Common Bile Duct Angiosarcoma / 대한췌담도학회지

Angiosarcoma is a very rare form of neoplasm derived from soft tissue. It is reported even more rarely in hepatobiliary system. Because of its nonspecific symptoms and difficulty in diagnosis, angiosarcoma is often presented in a progressed state and often results in poor prognosis. To our best knowledge, there has been no report regarding angiosarcoma in common bile duct worldwide. We report a case of 77-year-old-woman presented with right upper abdominal pain diagnosed as angiosarcoma in common bile duct.

Findings of Cardiac Magnetic Resonance Imaging in Hypertrophic Cardiomyopathy after 16 Years

A 58-year-old man had been diagnosed with non-obstructive hypertrophic cardiomyopathy (HCMP) according to echocardiography findings 16 years ago. Echocardiography showed ischemic cardiomyopathy (CMP)-like features with decreased systolic function but a non-dilated chamber. Coronary angiography was performed but showed a normal coronary artery. Cardiac magnetic resonance imaging (MRI) revealed multifocal transmural and subepicardial delayed-enhancing areas at the anteroseptal, septal, and inferoseptal left ventricular (LV) wall, and wall thinning and decreased motion of the anteroseptal LV wall. Findings of ischemic CMP-like features by echocardiography suggested microvascular dysfunction. This late stage of HCMP carries a high risk of sudden death. Cardiac MRI evaluation may be necessary in cases of ischemic CMP-like features in HCMP. In this case, the diagnosis of end-stage HCMP with microvascular dysfunction was confirmed by using cardiac MRI after a follow-up period of more than 16 years.

Use of Bortezomib as Anti-Humoral Therapy in Kidney Transplantation

This study aimed to investigate the effect of bortezomib in the desensitization and treatment of acute antibody mediated rejection (AAMR) in kidney transplantation. Nine patients who received bortezomib therapy for desensitization (DSZ group, n = 3) or treatment of AAMR (AAMR group, n = 6) were included in this study. In the DSZ group, 2 patients required DSZ owing to positive cross match and 1 owing to ABO mismatch with high baseline anti-ABO antibody titer (1:1,024). Bortezomib was used at 1, 3, 8, and 11 days from the start of the treatment. In the AAMR group, 3 patients showed full recovery of allograft function after bortezomib use and decrease in donor specific anti-HLA antibody (HLA-DSA). However, 3 patients did not respond to bortezomib and experienced allograft failure. In the DSZ group, negative conversion of T-CDC (complement-dependent cytotoxicity) was achieved, and HLA-DSA was decreased to lower than a weak level (median fluorescence intensity [MFI] < 5,000) in 2 patients. In the case of ABO mismatch kidney transplantation, the anti-A/B antibody titer decreased to below the target (< or = 1:16) after bortezomib therapy. Therefore, bortezomib could be an alternative therapeutic option for desensitization and treatment of AAMR that is unresponsive to conventional therapies.