diagnosis of pure red cell aplasia and seven months after thymectomy, bone marrow aspiration and biopsy showed an absence of megakaryocytes, marked erythroid hypoplasia with normal myeloid series. A diagnosis of amegakaryocytic thrombocytopenia and pure red cell aplasia was made. Oral steroid maintenance therapy resulted in recovery of platelet count. She has still transfusion-dependant anemia but platelet and neutrophil counts had been maintained in normal range for more than five months, until the last follow-up. We think that autoreactive T cells may induce a clinical autoimmune response even after eradication of thymoma, and aplastic anemia as a late complication following thymectomy was described in previous cases. This patient also has to be under a close observation because of the possibility to evolve into aplastic anemia.
Aged ; Bone Marrow/pathology ; Female ; Humans ; Imidazoles/therapeutic use ; Megakaryocytes/pathology ; Pregnadienetriols/therapeutic use ; Red-Cell Aplasia, Pure/complications/*diagnosis ; Thrombocytopenia/*diagnosis/drug therapy/*etiology ; Thymectomy/*adverse effects ; Thymoma/*complications/diagnosis/surgery ; Thymus Neoplasms/*complications/diagnosis/surgery

OBJECTIVETo study the expression of CD20 in thymomas and its clinical significance.

METHODSOne hundred and seventy-nine cases of thymoma were enrolled into the study. The histologic diagnosis was reviewed by two experienced pathologists on the basis of the 2004 WHO classification. One hundred and two cases were selected for immunohistochemical study for CD20, pancytokeratin, TdT, CD3, CD43, CD99 and S-100 protein. The cases were further categorized into two groups, according to the association with clinical evidence of myasthenia gravis. The immunostaining pattern was then statistically analyzed.

RESULTSAmongst the 102 cases studied, 7 cases belonged to type A thymoma, 32 cases type AB thymoma, 17 cases type B1 thymoma, 15 cases type B2 thymoma, 17 cases type B3 thymoma and 14 cases thymic carcinoma. The expression rates of CD20 in neoplastic epithelial cells of type A, type AB, type B1, type B2 and type B3 thymomas and thymic carcinomas were 3/7, 84.4% (27/32), 1/17, 2/15, 0/17, 0/14, respectively. The proportions of CD20-positive lymphocytes in the background were 3/7, 18.8% (6/32), 14/17, 11/15, 11/17, 6/14, respectively. The proportion of CD20-positive intra-tumoral B lymphocytes in the group of thymomas with myasthenia gravis was 67.5% (22/40), in contrast to 35.5% (22/62) in those without myasthenia gravis.

CONCLUSIONSThe neoplastic epithelial cells in cases of type A and type AB thymoma, as well as few cases of type B1 and B2 thymoma, express CD20. The immunostain highlights the presence of oval, stellate or spindly cells. Thymomas associated with myasthenia gravis contain a significant population of CD20-positive intra-tumoral B lymphocytes. Type AB thymomas may be originated from different populations of cells, rather than a simple admixture of type A and B thymoma cells.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Antigens, CD20 ; metabolism ; B-Lymphocytes ; immunology ; pathology ; Child ; Epithelial Cells ; immunology ; pathology ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; complications ; immunology ; pathology ; Thymoma ; classification ; complications ; immunology ; pathology ; Thymus Neoplasms ; classification ; complications ; immunology ; pathology ; Young Adult

Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatening. There are no reports of granulomatous lung disease in patients with Good's syndrome, although it has been observed in patients with common variable immunodeficiency, of which Good's syndrome is a subset. We describe a 53-year-old male thymoma patient who presented with respiratory symptoms caused by granulomatous lung disease and an opportunistic infection. He died of uncontrolled fungal infection despite repeated intravenous immunoglobulin and supportive care. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with severe recurrent infections, especially opportunistic infections.
Fatal Outcome ; Granuloma, Respiratory Tract/diagnosis/*etiology/therapy ; Humans ; Immunologic Deficiency Syndromes/*complications/immunology/pathology ; Lung Diseases/diagnosis/*etiology/therapy ; Male ; Middle Aged ; Thymoma/*complications/immunology/pathology ; Thymus Neoplasms/*complications/immunology/pathology

OBJECTIVETo study the prognostic and clinical relevance of histologic subtyping of thymoma according to the World Health Organization (WHO) classification.

METHODSThe clinicopathologic features of 108 patients with thymoma removed surgically were retrospectively reviewed. The histologic diagnosis of the tumors was made on the basis of 2004 WHO classification by two experienced pathologists. The correlation between Masaoka tumor stage, WHO histologic subtype, completeness of resection, presence of myasthenia gravis, other clinical parameters (including age, gender and tumor size) and survival was studied.

RESULTSAccording to WHO classification, there were 7 cases (6.5%) of type A thymoma, 19 cases (17.6%) of type AB thymoma, 23 cases (21.3%) of type B1 thymoma, 19 cases (17.6%) of type B2 thymoma, 27 cases (25.0%) of type B3 thymoma and 13 cases (12.0%) of type C thymoma. According to Masaoka tumor staging, 36 cases (33.3%) were in stage I, 34 cases (31.5%) in stage II, 27 cases (25.0%) in stage III and 11 cases (10.2%) in stage IV(a). The association between histologic subtype and Masaoka tumor stage was statistically significant (P = 0.000). The 5-year survival rates of type A, AB, B1, B2 and B3 thymoma cases were 100%, 100%, 93%, 83% and 43%, respectively; while the 10-year survival rates were 100%, 100%, 81%, 70% and 33%, respectively. The median survival time of type C thymoma was 62.5 months. Type B2 and B3 thymoma cases had an intermediate prognostic ranking in comparison with type C thymoma and other groups (P = 0.000). The 5-year survival rates of tumors in stage I, II and III were 100%, 77% and 54%, respectively; while the 10-year survival rates were 100%, 70% and 27%, respectively. The median survival time of patients in stage IV(a) was 14.0 months. Masaoka tumor stage was highly significant in predicting survival of patients (P = 0.000). On multivariate analysis, Masaoka tumor stage was an independent predictive factor for survival (P = 0.027). On the other hand, the WHO subtype (type A to B1 versus type B2 to B3 versus type C) and completeness of resection could predict the tumor-related survival.

CONCLUSIONSThe Masaoka tumor stage is the single most important prognostic factor of thymoma. The WHO histologic subtype and completeness of resection affect mainly the post-operative survival. The classification of thymoma may also reflect the clinical behavior of the tumor. Type A, AB and B1 thymomas belong to the low-risk group, while type B2 and B3 thymomas have an intermediate prognostic ranking. Type C thymoma carries the worst prognosis.

Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Myasthenia Gravis ; etiology ; Prognosis ; Survival Analysis ; Thymoma ; classification ; complications ; diagnosis ; pathology ; Thymus Neoplasms ; classification ; complications ; diagnosis ; pathology ; World Health Organization

OBJECTIVETo explore the clinical significance of serum anti-ryanodine receptor (RyR) antibody in the diagnosis of myasthenia gravis (MG).

METHODSThe crude sarcoplasmic reticulum was prepared from rabbit skeletal muscle, and then purified by differential centrifugation to produce the antigen. The serum anti-RyR antibody levels in 74 patients with MG (including 21 patients with comorbidic thymomas) were determined with ELISA.

RESULTSWestern blot demonstrated the presence of RyR in purified crude sarcoplasmic reticulum. The positive rate of anti-RyR antibody was significantly higher in MG patients who had comorbidic thymoma compared with those who had no such comorbidity (P < 0.01). Also, the positive rate was closely correlated with the severity of MG.

CONCLUSIONSerum anti-RyR antibody test is helpful in the diagnosis of MG associated with thymoma and can be used to judge the outcome of MG.

Autoantibodies ; blood ; Humans ; Myasthenia Gravis ; blood ; complications ; immunology ; Ryanodine Receptor Calcium Release Channel ; immunology ; Thymoma ; complications ; Thymus Neoplasms ; complications