The Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) is widely used for risk stratification in pheochromocytoma and paraganglioma (PPGL), but its clinical utility is limited by inter-observer variability of its parameters and inconsistent predictive performance. Methods: We conducted a multicenter retrospective study of 1,518 patients with PPGL from five tertiary referral centers in Korea. Prognostic utility of PASS system was assessed using logistic regression, Kaplan-Meier analysis, and receiver operating characteristic (ROC) curve analysis. Inter-observer variability was inferred by comparing area under the ROC curve (AUCs) across institutions. Simplified PASS systems were developed based on multivariable analysis of key histopathological parameters. Results: The PASS system was a significant predictor of adverse events and recurrence-free survival. Although the PASS system demonstrated only modest discriminative ability (AUC, 0.673), it showed a high negative predictive value (NPV, 0.885), supporting its usefulness as a screening tool for benign behavior. However, there was significant inter-institutional variability in PASS performance (AUC; range, 0.513 to 0.727; p < .05). The 3-factor Simple PASS, which incorporates necrosis, spindling, and mitotic figures, exhibited less inter-observer variation. The 4-factor Simple PASS, which adds vascular invasion to the 3-factor model, also showed reduced inter-observer variability and improved AUC and NPV compared to the original PASS system. Conclusions: In this multicenter cohort, the PASS system demonstrated high NPV and screening potential, but significant inter-observer variability remains a challenge. Simplification of the PASS system and enhanced pathologist training may improve reproducibility and clinical utility in PPGL risk stratification.

Midline palatal fractures compromise maxillary transverse stability and cause oro-nasal communication. We report a successful minimally invasive reconstruction based on AO CMF principles. A 60-year-old male with Le Fort II and midline palatal fractures following a fall exhibited nasal regurgitation. Associated facial fractures were managed conservatively, while the palatal fracture was addressed with a customized acrylic splint fabricated via model-based virtual surgery to restore pre-traumatic occlusion.Intraoperatively, segments were repositioned through manual reduction, followed by intermolar wire ligation and splint fixation. Nasal regurgitation resolved by postoperative day 4. Evaluation at 10 days confirmed anatomical arch width restoration. Stable occlusion was maintained throughout the 6-week splinting period. Closed reduction using customized splints and interdental ligation is an effective, minimally invasive alternative for managing midline palatal fractures, particularly when open reduction is challenging.

Replacing the retrohepatic inferior vena cava (IVC) following concurrent resection of the IVC and tumor-involved liver is an emerging surgical technique in living donor liver transplantation (LDLT), particularly for managing hepatocellular carcinomas near the IVC. This approach makes the extent of hepatectomy in LDLT comparable to that of deceased donor liver transplantation. We report a pediatric LDLT case involving IVC and portal vein (PV) replacement with an iliac vein homograft to treat advanced hepatoblastomas. The patient was a 24-month-old girl weighing 9.1 kg, presenting with large, multiple hepatoblastomas infiltrating the retrohepatic IVC and PV. After a 2-week waiting period, a cold-stored iliac vein graft was obtained, and the transplantation was performed using a left lateral section graft from her father.A 4-cm iliac vein homograft was anastomosed between the suprahepatic and suprarenal IVC stumps. The liver graft was implanted following standard procedures, with the PV reconstructed via a telescope-shaped anastomosis using the vein graft interposition. The patient experienced an uncomplicated recovery and is currently receiving scheduled adjuvant chemotherapy. In conclusion, IVC and PV replacement with vein homografts represents a feasible and effective surgical strategy for pediatric LDLT patients with advanced liver malignancies involving vascular invasion.

Purpose: This study aimed to report the real-world outcomes of intravesical gemcitabine for bacillus Calmette–Guérin (BCG)–unresponsive, high-risk, non–muscle-invasive bladder cancer (HR-NMIBC) in Korean patients who were unable or unwilling to undergo radical cystectomy (RC). Materials and Methods: This retrospective study included 131 patients (median age, 69 years; 88.5% men) treated with intravesical gemcitabine for BCG-unresponsive HR-NMIBC at nine centers between May 2019 and April 2022. The primary endpoint was 1-year recurrence-free survival (RFS). The secondary endpoints included factors influencing RFS, progression-free survival (PFS), cystectomy- free survival, cancer-specific survival (CSS), overall survival (OS), and safety. Survival analysis was performed using the Kaplan-Meier method, and risk factors for recurrence were assessed using Cox regression models. Results: Patients were followed up for a median duration of 25 months, with carcinoma in situ (CIS) in 41.9% of the patients. The 1-year and 2-year RFS rates were 68% and 42%, while the 1-year and 2-year PFS rates were 87% and 77%, respectively. No significant factors influencing RFS were identified. Seventeen patients underwent RC during a median follow-up of 16 months, with the condition in three patients progressing to muscle-invasive disease on final pathological analysis. The 2-year CSS and OS rates were 98% and 97%, respectively. Intravesical gemcitabine was well-tolerated, with only seven patients (5.3%) unable to complete the full induction course. Conclusion Our research highlights the potential of intravesical gemcitabine as a viable bladder-sparing treatment option for BCG-unresponsive HR-NMIBC, providing real-world evidence on its safety, efficacy, and tolerability.

Purpose: The aim of this study is to compare the detection ability of quality of life (QoL) changes and responsiveness of the KOrean QUality of life in Stomach cancer patients Study group (KOQUSS)-40 and European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ). Materials and Methods: A multicenter prospective observational study was conducted to evaluate QoL changes after various gastrectomies between January 2021 and April 2022. Participants were instructed to complete the KOQUSS-40 and EORTC QLQ-C30/STO22 preoperatively and at 1, 3, 6, and 12 months postoperatively. QoL changes over time and QoL responsiveness were assessed for each questionnaire. Results: Data from 491 patients who underwent curative gastrectomy for gastric cancer at 22 institutions were analyzed. The summary scores of the KOQUSS-40 and EORTC QLQ-STO22 showed significant differences between the total and proximal gastrectomy groups (p=0.044 and p=0.038, respectively), but no difference was observed for the EORTC QLQ-C30. Dysphagia on the KOQUSS-40 was significantly different between the total and proximal gastrectomy groups (p=0.031); however, dysphagia on the EORTC QLQ-STO22 did not differ. The responsiveness of the KOQUSS-40 was similar to that of the EORTC QLQ in patients who experienced ≥ 10% body weight loss, but approximately 10% less in patients receiving adjuvant chemotherapy than the EORTC QLQ. Conclusion KOQUSS-40 has several advantages over EORTC QLQ-C30/STO22 when comparing QoL between the total and proximal gastrectomy groups. The findings provide information for researchers investigating the QoL of patients who have undergone curative gastrectomy for gastric cancer.

Background: This study was performed to evaluate the prognostic utility of a positive T wave in lead augmented vector right (TaVR) and elevated E/e′ ratio in predicting major adverse cardiovascular events (MACE) in patients receiving maintenance hemodialysis. Methods: We retrospectively examined 296 adults on thrice-weekly hemodialysis with baseline electrocardiography and transthoracic echocardiography (October 2018–April 2024). TaVR positivity was T-wave amplitude, >0 mV and high E/e′, ≥19. Primary outcome was the first MACE—cardiovascular death, myocardial infarction, stroke, heart-failure admission, or revascularization. Multivariable Cox models adjusted for clinical covariates; incremental value was gauged with Harrell’s C-index, integrated discrimination improvement (IDI), and continuous net reclassification improvement (NRI). Sensitivity analysis was performed using a guideline-recommended E/e′ threshold of ≥15 to assess robustness. Results: Over 56.5 months (1,325 patient-years), 118 MACE occurred (8.9/100 patient-years). Incidence was higher with TaVR positivity than negativity (16.0/100 patient-years vs. 3.7/100 patient-years; log-rank p < 0.001). Adjusted hazard ratios were 3.19 (95% confidence interval [CI], 2.00–5.08) for TaVR and 2.92 (95% CI, 1.71–4.96) for high E/e′. Adding both markers to the clinical model increased the C-index from 0.65 to 0.75 (Δ 0.10) and improved IDI (0.10) and NRI (0.16) (all p < 0.01). A significant negative interaction (hazard ratio, 0.21; p = 0.01) indicated complementary but partly overlapping information. Sensitivity results were similar. Conclusion TaVR positivity is a strong independent electrocardiography predictor of cardiovascular events in hemodialysis. Combining TaVR with E/e′ adds prognostic value and supports a pragmatic two-step strategy— electrocardiography triage followed by focused echocardiography—for cardiovascular risk stratification in this high-risk population.

Background: Deep neuromuscular blockade (NMB) optimizes surgical conditions, particularly during laparoscopic procedures. However, its effects on systemic cytokines associated with anesthesia-related complications, including postoperative delirium and cognitive dysfunction, remain unclear. In this review, we quantified the impact of deep NMB on serum cytokine levels. Methods: PubMed, EMBASE, CENTRAL, CINAHL, Scopus, Web of Science, and Google Scholar databases were searched to identify randomized controlled trials (RCTs) evaluating serum cytokine levels in surgical patients under deep or moderate NMB. Results: Eight RCTs, including 661 patients undergoing laparoscopic and orthopedic surgeries, met the inclusion criteria. Immediately postoperatively, meta-analysis suggested a potential reduction in tumor necrosis factor-α (TNF-α, standardized mean difference: −0.46, 95% CI [−0.87 to −0.06], P = 0.03), with no significant differences in interleukin-1β (IL-1β) or interleukin-6 (IL-6) levels. At 24-h and 48-h postoperatively, no significant differences were observed in IL-1β, IL-6, TNF-α, or C-reactive protein levels. Meta-regression analysis indicated that inhalational anesthesia was associated with high IL-1β (estimate = 1.2135, 95% CI [0.5107–1.9162], P < 0.01) and TNF-α levels (estimate = 0.6271, 95% CI [0.0544–1.1997], P = 0.032) immediately postoperatively; however, younger patients exhibited elevated IL-1β levels under moderate NMB at 24-h postoperatively (estimate = 0.0242, 95% CI [0.0065–0.0419], P < 0.01). Conclusions Deep NMB may be associated with reduced TNF-α levels immediately postoperatively. Inhalational anesthesia and younger age may contribute more to higher serum cytokine levels compared with total intravenous anesthesia and older age, respectively, suggesting a potential immunomodulatory effect of deep NMB. Further studies should clarify its clinical relevance.

Background: and Purpose To compare the epidemiological and clinical features of the rare patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) with age- and sex-matched controls in a nationwide cohort from South Korea. Methods: This observational cohort study analyzed newly diagnosed CADASIL patients aged at least 20 years and matched controls using data from the National Health Information Database for 2004–2022. The cumulative incidence of CADASIL was assessed by age and sex, and compared between regions. Neurologic and systemic diseases were compared between the CADASIL and control groups. Results: The study analyzed 816 CADASIL patients and 816 age- and sex-matched controls aged 56.8±15.2 years (mean±standard deviation), among whom 48.3% were male. The cumulative incidence of CADASIL was 1.86 per 100,000 people (95% confidence interval [CI]=1.85– 1.87 per 100,000), and peaked at 60–69 years of age. In terms of regional distribution, the incidence was highest for Jeju, at 39.67 per 100,000 (95% CI 37.84–41.49 per 100,000). Neurologic diseases were more frequent in CADASIL patients, including Alzheimer’s disease (33.1% vs.20.0%), vascular dementia (84.9% vs. 5.0%), epilepsy (34.6% vs. 15.9%), stroke (70.7% vs. 27.6%), parkinsonism (18.9% vs. 11.0%), and depression (60.8% vs. 44.9%). Systemic diseases such as diabetes mellitus (78.9% vs. 68.9%) were also more common in CADASIL patients, while cancer (27.9% vs. 38.7%) and myocardial infarction (10.0% vs. 13.6%) were less common than in controls. The onset ages of all diseases were lower in CADASIL patients. Conclusions This study has provided a precise nationwide estimate of the CADASIL incidence and its regional distribution in South Korea. CADASIL patients showed higher incidence rates and earlier onsets of diverse clinical manifestations.

In children with biliary atresia, recurrent episodes of cholangitis often lead to portal vein (PV) phlebosclerosis. We report a case of pediatric living donor liver transplantation (LDLT) for biliary atresia with PV occlusion and a large splenorenal shunt. A 3-year-5-month-old girl was diagnosed with syndromic biliary atresia and polysplenia. Because the patient’s condition progressively deteriorated with worsening jaundice, we opted to perform LDLT using a left lateral section graft from her father. The recipient’s native PV was severely atrophic and completely occluded, and the collateral veins around the hepatoduodenal ligament were too small to serve as a viable source of portal inflow.Consequently, renoportal anastomosis (RPA) was selected as an alternative approach.Iliac vein interposition was performed to provide a new portal inflow source, and anatomy-compliant PV reconstruction was performed. The patient recovered from transplant surgery, but PV conduit stenosis occurred with the development of ascites 2 months posttransplant. This RPA-related vascular complication was resolved through percutaneous balloon angioplasty of the PV conduit. The patient has been doing well for 6 months posttransplantation. This case demonstrates that RPA can represent a viable reconstructive option for PV inflow in liver transplantation for pediatric patients with phlebosclerotic PV and prominent splenorenal shunt.

In patients with differentiated thyroid cancer (DTC), initial recurrence risk stratification based on clinical, histopathological, and perioperative data remains the key determinant for guiding management strategies during the first 1-2 years post-treatment. However, the adoption of ongoing risk stratification (ORS), which dynamically reassesses risk by integrating longitudinal clinical data and treatment response, enables more precise long-term prognostic assessment and facilitates highly individualized management. Building upon recent guidelines, the 2026 KTA guideline has been further refined by incorporating robust evidence from large-scale national cohorts and comprehensive systematic reviews. These updated recommendations outline contemporary concepts of ORS, risk-adapted TSH suppression targets, optimized surveillance modalities for recurrence detection, and disease-specific long-term follow-up strategies. Reflecting the paradigm shift toward de-escalated treatment, this revision integrates evolved perspectives on TSH suppression intensity, the clinical interpretation of thyroglobulin levels, and tailored follow-up intervals. These evidence-based recommendations aim to minimize unnecessary treatment and excessive surveillance in the large proportion of patients with excellent prognosis after initial therapy, while ensuring that each patient receives appropriately tailored and effective long-term management.