Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

Mechanical thrombectomy using a stent retriever is a widely-used technique for recanalizing occluded cerebral arteries in acute ischemic stroke. Although rare, inadvertent stent detachment has primarily been reported with earlier stent retriever designs. We present 2 cases of inadvertent stent detachment with the pRESET stent. In the first case, an elderly patient presented with right middle cerebral artery occlusion and experienced stent detachment during the second retrieval. Despite this, successful recanalization was achieved through balloon angioplasty. In the second case, an elderly patient experienced stent detachment after the third retrieval, and recanalization of the M1 segment was unsuccessful due to the inability to pass the microcatheter. The first patient had a modified Rankin scale (mRS) score of 3, while the second had a poor prognosis with an mRS score of 5. These cases highlight that inadvertent stent detachment can impact outcomes, and balloon angioplasty may serve as a useful salvage therapy.

Objective: To develop a deep-learning-based bone age prediction model optimized for Korean children and adolescents and evaluate its feasibility by comparing it with a Greulich-Pyle-based deep-learning model. Materials and Methods: A convolutional neural network was trained to predict age according to the bone development shown on a hand radiograph (bone age) using 21036 hand radiographs of Korean children and adolescents without known bone development-affecting diseases/conditions obtained between 1998 and 2019 (median age [interquartile range {IQR}], 9 [7–12] years; male:female, 11794:9242) and their chronological ages as labels (Korean model). We constructed 2 separate external datasets consisting of Korean children and adolescents with healthy bone development (Institution 1: n = 343;median age [IQR], 10 [4–15] years; male: female, 183:160; Institution 2: n = 321; median age [IQR], 9 [5–14] years; male:female, 164:157) to test the model performance. The mean absolute error (MAE), root mean square error (RMSE), and proportions of bone age predictions within 6, 12, 18, and 24 months of the reference age (chronological age) were compared between the Korean model and a commercial model (VUNO Med-BoneAge version 1.1; VUNO) trained with Greulich-Pyle-based age as the label (GP-based model). Results: Compared with the GP-based model, the Korean model showed a lower RMSE (11.2 vs. 13.8 months; P = 0.004) and MAE (8.2 vs. 10.5 months; P = 0.002), a higher proportion of bone age predictions within 18 months of chronological age (88.3% vs. 82.2%; P = 0.031) for Institution 1, and a lower MAE (9.5 vs. 11.0 months; P = 0.022) and higher proportion of bone age predictions within 6 months (44.5% vs. 36.4%; P = 0.044) for Institution 2. Conclusion The Korean model trained using the chronological ages of Korean children and adolescents without known bone development-affecting diseases/conditions as labels performed better in bone age assessment than the GP-based model in the Korean pediatric population. Further validation is required to confirm its accuracy.

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder characterized by heterogenous lesions infiltrated with CD1a+/CD207+ cells. Although LCH has a relatively good prognosis, the prognosis for patients with LCH refractory to standard chemotherapy is poor. Neurodegenerative LCH (ND-LCH) is a central nervous system complication of LCH that is characterized by progressive radiological and clinical abnormalities. Symptomatic ND-LCH is difficult to treat and therefore has a poor prognosis. A two-year-old boy presented with a scalp mass. Biopsy confirmed LCH. Whole-body imaging revealed LCH involvement in multiple bones of the skull, facial bones, and lungs. Prednisolone and vinblastine chemotherapy was initiated. One-year post-treatment, most of the lesions in the bones and lung nodules disappeared, and chemotherapy was discontinued. New neurodegenerative lesions appeared 4 months after chemotherapy was discontinued. Second-line chemotherapy using cytarabine, vincristine, and prednisolone was initiated. However, neurological manifestations of ND-LCH worsened post second-line treatment, and the treatment was switched to cytarabine and cladribine. Despite third-line chemotherapy, the lesions progressed, and neurological deficits worsened. After identifying BRAF V600E mutation in the tumor tissue using next-generation sequencing, cytotoxic chemotherapy was discontinued and vemurafenib treatment was initiated. One-year post-vemurafenib therapy, ND-LCH manifestations regressed, and the patient experienced neurological improvement.

Background: Mechanical thrombectomy (MT) of ischemic stroke was recommended as a clinical guideline in 2015, and the indication for time was expanded in 2018 based on two clinical studies. We aimed to compare and analyze the prognosis of patients treated under the extended time indication before and after the introduction of advanced software. Methods: We obtained data from medical records between 2016 to 2020. From 2016 to 2017, patients who did not receive MT who visited the hospital within 24 hours from the last normal time (LNT) were classified as standard medical treatment (SMT) group. Among patients who underwent MT between 2019 and 2020, patients who visited the hospital between 6-24 hours from the LNT were classified into the extended MT (EMT) group. Good outcome was defined as 3-months modified rankin scale (mRS) ≤ 2, and a poor outcome as mRS ≥ 4. Results: From 2016 to 2017, 1,058 patients were hospitalized for ischemic stroke, of which 60 (5.7%) received MT, and 27 patients were classified into the SMT group. Among 1,019 patients between 2019 and 2020, 85 (8.3%) received MT, and 24 patients were in the EMT group. Among the SMT group, only 3 had a good prognosis, and 24 (88.9%) had a poor prognosis. However, in the EMT group, 10 (41.7%) had a good prognosis, and 9 (37.5%) had a poor prognosis. The SMT group had a 49.1 times higher risk of poor prognosis compared to the EMT group (P = 0.008). Conclusion The number of patients with ischemic stroke who receive MT has increased by using advanced imaging software. It was confirmed that patients treated based on the extended time indication also had a good prognosis.

Purpose: Additional surgery is recommended for patients after a non-curative endoscopic submucosal dissection (ESD) to prevent residual cancer (RC) or lymph node metastasis (LNM). We aimed to evaluate the clinicopathologic characteristics of patients who underwent an additional gastrectomy after a non-curative ESD procedure and identify the risk factors of RC and LNM. Methods: We retrospectively assessed the clinicopathological factors of 73 patients who underwent additional gastrectomy following a non-curative ESD between January 2009 and December 2019 at our center. Results: RC and LNM rates after additional gastrectomy were 9.6% and 8.2%, respectively. Invasion deeper than 500 μm (P = 0.045), positive horizontal resection margin (P < 0.001), and positive ESD margin (P = 0.001) were identified as statistically significant factors in univariate analysis for RC, but not in multivariate analysis. Lymphatic invasion was the only risk factor found to be significant in both univariate and multivariate analyses (P = 0.005 and P = 0.012). Conclusion Additional gastrectomy is necessary to prevent RC or LNM after non-curative ESD. Lymphatic invasion was also associated with LNM in patients who underwent an additional gastrectomy after a non-curative ESD, and in such cases, active treatment is required.

PURPOSE: Recently, a nomogram predicting overall survival after gastric resection was developed and externally validated in Korea and Japan. However, this gastric cancer nomogram is derived from large-volume centers, and the applicability of the nomogram in smaller centers must be proven. The purpose of this study is to externally validate the gastric cancer nomogram using a dataset from a medium-volume center in Korea. MATERIALS AND METHODS: We retrospectively analyzed 610 patients who underwent radical gastrectomy for gastric cancer from August 1, 2005 to December 31, 2011. Age, sex, number of metastatic lymph nodes (LNs), number of examined LNs, depth of invasion, and location of the tumor were investigated as variables for validation of the nomogram. Both discrimination and calibration of the nomogram were evaluated. RESULTS: The discrimination was evaluated using Harrell's C-index. The Harrell's C-index was 0.83 and the discrimination of the gastric cancer nomogram was appropriate. Regarding calibration, the 95% confidence interval of predicted survival appeared to be on the ideal reference line except in the poorest survival group. However, we observed a tendency for actual survival to be constantly higher than predicted survival in this cohort. CONCLUSIONS: Although the discrimination power was good, actual survival was slightly higher than that predicted by the nomogram. This phenomenon might be explained by elongated life span in the recent patient cohort due to advances in adjuvant chemotherapy and improved nutritional status. Future gastric cancer nomograms should consider elongated life span with the passage of time.
Calibration ; Chemotherapy, Adjuvant ; Cohort Studies ; Dataset* ; Discrimination (Psychology) ; Gastrectomy ; Humans ; Japan ; Korea ; Lymph Nodes ; Nomograms* ; Nutritional Status ; Retrospective Studies ; Stomach Neoplasms*