Dialysis-related amyloidosis (DRA) is a clinically significant complication of long-term hemodialysis, primarily affecting the bones and joints. Involvement of oral cavity is uncommon, and almost all reported cases are localized to the tongue. Here, we report a rare case of DRA presenting as a large tumor originating from the maxillary gingiva in a hemodialysis patient. We also review previously reported cases of oral DRA. A 77-year-old Japanese woman with diabetic kidney disease had been receiving maintenance hemodialysis for 11 years. She was referred to our hospital for evaluation of a large tumor extending from the maxillary gingiva to the palate. Although she had been completely edentulous in the maxilla for years, she did not wear dentures. A biopsy specimen from the tumor revealed diffuse subepithelial deposition of eosinophilic, amorphous material, which was positive for direct fast scarlet stain and exhibited apple-green birefringence under polarized light. Immunohistochemistry confirmed the presence of β2-microglobulin, leading to the diagnosis of DRA. The tumor was surgically resected, and no recurrence was noted during follow-up. Our case and literature review suggest that chronic mechanical stimulation in the oral cavity may contribute to the development of oral DRA. These findings highlight the importance of regular oral examinations and the elimination of mechanical irritation in patients receiving maintenance hemodialysis.
Background Postoperative regional nodal irradiation (RNI) is a standard treatment for breast cancer at high risk of regional recurrence; however, the necessity of including the internal mammary node (IMN) region in the radiation field remains unclear. This study aimed to evaluate treatment outcomes in a large cohort of patients who received postoperative radiotherapy with RNI excluding the IMN region. Methods This study included patients with breast cancer who underwent surgery followed by RNI without IMN irradiation between 2007 and 2018. The primary endpoint was disease-free survival (DFS), and the secondary endpoints were overall survival (OS), breast cancer-specific mortality (BCM), distant metastasis-free survival (DMFS), recurrence patterns, and treatment-related adverse events. Results In total, 799 patients were included. The 5-year DFS, OS, BCM, and DMFS rates were 75.9, 88.3, 9.7, and 77.1%, respectively. Worse outcomes were associated with a higher number of positive lymph nodes and estrogen receptor (ER)-negative disease. Medial/central tumor location and younger age were each significantly associated with poorer outcomes, being associated with worse DFS and DMFS. Bone was the most common recurrence site. ER-negative disease, a higher number of positive lymph nodes, medial/central location, and younger age were significant risk factors for recurrence, particularly distant metastasis. IMN recurrence was rare. Conclusions In this cohort, medial/central tumor location, ER-negative disease, and extensive nodal involvement were associated with poorer outcomes, suggesting that these factors may identify patients who can benefit from IMN irradiation. These findings may serve as important reference data when determining the indication for IMN irradiation on an individual patient basis.
Mucinous cystic neoplasm of the liver (MCN-L) is a rare cyst-forming epithelial neoplasm that typically lacks bile duct communication. Intraductal protrusion is uncommon and can mimic cystic intraductal papillary neoplasm of the bile duct (IPNB). A 39-year-old woman undergoing fertility treatment presented with recurrent hyperbilirubinemia and predominantly cholestatic liver test abnormalities. MRI demonstrated a 10.5-cm multilocular cystic lesion in segment 4 of the liver with downstream biliary dilatation, and internal septa were visible within the dilated bile duct on T2-weighted single-shot fast spin-echo (SSFSE) images. Contrast-enhanced computed tomography (CT) revealed enhanced cyst wall and septa, while endoscopic retrograde cholangiography revealed a smooth, oval-shaped filling defect. Endoscopic and intraductal ultrasonography indicated septate cystic components of the bile duct, and biopsy revealed mucin-producing columnar epithelium overlying the ovarian-like stroma, confirming MCN. The patient was treated via extended left hepatectomy with extrahepatic bile duct resection and reconstruction. Pathological examination revealed low-grade intraepithelial neoplasia. The patient remained recurrence-free for 20 months. This case suggests that, although biliary communication usually favors cystic IPNB over MCN-L, identification of intraductal extension of the cyst wall or septa within the dilated bile duct may provide an important clue to the diagnosis of MCN-L.
The Sequential Organ Failure Assessment (SOFA)-2 score was developed to better reflect contemporary critical care practice by incorporating modern organ support modalities and updated thresholds based on recent data. However, the generalizability of this framework to intensive care unit (ICU) populations beyond the development cohort, particularly across organ support subgroups and major disease categories, remains uncertain. We aimed to evaluate the external validity of SOFA-2 using the OneICU database, a large Japanese critical care database with comprehensive domain-level data. Adult ICU stays between February 2013 and August 2025 were included and classified into two cohorts: those with complete SOFA-1 and SOFA-2 component data on the day of ICU admission, and those with complete SOFA-2 data on that day. Discriminatory performance for ICU mortality was evaluated using the area under the receiver operating characteristic curve (AUROC) and compared between SOFA-1 and SOFA-2 using the DeLong test. Subgroup analyses were performed by major organ support device use and across disease categories. Among 152,883 eligible ICU stays, 67,116 had complete SOFA-1 and SOFA-2 data, and 121,443 had complete SOFA-2 data. SOFA-2 showed a slightly higher AUROC for ICU mortality than SOFA-1 (0.859 vs. 0.853; p < 0.001), although the absolute difference was small. Across subgroups defined by mechanical circulatory support use, SOFA-2 showed higher discrimination than SOFA-1. Discrimination was similar in other device-defined subgroups and in patients readmitted to the ICU. SOFA-2 also demonstrated good discrimination across major diagnostic groups. SOFA-2 showed similar discrimination for ICU mortality compared with SOFA-1 and maintained broadly comparable performance across clinically relevant subgroups, supporting its applicability for early severity assessment in heterogeneous ICU populations.
BACKGROUND:Gorham-Stout disease (GSD) is an extremely rare osteolytic disorder characterized by progressive bone resorption associated with nonneoplastic vascular or lymphatic proliferation. Craniovertebral junction involvement is particularly uncommon and may cause life-threatening instability or neurological compromise. To the authors' knowledge, bow hunter syndrome caused by craniovertebral GSD has not been reported. OBSERVATIONS:A 71-year-old woman presented with dizziness and inability to stand. Brain MRI and MR angiography demonstrated a left cerebellar infarction and left vertebral artery occlusion. CT and MRI showed extensive C1-3 osteolysis and cranial settling of the osteolytic C2 vertebral body. CT angiography in the neutral head position demonstrated vertebral artery recanalization, suggesting dynamic occlusion consistent with bow hunter syndrome. Additional osteolytic lesions in the L1 vertebral body, left seventh rib, and phalanges suggested GSD. After halo vest immobilization, occipitocervical (O-C7) fusion was performed using an autologous fibular strut graft, with angiographic confirmation of vertebral artery patency. Histopathological analysis confirmed GSD. No progression of the C1-3 osteolytic lesions was observed during 4 years of follow-up. LESSONS:Craniovertebral GSD can cause bow hunter syndrome and cerebellar infarction. Angiographic confirmation of vertebral artery patency, rigid fixation, and a fibular graft potentially resistant to osteolysis may be important for stabilization. https://thejns.org/doi/10.3171/CASE26576.
