Purpose: This multicenter, open-label, phase II trial evaluated the efficacy and safety of bortezomib combined with dexamethasone for the treatment of relapsed/refractory cutaneous T-cell lymphoma (CTCL) in previously treated patients across 14 institutions in South Korea. Materials and Methods: Between September 2017 and July 2020, 29 patients with histologically confirmed CTCL received treatment, consisting of eight 4-week cycles of induction therapy followed by maintenance therapy, contingent upon response, for up to one year. The primary endpoint was the proportion of patients achieving an objective global response. Results: Thirteen of the 29 patients (44.8%) achieved an objective global response, including two complete responses. The median progression-free survival (PFS) was 5.8 months, with responders showing a median PFS of 14.0 months. Treatment-emergent adverse events were generally mild, with a low incidence of peripheral neuropathy and hematologic toxicities. Despite the trend toward shorter PFS in patients with higher mutation burdens, genomic profiling before and after treatment showed no significant emergence of new mutations indicative of disease progression. Conclusion This study supports the use of bortezomib and dexamethasone as a viable and safe treatment option for previously treated CTCL, demonstrating substantial efficacy and manageability in adverse effects. Further research with a larger cohort is suggested to validate these findings and explore the prognostic value of mutation profiles.

Purpose: This multicenter, open-label, phase II trial evaluated the efficacy and safety of bortezomib combined with dexamethasone for the treatment of relapsed/refractory cutaneous T-cell lymphoma (CTCL) in previously treated patients across 14 institutions in South Korea. Materials and Methods: Between September 2017 and July 2020, 29 patients with histologically confirmed CTCL received treatment, consisting of eight 4-week cycles of induction therapy followed by maintenance therapy, contingent upon response, for up to one year. The primary endpoint was the proportion of patients achieving an objective global response. Results: Thirteen of the 29 patients (44.8%) achieved an objective global response, including two complete responses. The median progression-free survival (PFS) was 5.8 months, with responders showing a median PFS of 14.0 months. Treatment-emergent adverse events were generally mild, with a low incidence of peripheral neuropathy and hematologic toxicities. Despite the trend toward shorter PFS in patients with higher mutation burdens, genomic profiling before and after treatment showed no significant emergence of new mutations indicative of disease progression. Conclusion This study supports the use of bortezomib and dexamethasone as a viable and safe treatment option for previously treated CTCL, demonstrating substantial efficacy and manageability in adverse effects. Further research with a larger cohort is suggested to validate these findings and explore the prognostic value of mutation profiles.

A schwannoma is a benign soft tissue tumor derived from Schwann cells of the nerve sheath. The lateral femoral cutaneous nerve is a sensory nerve originating from the second and third lumbar vertebra nerve roots, passing under the inguinal ligament and dividing into anterior and posterior branches. The anterior branch which is a major division and the main nerve that causes meralgia paresthetica supplies the knee joint from the anterior thigh. In addition, the posterior branch supplies the lateral thigh at the greater trochanter level.This paper reports the case of a 52-year-old woman with a very rare schwannoma in the terminal twig of posterior branch of the lateral femoral cutaneous nerve of right thigh protruded through the fascia lata with literatures review.

Purpose: This multicenter, open-label, phase II trial evaluated the efficacy and safety of bortezomib combined with dexamethasone for the treatment of relapsed/refractory cutaneous T-cell lymphoma (CTCL) in previously treated patients across 14 institutions in South Korea. Materials and Methods: Between September 2017 and July 2020, 29 patients with histologically confirmed CTCL received treatment, consisting of eight 4-week cycles of induction therapy followed by maintenance therapy, contingent upon response, for up to one year. The primary endpoint was the proportion of patients achieving an objective global response. Results: Thirteen of the 29 patients (44.8%) achieved an objective global response, including two complete responses. The median progression-free survival (PFS) was 5.8 months, with responders showing a median PFS of 14.0 months. Treatment-emergent adverse events were generally mild, with a low incidence of peripheral neuropathy and hematologic toxicities. Despite the trend toward shorter PFS in patients with higher mutation burdens, genomic profiling before and after treatment showed no significant emergence of new mutations indicative of disease progression. Conclusion This study supports the use of bortezomib and dexamethasone as a viable and safe treatment option for previously treated CTCL, demonstrating substantial efficacy and manageability in adverse effects. Further research with a larger cohort is suggested to validate these findings and explore the prognostic value of mutation profiles.

A schwannoma is a benign soft tissue tumor derived from Schwann cells of the nerve sheath. The lateral femoral cutaneous nerve is a sensory nerve originating from the second and third lumbar vertebra nerve roots, passing under the inguinal ligament and dividing into anterior and posterior branches. The anterior branch which is a major division and the main nerve that causes meralgia paresthetica supplies the knee joint from the anterior thigh. In addition, the posterior branch supplies the lateral thigh at the greater trochanter level.This paper reports the case of a 52-year-old woman with a very rare schwannoma in the terminal twig of posterior branch of the lateral femoral cutaneous nerve of right thigh protruded through the fascia lata with literatures review.

A schwannoma is a benign soft tissue tumor derived from Schwann cells of the nerve sheath. The lateral femoral cutaneous nerve is a sensory nerve originating from the second and third lumbar vertebra nerve roots, passing under the inguinal ligament and dividing into anterior and posterior branches. The anterior branch which is a major division and the main nerve that causes meralgia paresthetica supplies the knee joint from the anterior thigh. In addition, the posterior branch supplies the lateral thigh at the greater trochanter level.This paper reports the case of a 52-year-old woman with a very rare schwannoma in the terminal twig of posterior branch of the lateral femoral cutaneous nerve of right thigh protruded through the fascia lata with literatures review.

Background: Deceased donor liver transplantation (DDLT) and living donor liver transplantation (LDLT) are employed to address highly urgent patients, including those with acute liver failure (ALF), acute-on-chronic liver failure (ACLF), or critical cirrhosis. This study compares outcomes between LDLT and DDLT patients with ALF, ACLF, or critical cirrhosis in highly urgent LDLT (HU-LDLT) applications. Methods: This study conducted a retrospective analysis of the Korean Network for Organ Sharing (KONOS) data, which included 391 consecutive HU-LDLT applications from 2017 to 2021. Results: The proportion of DDLT was 15.1% (n=59) within the cohort of HU-LDLT applications. The prevalence of hepatorenal syndrome, duration of pre-transplant intensive care unit (ICU) care, incidence of pre-transplant continuous renal replacement therapy, and median model for end-stage liver disease scores were significantly greater and prolonged in DDLT patients compared to LDLT patients. Statistical analysis revealed no significant differences in postoperative complications or overall survival between the two groups. In the multivariate analysis, only pre-transplant ventilator care emerged as a significant predisposing factor for mortality. Conclusion The present study indicates that LDLT is a viable option, yielding comparable perioperative and long-term outcomes to DDLT for HU patients, which can encourage living liver donation to overcome organ shortages in HU patients.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

An arachnoid cyst is a rare cerebrospinal fluid–filled non-neoplastic cystic lesion. Most arachnoid cysts are asymptomatic; however, depending on their location and size, they may show various clinical symptoms and rarely can cause serious complications. We report a case of an arachnoid cyst incidentally found in a 25-year-old male soldier, which was not the cause of death. A 2.3×1.7 cm unilocular cystic lesion filled with colorless serous fluid was observed near the interpeduncular fossa of the brain. Microscopy revealed the cyst membrane to be lined by a single layer of meningothelial cells with no edema or inflammatory reaction in the surrounding structures. Considering the medical history, circumstances of death, and autopsy findings, the cause of death was determined to be sudden cardiac death, and the cyst found in the brain was consistent with an arachnoid cyst. Reviewing this case and related literature would help to determine the cause and manner of death if an arachnoid cyst is incidentally found during an autopsy.

Calcific aortic valve disease (CAVD) is an uncommon disease in young adults, unless there is a congenital anomaly in the aortic valve. We report a case of CAVD identified during the autopsy of a 26-year-old male soldier who died in his room while sitting on a chair. Macroscopic examination revealed multiple nodules in the semilunar cusps of the aortic valve, and that the commissure of semilunar valve cusps was slightly fused. Under microscopic examination, fibrotic nodules with neovascularization were identified in the fibrosa layer of the aortic valve, although neither calcification nor chronic inflammation were identified in the fibrotic nodule. Mitral valve prolapse and left ventricular hypertrophy with interstitial fibrosis were also identified. Microscopic examination of CAVD typically shows calcified nodules in the fibrosa layer of the valve, but fibrosis alone, without calcification, can be observed in the early stage of the disease. Death from CAVD is rare in young adults, so careful examination must be taken not to misdiagnose this condition with rheumatic valvular disease, which is relatively common in this population.