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Jianyang Wang, Shulian Wang, Yongwen Song, Xinfan Liu, Jing Jin, Weihu Wang, Zihao Yu, Yueping Liu, Yexiong Li Department of Radiation Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing,People’s Republic of China Purpose: To investigate the efficacy and toxicity of postoperative intensity-modulated radiation therapy (IMRT) for patients with soft tissue sarcomas (STSs) in the extremities and trunk wall. Patients and methods: Eighty patients with localized STSs in the extremities and trunk wall treated with function-conserving surgery and postoperative IMRT were analyzed. The primary locations were in the extremities in 51 patients and in the trunk wall in 29 patients. The margins were positive in nine patients and negative in 71 patients. The median dose of IMRT was 64Gy. Results: At a median follow-up time of 38months, eight patients developed local recurrences. The 5-year local control (LC) rate was 88.1%. The patients with negative margins exhibited much better LC than did those with positive margins (90% vs 64.8%, P=0.023). Multivariate analysis revealed that positive margin was an independent risk factor for LC. The 5-year distant metastasis-free survival, disease-free survival, and overall survival rates were 75.2%, 72.6%, and 83.6%, respectively. Large tumor size (>5cm) was significantly associated with poor overall survival. Edema and joint stiffness were observed in 17.6% and 9.8% of patients with extremity STSs, respectively. Conclusion: IMRT provides excellent LC and low toxicity for patients with STSs in the extremities and trunk wall. Keywords: soft tissue sarcoma, extremities and trunk wall, intensity-modulated radiation therapy, local control, toxicitiess

Minji Jennifer Kim1, Yong Suk Jo2, Jee Hyun Kim2, Yong-Jin Kim3, Kyung Hwan Kim4, Eun Bong Lee2, Yeong Wook Song2, Eun Young Lee21School of Medicine, University of Manchester, Manchester, UK; 2Division of Rheumatology, 3Division of Cardiology, Department of Internal Medicine, 4Department of Thoracic Surgery, Seoul National University College of Medicine, Seoul, Republic of KoreaAbstract: Budd–Chiari syndrome has been described as a late complication of Behçet’s disease. Although the mortality rate associated with Behçet’s disease is low, it can escalate in the presence of Budd–Chiari syndrome and may be further complicated by intracardial thrombus formation. It is therefore important to detect and initiate management early in the disease course. The imaging modalities of choice should be minimally invasive as certain procedures may aggravate Behçet’s disease by initiating a thrombosis or aggravating an existing one. In Behçet’s disease-induced Budd–Chiari syndrome, cardiac investigation is crucial in the work-up in order to identify any cardiac involvement and determine the etiology of intracardial thrombus. Furthermore, the treatment should ultimately focus on controlling the activity of Behçet’s disease. We report an unusual case of Behçet’s disease presenting with Budd–Chiari syndrome complicated by intracardial thrombus in a young Korean man.Keywords: Behçet’s disease, Budd–Chiari syndrome, intracardial thrombus

Background: Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown aetiology, frequently accompanying multiple lymphadenopathy. It often mimics malignant lymphoma, and immunohistochemical and molecular studies are needed for definite diagnosis. Aims: To aid in diagnosis and understand the pathogenesis of the disease by clarifying lymph node (LN) pathology in AOSD. Methods: Thirteen biopsies (one follow up biopsy) and medical records of 12 patients were reviewed. Immunohistochemistry, polymerase chain reaction for T cell receptor γ chain (TCRγ) and immunoglobulin heavy chain gene rearrangement, and Epstein-Barr virus in situ hybridisation were performed. Results: Histologically, LN lesions were classified into four patterns. The most common (six biopsies) showed paracortical hyperplasia, with prominent vascular proliferation, scattered large B/T immunoblasts, and infiltration by reactive lymphocytes and inflammatory cells. In the second pattern (two biopsies), paracortical hyperplasia was accompanied by massive sinus histiocytosis and S-100 positive histiocyte aggregates. The third pattern (three patients) showed an exuberant immunoblastic reaction, in the form of patchy/diffuse infiltration of large T immunoblasts with high mitotic activity, although clonal rearrangement of the TCRγ gene was not detected. The fourth pattern showed distinct follicular hyperplasia (two cases). One patient with a follow up biopsy showed a pattern change from pronounced follicular hyperplasia to atypical paracortical hyperplasia. Conclusions: AOSD LN lesions show a dynamic histological spectrum, including atypical paracortical hyperplasia, burnt out histiocytic reaction, exuberant immunoblastic reaction, and follicular hyperplasia. During the course of disease, LN reactivity changes and mixed B and T cells are involved in the pathogenesis.

Anti-double stranded(ds) DNA antibody is one of markers of systemic lupus erythematosus (SLE). Two human monoclonal anti-DNA antibody-producing cell lines were established from two SLE patients. One cell line secreted IgG isotype antibody (KSUG) and the other secreted IgM isotype antibody (KSUN). The light chains of the two immunoglobulins were lambda chains. The nucleotide sequences for the immunoglobulin variable region genes of the two antibodies were determined and compared to germline sequences. The heavy and lambda light chains of KSUG were VH3 family and V lambda IIIb, respectively. The heavy and lambda light chains of KSUN were VH4 family and V lambda IX, respectively. Antibody KSUG, IgG isotype, showed somatic mutations, whereas KSUN, IgM isotype, used the germline gene without mutation. These findings reconfirm the current paradigms that IgM anti-DNA antibodies are produced by utilizing germline genes whereas IgG anti-DNA antibodies are produced by somatic mutations.