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*

A 53-year-old male patient presented with hypopsia of his right eye for 2 months and lower extremities weakness for 8 days. Thoracic MRI demonstrated a lesion at T3 level appearing as hyperintense on T2-weighted images with non-enhancement by contrast medium and demyelinating lesion was considered. Aquaporin-4-Ab was positive and the antibody titer was 1:320 in serum. The diagnosis of neuromyelitis optica spectrum disorders was made. In addition, systemic lupus erythematosus and thymoma coexisted in this patient. After methylprednisolone impact treatment, plasma exchange and immunosuppressive therapy, the right vision and lower extremities weakness of the patient were improved.
Anti-Inflammatory Agents ; therapeutic use ; Antibodies ; blood ; Aquaporin 4 ; immunology ; Humans ; Lupus Erythematosus, Systemic ; complications ; Male ; Methylprednisolone ; therapeutic use ; Middle Aged ; Neuromyelitis Optica ; complications ; diagnostic imaging ; drug therapy ; Thymoma ; complications ; Treatment Outcome

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

BACKGROUND: Thymoma is frequently associated with myasthenia gravis (MG). However, whether MG is a factor for the outcome of patients with thymoma following complete thymectomy remains unknown. The aim of this study is to investigate the effect of thymoma with MG prognostic factors. METHODS: A retrospective analysis of The Chinese Alliance for Research in Thymomas (ChART) database within 1992-2012 complete cases 875 cases, 20 years follow-up data analysis thymic tumor tissue type credits and MG, Masaoka staging and prognosis, postoperative adjuvant therapy and relationship with the prognosis of surgical removal of the way. RESULTS: Thymic tumor tissue type credit has correlation with MG, difference was statistically significant (χ²=24.908, P<0.001). MG: incidence of B2 type (58/178, 32.58%) > B3 type (65/239, 27.20%) > B1 (27/132, 20.45%) > AB (43/267, 16.10%) > type A, 10.17% (6/59), Masaoka stage has no correlation with MG (χ²=0.365, P=1.365). Survival analysis showed that the WHO classification, Masaoka stage associated with prognosis (P<0.05), and whether the merger MG (χ²=0.113, P=0.736), postoperative adjuvant radiotherapy (χ²=0.380, P=0.538) has nothing to do with the prognosis, postoperative adjuvant chemotherapy is associated with poor prognosis (χ²=14.417, P<0.001). Whether has nothing to do with the prognosis of the thymus resection (χ²=1.548, P=1.548), whether the whole correlated with the curative effect of thymus excision with MG (χ²=24.695, P<0.001). CONCLUSIONS Thymoma patients with MG and extended thymectomy have no correlation with prognosis. Extended thymectomy can improve the effect of MG patients.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; complications ; diagnosis ; surgery ; Prognosis ; Retrospective Studies ; Survival Analysis ; Thymoma ; complications ; Young Adult

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

Humans ; Male ; Middle Aged ; Positron-Emission Tomography ; Situs Inversus ; complications ; pathology ; Thymoma ; diagnosis ; pathology

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 investigate the clinical and laboratory features of 2 cases of pure red cell aplasia (PRCA) with thymoma/T-cell large granular lymphocyte leukemia (T-LGLL), and to improve the recognition of the disease and the role of lymphocyte in its mechanism.

METHODSTwo cases of PRCA with thymoma/T-LGLL were reported and the related literatures were reviewed.

RESULTSCase 1 was a 63-years old male with hemoglobin level of 54 g/L at admission. Case 2 was a 52-years old female with hemoglobin level of 79 g/L at admission. They were both diagnosed as PRCA with thymoma before admission to our hospital and had no benefit from their thymectomy. Further examinations in our hospital showed that CD3⁺CD4⁻CD8⁺CD57⁺ large granular lymphocytes amplified with clonal TCR rearrangement in their peripheral blood. The diagnosis of PRCA with thymoma/T-LGLL was clarified. Case 1 did not respond to any of the frontline therapies while case 2 responded completely to cyclosporine.

CONCLUSIONBoth thymoma and T-LGLL could be the cause of secondary PRCA, lymphocyte proliferation may play critical role in the pathogenesis.

Female ; Humans ; Leukemia, Large Granular Lymphocytic ; complications ; Male ; Middle Aged ; Red-Cell Aplasia, Pure ; complications ; Thymoma ; complications

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