1.Transformation of Pleomorphic Xanthoastrocytoma with Germline ATM Mutation into a SMARCB1-Deficient Rhabdoid Tumor: A Case Report
Hyeonseung LEE ; Hyun Jin PARK ; Bo Kyung KIM ; Kyung Taek HONG ; Hyoung Jin KANG ; Sung-Hye PARK ; Ji Hoon PHI ; June-Young KOH ; Jung Yoon CHOI
Clinical Pediatric Hematology-Oncology 2026;33(1):34-38
Secondary rhabdoid tumors (RTs) with atypical teratoid/rhabdoid tumor-like features rarely arise from, or coexist with, pleomorphic xanthoastrocytomas (PXAs), and their clinicopathological and molecular characteristics remain poorly understood. We report a 17-year-old girl with a temporal lobe mass that, upon gross total resection, pathologically contained both RT and PXA components. Immunohistochemistry revealed loss of INI1 expression restricted to the RT component, while the PXA area retained INI1. Next-generation sequencing identified a shared BRAF::TRIM24 fusion and homozygous deletion of CDKN2A/2B in both components, indicating a shared clonal origin. Additionally, a germline ATM frameshift mutation (c.5288_5289insGA) was identified in both tumor components, making the first such report in central nervous system tumors. SMARCB1 loss was confined to the RT component, further supporting the hypotheses of clonal evolution and secondary transformation. Despite gross total resection, craniospinal irradiation, and chemotherapy, the patient developed rapid leptomeningeal dissemination and died 5 months after surgery. This case provides clinicopathological and molecular evidence for clonal evolution and secondary transformation of PXA into an RT. The presence of germline ATM mutation may have therapeutic and biological relevance. Further studies are required to clarify the pathogenesis and optimal management of these rare and aggressive tumors.
2.Comparing haploidentical transplantation with post‑transplantation cyclophosphamide and umbilical cord blood transplantation using targeted busulfan in children and adolescents with hematologic malignancies
Kyung Taek HONG ; Bo Kyung KIM ; Hong Yul AN ; Jung Yoon CHOI ; Sang Hoon SONG ; Kyung‑Sang YU ; In‑Jin JANG ; Hyoung Jin KANG
Blood Research 2025;60():7-
Purpose:
This study compared the outcomes of haploidentical-related donor (HRD) and umbilical cord blood (UCB) hematopoietic stem cell transplantation (HSCT) in pediatric patients with hematologic malignancies.
Methods:
Data on patients who underwent HRD HSCT with post-transplant cyclophosphamide (n = 41) and UCB HSCT (n = 24) after targeted busulfan-based myeloablative conditioning with intensive pharmacokinetic monitoring between 2009 and 2018 were retrospectively analyzed.
Results:
The median follow-up durations in the HRD and UCB groups were 7.0 and 10.9 years, respectively. The cumu‑ lative incidence of acute graft-versus-host disease (GVHD) grades II–IV and moderate-to-severe chronic GVHD did not differ significantly between the groups. However, the HRD group demonstrated significantly lower rates of acute GVHD grades III–IV (4.9% vs. 29.2%, p = 0.009) and non-relapse mortality (2.6% vs. 34.2%, p < 0.001) but a higher relapse incidence (32.1% vs. 8.8%, p = 0.004) than the UCB group. The 5-year event-free and overall survival rates were 65.8% and 54.2% (p = 0.204) and 78.0% and 65.7% (p = 0.142) for the HRD and UCB groups, respectively. Multivariate analysis identified disease status as a significant risk factor for overall survival (hazard ratio, 3.24; p = 0.016). Additionally, UCB HSCT exhibited a trend toward worse event-free survival compared to HRD HSCT (hazard ratio, 2.63; p = 0.05).
Conclusions
These findings indicate that HRD HSCT with post-transplant cyclophosphamide provides promising outcomes compared to UCB HSCT in pediatric patients, with a trend toward improved survival over a long-term follow-up period exceeding a median of 7 years. Thus, HRD HSCT may be a valuable option for pediatric patients with‑ out human leukocyte antigen-matched donors.
3.Comparing haploidentical transplantation with post‑transplantation cyclophosphamide and umbilical cord blood transplantation using targeted busulfan in children and adolescents with hematologic malignancies
Kyung Taek HONG ; Bo Kyung KIM ; Hong Yul AN ; Jung Yoon CHOI ; Sang Hoon SONG ; Kyung‑Sang YU ; In‑Jin JANG ; Hyoung Jin KANG
Blood Research 2025;60():7-
Purpose:
This study compared the outcomes of haploidentical-related donor (HRD) and umbilical cord blood (UCB) hematopoietic stem cell transplantation (HSCT) in pediatric patients with hematologic malignancies.
Methods:
Data on patients who underwent HRD HSCT with post-transplant cyclophosphamide (n = 41) and UCB HSCT (n = 24) after targeted busulfan-based myeloablative conditioning with intensive pharmacokinetic monitoring between 2009 and 2018 were retrospectively analyzed.
Results:
The median follow-up durations in the HRD and UCB groups were 7.0 and 10.9 years, respectively. The cumu‑ lative incidence of acute graft-versus-host disease (GVHD) grades II–IV and moderate-to-severe chronic GVHD did not differ significantly between the groups. However, the HRD group demonstrated significantly lower rates of acute GVHD grades III–IV (4.9% vs. 29.2%, p = 0.009) and non-relapse mortality (2.6% vs. 34.2%, p < 0.001) but a higher relapse incidence (32.1% vs. 8.8%, p = 0.004) than the UCB group. The 5-year event-free and overall survival rates were 65.8% and 54.2% (p = 0.204) and 78.0% and 65.7% (p = 0.142) for the HRD and UCB groups, respectively. Multivariate analysis identified disease status as a significant risk factor for overall survival (hazard ratio, 3.24; p = 0.016). Additionally, UCB HSCT exhibited a trend toward worse event-free survival compared to HRD HSCT (hazard ratio, 2.63; p = 0.05).
Conclusions
These findings indicate that HRD HSCT with post-transplant cyclophosphamide provides promising outcomes compared to UCB HSCT in pediatric patients, with a trend toward improved survival over a long-term follow-up period exceeding a median of 7 years. Thus, HRD HSCT may be a valuable option for pediatric patients with‑ out human leukocyte antigen-matched donors.
4.Comparing haploidentical transplantation with post‑transplantation cyclophosphamide and umbilical cord blood transplantation using targeted busulfan in children and adolescents with hematologic malignancies
Kyung Taek HONG ; Bo Kyung KIM ; Hong Yul AN ; Jung Yoon CHOI ; Sang Hoon SONG ; Kyung‑Sang YU ; In‑Jin JANG ; Hyoung Jin KANG
Blood Research 2025;60():7-
Purpose:
This study compared the outcomes of haploidentical-related donor (HRD) and umbilical cord blood (UCB) hematopoietic stem cell transplantation (HSCT) in pediatric patients with hematologic malignancies.
Methods:
Data on patients who underwent HRD HSCT with post-transplant cyclophosphamide (n = 41) and UCB HSCT (n = 24) after targeted busulfan-based myeloablative conditioning with intensive pharmacokinetic monitoring between 2009 and 2018 were retrospectively analyzed.
Results:
The median follow-up durations in the HRD and UCB groups were 7.0 and 10.9 years, respectively. The cumu‑ lative incidence of acute graft-versus-host disease (GVHD) grades II–IV and moderate-to-severe chronic GVHD did not differ significantly between the groups. However, the HRD group demonstrated significantly lower rates of acute GVHD grades III–IV (4.9% vs. 29.2%, p = 0.009) and non-relapse mortality (2.6% vs. 34.2%, p < 0.001) but a higher relapse incidence (32.1% vs. 8.8%, p = 0.004) than the UCB group. The 5-year event-free and overall survival rates were 65.8% and 54.2% (p = 0.204) and 78.0% and 65.7% (p = 0.142) for the HRD and UCB groups, respectively. Multivariate analysis identified disease status as a significant risk factor for overall survival (hazard ratio, 3.24; p = 0.016). Additionally, UCB HSCT exhibited a trend toward worse event-free survival compared to HRD HSCT (hazard ratio, 2.63; p = 0.05).
Conclusions
These findings indicate that HRD HSCT with post-transplant cyclophosphamide provides promising outcomes compared to UCB HSCT in pediatric patients, with a trend toward improved survival over a long-term follow-up period exceeding a median of 7 years. Thus, HRD HSCT may be a valuable option for pediatric patients with‑ out human leukocyte antigen-matched donors.
5.Domiciliary dental care for the older adults across countries:a scoping review
Sunwoo PARK ; Sol HAN ; Bo-Hyoung JIN
Journal of Korean Academy of Oral Health 2025;49(4):212-224
Objectives:
With the rapid aging of the global population, maintaining oral health in older adults with frailty has become a major public health priority. Domiciliary dental care, which provides dental services directly at home or in long-term care facilities, has emerged as a promising approach for overcoming barriers to mobility, access, and equity. However, evidence of its structure, effectiveness, and sustainability remains fragmented across countries. This scoping review aimed to map and synthesize international models of domiciliary dental care, focusing on their organizational structures, delivery processes, reported outcomes, limitations, and policy implications. We also sought to identify gaps in evidence and propose future research directions.
Methods:
Following the PRISMA-ScR framework, we conducted a structured review of the literature on domiciliary dental care systems in Japan, Hong Kong, Taiwan, Germany, the United Kingdom, Canada, and Australia. The data were charted on program design; service coverage; financing;workforce organization; and outcomes related to oral health, patient experience, and systemic challenges.
Results:
Domiciliary dental care improved access for frail or homebound older adults, facilitated early detection and prevention, enhanced oral health maintenance, and increased patient and caregiver satisfaction across countries. Education components for caregivers and facility staff further contributed to sustained oral hygiene practices. Nevertheless, most services were limited to preventive and basic restorative care, constrained by a lack of portable equipment, inadequate workforce capacity, low reimbursement levels, administrative burden, and legal liability concerns. Evidence from low- and middle-income countries was extremely limited, and few longitudinal and experimental studies were available to assess the cost-effectiveness or long-term outcomes.
Conclusions
This scoping review highlights domiciliary dental care as a valuable but underdeveloped public health strategy for aging societies. To achieve sustainability and a broader impact, future efforts must address streamlined administration, standardized guidelines, adequate financing, professional training, and legal safeguards supported by stronger evidence, including economic evaluations and long-term outcome studies.
6.Study on the plaque removal efficacy of single-tufted toothbrush:scoping review
Youngjin LEE ; Bhumgey David LEE ; Bo-Hyoung JIN
Journal of Korean Academy of Oral Health 2024;48(4):216-222
Objectives:
This study aims to evaluate the efficacy of single-tuft toothbrushes in removing dental plaque, particularly in areas that are difficult to reach using conventional toothbrushes. The study also explores the current level of awareness and usage of these brushes, and it identifies gaps in research and education regarding their application.
Methods:
A scoping review was conducted using the framework proposed by Arksey and O’Malley and guidelines by Peters et al. Databases including PubMed, Scopus, and Korean academic repositories were searched using keywords like “single-tuft toothbrush” and “end-tuft toothbrush.” A total of 403 articles were initially identified, with 9 studies meeting the inclusion criteria. These studies included 8 randomized controlled trials and 1 in-vitro experiment, focusing on the effectiveness of single-tuft toothbrushes in various contexts.
Results:
Single-tuft toothbrushes demonstrated superior plaque removal in challenging areas, such as interproximal surfaces of molars, lingual surfaces, and around orthodontic appliances. In combination with conventional toothbrushes, they showed significantly better results compared to conventional tools alone. However, they did not consistently outperform other interdental cleaning devices like floss or interdental brushes across all metrics. User satisfaction with single-tuft toothbrushes was high, especially among orthodontic patients, although their usage rate was significantly lower due to limited awareness and accessibility.
Conclusions
Single-tuft toothbrushes are highly effective as adjuncts to conventional oral hygiene tools, particularly in managing hard-to-reach areas. However, their overall efficacy in replacing other oral hygiene tools is limited. To maximize their potential, increased public awareness, improved accessibility, and targeted education programs are needed. Further research should explore their role in specific patient populations, such as those with orthodontic appliances or implant restorations.
8.Study on the plaque removal efficacy of single-tufted toothbrush:scoping review
Youngjin LEE ; Bhumgey David LEE ; Bo-Hyoung JIN
Journal of Korean Academy of Oral Health 2024;48(4):216-222
Objectives:
This study aims to evaluate the efficacy of single-tuft toothbrushes in removing dental plaque, particularly in areas that are difficult to reach using conventional toothbrushes. The study also explores the current level of awareness and usage of these brushes, and it identifies gaps in research and education regarding their application.
Methods:
A scoping review was conducted using the framework proposed by Arksey and O’Malley and guidelines by Peters et al. Databases including PubMed, Scopus, and Korean academic repositories were searched using keywords like “single-tuft toothbrush” and “end-tuft toothbrush.” A total of 403 articles were initially identified, with 9 studies meeting the inclusion criteria. These studies included 8 randomized controlled trials and 1 in-vitro experiment, focusing on the effectiveness of single-tuft toothbrushes in various contexts.
Results:
Single-tuft toothbrushes demonstrated superior plaque removal in challenging areas, such as interproximal surfaces of molars, lingual surfaces, and around orthodontic appliances. In combination with conventional toothbrushes, they showed significantly better results compared to conventional tools alone. However, they did not consistently outperform other interdental cleaning devices like floss or interdental brushes across all metrics. User satisfaction with single-tuft toothbrushes was high, especially among orthodontic patients, although their usage rate was significantly lower due to limited awareness and accessibility.
Conclusions
Single-tuft toothbrushes are highly effective as adjuncts to conventional oral hygiene tools, particularly in managing hard-to-reach areas. However, their overall efficacy in replacing other oral hygiene tools is limited. To maximize their potential, increased public awareness, improved accessibility, and targeted education programs are needed. Further research should explore their role in specific patient populations, such as those with orthodontic appliances or implant restorations.
9.Study on the plaque removal efficacy of single-tufted toothbrush:scoping review
Youngjin LEE ; Bhumgey David LEE ; Bo-Hyoung JIN
Journal of Korean Academy of Oral Health 2024;48(4):216-222
Objectives:
This study aims to evaluate the efficacy of single-tuft toothbrushes in removing dental plaque, particularly in areas that are difficult to reach using conventional toothbrushes. The study also explores the current level of awareness and usage of these brushes, and it identifies gaps in research and education regarding their application.
Methods:
A scoping review was conducted using the framework proposed by Arksey and O’Malley and guidelines by Peters et al. Databases including PubMed, Scopus, and Korean academic repositories were searched using keywords like “single-tuft toothbrush” and “end-tuft toothbrush.” A total of 403 articles were initially identified, with 9 studies meeting the inclusion criteria. These studies included 8 randomized controlled trials and 1 in-vitro experiment, focusing on the effectiveness of single-tuft toothbrushes in various contexts.
Results:
Single-tuft toothbrushes demonstrated superior plaque removal in challenging areas, such as interproximal surfaces of molars, lingual surfaces, and around orthodontic appliances. In combination with conventional toothbrushes, they showed significantly better results compared to conventional tools alone. However, they did not consistently outperform other interdental cleaning devices like floss or interdental brushes across all metrics. User satisfaction with single-tuft toothbrushes was high, especially among orthodontic patients, although their usage rate was significantly lower due to limited awareness and accessibility.
Conclusions
Single-tuft toothbrushes are highly effective as adjuncts to conventional oral hygiene tools, particularly in managing hard-to-reach areas. However, their overall efficacy in replacing other oral hygiene tools is limited. To maximize their potential, increased public awareness, improved accessibility, and targeted education programs are needed. Further research should explore their role in specific patient populations, such as those with orthodontic appliances or implant restorations.
10.Study on the plaque removal efficacy of single-tufted toothbrush:scoping review
Youngjin LEE ; Bhumgey David LEE ; Bo-Hyoung JIN
Journal of Korean Academy of Oral Health 2024;48(4):216-222
Objectives:
This study aims to evaluate the efficacy of single-tuft toothbrushes in removing dental plaque, particularly in areas that are difficult to reach using conventional toothbrushes. The study also explores the current level of awareness and usage of these brushes, and it identifies gaps in research and education regarding their application.
Methods:
A scoping review was conducted using the framework proposed by Arksey and O’Malley and guidelines by Peters et al. Databases including PubMed, Scopus, and Korean academic repositories were searched using keywords like “single-tuft toothbrush” and “end-tuft toothbrush.” A total of 403 articles were initially identified, with 9 studies meeting the inclusion criteria. These studies included 8 randomized controlled trials and 1 in-vitro experiment, focusing on the effectiveness of single-tuft toothbrushes in various contexts.
Results:
Single-tuft toothbrushes demonstrated superior plaque removal in challenging areas, such as interproximal surfaces of molars, lingual surfaces, and around orthodontic appliances. In combination with conventional toothbrushes, they showed significantly better results compared to conventional tools alone. However, they did not consistently outperform other interdental cleaning devices like floss or interdental brushes across all metrics. User satisfaction with single-tuft toothbrushes was high, especially among orthodontic patients, although their usage rate was significantly lower due to limited awareness and accessibility.
Conclusions
Single-tuft toothbrushes are highly effective as adjuncts to conventional oral hygiene tools, particularly in managing hard-to-reach areas. However, their overall efficacy in replacing other oral hygiene tools is limited. To maximize their potential, increased public awareness, improved accessibility, and targeted education programs are needed. Further research should explore their role in specific patient populations, such as those with orthodontic appliances or implant restorations.

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