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Extra-Ocular Vascular and Cardiorespiratory Events During Intra-Arterial Chemotherapy in Children With Retinoblastoma: Incidence, Predictive Risk Factors, Management, and Impact on Further Treatment. 视网膜母细胞瘤儿童动脉化疗期间的眼外血管和心肺事件:发病率、预测危险因素、管理和对进一步治疗的影响
IF 2.4 3区 医学
Pediatric Blood & Cancer Pub Date : 2025-04-16 DOI: 10.1002/pbc.31724
Bastien Marti, Aziz Chaouch, Francesco Puccinelli, Mattia Rizzi, E Lemmel, Philippe Maeder, Mirko Dolci, Christina Stathopoulos, Francis Munier, Maja Beck-Popovic
{"title":"Extra-Ocular Vascular and Cardiorespiratory Events During Intra-Arterial Chemotherapy in Children With Retinoblastoma: Incidence, Predictive Risk Factors, Management, and Impact on Further Treatment.","authors":"Bastien Marti, Aziz Chaouch, Francesco Puccinelli, Mattia Rizzi, E Lemmel, Philippe Maeder, Mirko Dolci, Christina Stathopoulos, Francis Munier, Maja Beck-Popovic","doi":"10.1002/pbc.31724","DOIUrl":"https://doi.org/10.1002/pbc.31724","url":null,"abstract":"<p><strong>Background: </strong>The purpose of this study was to report on the incidence of extra-ocular vascular and cardiorespiratory adverse events in patients treated with intra-arterial chemotherapy (IAC) for retinoblastoma and identify risk factors for their occurrence.</p><p><strong>Procedure: </strong>This is a single-center retrospective study including 195 patients and 578 IACs performed at the Centre Hospitalier Universitaire Vaudois (CHUV) between 2008 and 2018. All patient's medical records of the study cohort were reviewed and securely stored in the REDCap database. Generalized estimating equations were used to identify risk factors for the occurrence of adverse events.</p><p><strong>Results: </strong>Extra-ocular vascular events in IAC patients were observed in 14% of patients, were transient, and occured mainly during the procedure. Potential risk factors were younger age, increased procedure time, ophthalmic artery (OA) access via the external carotid artery (ECA), as well as bridge IAC. Cardiorespiratory events were observed in 25% of patients, and were all transient and manageable without consequences. Younger age was the only associated risk factor.</p><p><strong>Conclusions: </strong>Extra-ocular and cardiorespiratory events during IAC are frequent, but are manageable by a trained team of pediatric interventional neuro-radiologists and anesthesiologists at a tertiary center, aware of these specific treatment-related risks. In addition, catheterization via the ECA should be avoided, when possible. Further studies are needed to ascertain the increased risk of extra-ocular vascular events via the ECA route.</p>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31724"},"PeriodicalIF":2.4,"publicationDate":"2025-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144025495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pulmonary Toxicities in Pediatric Patients Treated With Total Body Irradiation Using the Lateral Opposed Fields Technique. 采用侧对野技术进行全身照射治疗的儿科患者的肺毒性。
IF 2.4 3区 医学
Pediatric Blood & Cancer Pub Date : 2025-04-15 DOI: 10.1002/pbc.31713
Natalie Boyce, Safia Ahmed, W Scott Harmsen, Sharon Boyce, Robert Dahl, Nadia Laack
{"title":"Pulmonary Toxicities in Pediatric Patients Treated With Total Body Irradiation Using the Lateral Opposed Fields Technique.","authors":"Natalie Boyce, Safia Ahmed, W Scott Harmsen, Sharon Boyce, Robert Dahl, Nadia Laack","doi":"10.1002/pbc.31713","DOIUrl":"https://doi.org/10.1002/pbc.31713","url":null,"abstract":"<p><strong>Purpose: </strong>Pulmonary toxicity (PT) remains a concern following total body irradiation (TBI). This study describes the incidence and factors associated with PT in a cohort of patients homogeneously treated with the lateral opposed fields technique.</p><p><strong>Methods: </strong>Medical records of 61 patients ≤21 years of age treated with TBI as a component of stem cell transplantation from 1993-2017 were retrospectively reviewed. The incidences of PT, graft-versus-host disease (GVHD), and other toxicities were recorded for each patient. PT was subdivided into its etiologies, and symptomatic PT was established by clinical diagnosis. The association of PT with other variables was examined using chi-square tests, and overall survival (OS) was estimated using Kaplan-Meier.</p><p><strong>Results: </strong>Three-year OS was 65% (54%-79%). Median TBI dose rate was 7.7 cGy/min (4.0-10 cGy/min). PT occurred in 20 patients (33%). A higher rate of PT was observed in patients with GVHD (65% vs. 35%, p ≤ 0.01). TBI dose rate (</≥ 7.7 cGy/min) was not associated with the risk of PT; however, on continuous analysis, a higher dose rate increased the risk of PT (HR = 1.9, p = 0.04). PT (HR = 3.4, p = 0.01), noninfectious PT (HR = 5.0, p ≤ 0.01), and pneumonitis (HR = 4.3, p = 0.01) were associated with inferior OS.</p><p><strong>Conclusions: </strong>This cohort of patients treated with a low dose rate, bilateral technique, and total dose of 1000-1320 cGy experienced an acceptable incidence of PT, comparable to other published studies. Because PT correlated with reduced survival, current practice should seek to address factors associated with PT by aiming to prevent GVHD and by choosing low dose rates, such as in the range of 4.0-10 cGy/min.</p>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31713"},"PeriodicalIF":2.4,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144049610","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Prevalence and Impact of Autism Spectrum Disorder and Selected Neurodevelopmental Diagnoses in Hospitalized Youth With Sickle Cell Disease. 镰状细胞病住院青少年自闭症谱系障碍的患病率和影响及选定的神经发育诊断
IF 2.4 3区 医学
Pediatric Blood & Cancer Pub Date : 2025-04-14 DOI: 10.1002/pbc.31725
Megan M Griffith, Joseph R Stanek, Kathleen L Lemanek, Joseph Walden, Leena Nahata, Susan E Creary
{"title":"Prevalence and Impact of Autism Spectrum Disorder and Selected Neurodevelopmental Diagnoses in Hospitalized Youth With Sickle Cell Disease.","authors":"Megan M Griffith, Joseph R Stanek, Kathleen L Lemanek, Joseph Walden, Leena Nahata, Susan E Creary","doi":"10.1002/pbc.31725","DOIUrl":"https://doi.org/10.1002/pbc.31725","url":null,"abstract":"<p><strong>Background: </strong>Sickle cell disease (SCD) is a chronic illness accounting for 134,000 hospital admissions annually. Youth with SCD and youth with autism spectrum disorder (ASD) experience health disparities, yet the hospitalization outcomes of these youth have not been assessed. This study aimed to (i) determine the prevalence of ASD and selected neurodevelopmental disabilities (NDD) among hospitalized youth with SCD and (ii) compare hospitalization outcomes in youth with selected NDD and SCD to youth with SCD.</p><p><strong>Procedure: </strong>ICD-10 diagnosis codes were used to identify admitted youth (2-18 year olds) with SCD, ASD, and selected NDD (i.e., developmental delay) in the Pediatric Health Information System (October 2015 to April 2024). Demographic (age, race) and clinical variables (length of stay, intensive care unit [ICU] admission, 30-day readmissions, hydroxyurea use, genotype) were assessed.</p><p><strong>Results: </strong>Among 16,369 unique inpatients diagnosed with SCD (54.7% hemoglobin SS, median age = 11.9 years, and 86.4% non-Hispanic Black), 2.6% were diagnosed with a selected NDD; 1.7% were diagnosed with ASD. Hydroxyurea use during hospitalization did not differ between groups (3.3% vs. 3.5%; p = 0.19). Individuals with selected NDD had significantly higher annualized rates of hospitalization (0.88 vs. 0.65; p < .0001), ICU admission (0.12 vs. 0.05; p < .0001), and 30-day readmissions (20.2% vs. 17.4%; p = 0.0004) compared to youth without these neurodevelopmental diagnoses. The median length of stay in both groups was 3 days.</p><p><strong>Conclusions: </strong>Youth with selected NDD and SCD are at an increased risk of frequent and complicated hospitalizations. Additional research should investigate how inpatient and outpatient care delivery can be tailored and optimized to reduce the frequency and severity of hospitalizations.</p>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31725"},"PeriodicalIF":2.4,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143993576","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Hemoglobin Richmond Masquerading as Fetal Hemoglobin in a Child With Sickle Cell Disease: A Case Report and Review of the Literature. 血红蛋白里士满伪装成胎儿血红蛋白在儿童镰状细胞病:一个病例报告和文献回顾。
IF 2.4 3区 医学
Pediatric Blood & Cancer Pub Date : 2025-04-14 DOI: 10.1002/pbc.31729
Jordyn Hollander, Dominique Tran, Jennifer Jones, Dawn Gates, Carrie Gann, Cassandra Josephson, Tamara N New
{"title":"Hemoglobin Richmond Masquerading as Fetal Hemoglobin in a Child With Sickle Cell Disease: A Case Report and Review of the Literature.","authors":"Jordyn Hollander, Dominique Tran, Jennifer Jones, Dawn Gates, Carrie Gann, Cassandra Josephson, Tamara N New","doi":"10.1002/pbc.31729","DOIUrl":"https://doi.org/10.1002/pbc.31729","url":null,"abstract":"","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31729"},"PeriodicalIF":2.4,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143986968","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Illness Uncertainty in Parents of Children With Cancer-An Exploratory Analysis to Inform Intervention Development. 癌症患儿家长的疾病不确定性:为干预制定提供信息的探索性分析。
IF 2.4 3区 医学
Pediatric Blood & Cancer Pub Date : 2025-04-14 DOI: 10.1002/pbc.31726
Ijeoma Julie Eche-Ugwu, Emanuele Mazzola, Sarah E Stevens, Joanne Wolfe, Angela M Feraco
{"title":"Illness Uncertainty in Parents of Children With Cancer-An Exploratory Analysis to Inform Intervention Development.","authors":"Ijeoma Julie Eche-Ugwu, Emanuele Mazzola, Sarah E Stevens, Joanne Wolfe, Angela M Feraco","doi":"10.1002/pbc.31726","DOIUrl":"https://doi.org/10.1002/pbc.31726","url":null,"abstract":"<p><strong>Background: </strong>Illness uncertainty-a state of struggling to make sense of illness-related events-contributes to distress among parents of children with cancer. While some illness uncertainty is inescapable, uncertainty may be minimized through interventions targeting modifiable antecedents. Within the context of childhood cancer, parent and child characteristics associated with high illness uncertainty have not been identified. We sought to describe illness uncertainty among parents of children with cancer and explore potential associations with parent/child characteristics to identify populations that might benefit most from interventions to mitigate illness uncertainty.</p><p><strong>Procedure: </strong>We conducted an exploratory cross-sectional analysis of parent-reported survey data. We recruited parents of children with cancer cared for at a single academic pediatric cancer program in the northeastern United States who participated in two prospective studies (n = 89 parents) between September 2016 and June 2018. Illness uncertainty was measured by the 31-item Parent Perception of Uncertainty Scale (PPUS).</p><p><strong>Results: </strong>PPUS score distribution was slightly right-skewed. Median PPUS score was 74, with an interquartile range of 67-83. Twenty-four percent of parents strongly endorsed uncertainty regarding future planning, what their child can and cannot do, and their child's illness trajectory. Twenty percent strongly endorsed uncertainty from unanswered questions. Only 3% of parents reported their child's treatment regimen was too complex to understand. No significant associations were found between PPUS and parent/child characteristics.</p><p><strong>Conclusions: </strong>Future studies should examine additional parent and child characteristics to identify whether specific subgroups of parents may benefit most from interventions to reduce and manage illness uncertainty.</p>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31726"},"PeriodicalIF":2.4,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144016502","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Dehydrated Hereditary Stomatocytosis in a Very Preterm Twin. 极早产双胞胎的脱水遗传性口细胞增多症。
IF 2.4 3区 医学
Pediatric Blood & Cancer Pub Date : 2025-04-14 DOI: 10.1002/pbc.31730
Sho Horibe, Kyohei Isshiki, Saki Tamura, Takuro Watanabe, Yoshiteru Miyamoto, Kazushige Ikeda
{"title":"Dehydrated Hereditary Stomatocytosis in a Very Preterm Twin.","authors":"Sho Horibe, Kyohei Isshiki, Saki Tamura, Takuro Watanabe, Yoshiteru Miyamoto, Kazushige Ikeda","doi":"10.1002/pbc.31730","DOIUrl":"https://doi.org/10.1002/pbc.31730","url":null,"abstract":"","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31730"},"PeriodicalIF":2.4,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144023234","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Barriers to Hematopoietic Stem Cell Transplant Among Caregivers and Adolescents With Sickle Cell Disease. 护理人员和青少年镰状细胞病患者造血干细胞移植的障碍
IF 2.4 3区 医学
Pediatric Blood & Cancer Pub Date : 2025-04-14 DOI: 10.1002/pbc.31698
Chibuzo Ilonze, Alexandria Broadnax, Hilary Haines, Jeffrey Lebensburger, Frederick Goldman
{"title":"Barriers to Hematopoietic Stem Cell Transplant Among Caregivers and Adolescents With Sickle Cell Disease.","authors":"Chibuzo Ilonze, Alexandria Broadnax, Hilary Haines, Jeffrey Lebensburger, Frederick Goldman","doi":"10.1002/pbc.31698","DOIUrl":"https://doi.org/10.1002/pbc.31698","url":null,"abstract":"<p><strong>Background: </strong>Hematopoietic stem cell transplantation (HSCT) is a curative treatment for sickle cell disease (SCD). However, barriers exist that prevent access to this treatment modality.</p><p><strong>Methods: </strong>An IRB-approved mixed-method survey of barriers to HSCT in SCD was given to parents, caregivers, and adolescents obtaining care at the Children's of Alabama/University of Alabama at Birmingham Comprehensive Pediatric Sickle Cell Clinic.</p><p><strong>Results: </strong>We performed 103 surveys; 97 surveys (94%) were completed by a parent/guardian/caregiver, and 6 (6%) were completed by the patient. The most common SCD complication reported was three or more yearly pain crises (43%). Most (57%) respondents heard about HSCT from their healthcare provider. The most important concern with HSCT identified by respondents (67%) was the possibility of end-organ damage with the potential to worsen over time. The greatest barrier for respondents (77%) was a 5% chance of death with HSCT. Other barriers identified include the risk of reversible or permanent organ damage (73%), HSCT failure (54%), and infertility (42%).</p><p><strong>Conclusion: </strong>Respondents identified barriers to HSCT; however, they are not insurmountable. Provider education regarding the benefits and potential risks of HSCT with a clear explanation of mitigation strategies can improve acceptance of HSCT among the SCD population.</p>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31698"},"PeriodicalIF":2.4,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144015066","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Results of the Latin American Pediatric Oncology Group (GALOP) Trial for Patients With Metastatic Ewing Sarcoma: Multicentric study of Interval-Compressed Multiagent and Metronomic Chemotherapy. 拉丁美洲儿童肿瘤组(GALOP)转移性尤因肉瘤患者试验的结果:间隔压缩多药和节律化疗的多中心研究。
IF 2.4 3区 医学
Pediatric Blood & Cancer Pub Date : 2025-04-13 DOI: 10.1002/pbc.31707
Adriana Rose, Lauro Jose Gregianin, Erica Boldrini, Carla Macedo, Sima Ferman, Tatiana El Jaick Bonifácio Costa, Marcelo Scopinaro, Algemir Lunardi Brunetto, André Tesainer Brunetto, Milena Villarroel
{"title":"Results of the Latin American Pediatric Oncology Group (GALOP) Trial for Patients With Metastatic Ewing Sarcoma: Multicentric study of Interval-Compressed Multiagent and Metronomic Chemotherapy.","authors":"Adriana Rose, Lauro Jose Gregianin, Erica Boldrini, Carla Macedo, Sima Ferman, Tatiana El Jaick Bonifácio Costa, Marcelo Scopinaro, Algemir Lunardi Brunetto, André Tesainer Brunetto, Milena Villarroel","doi":"10.1002/pbc.31707","DOIUrl":"https://doi.org/10.1002/pbc.31707","url":null,"abstract":"<p><strong>Background: </strong>GALOP investigators developed a multicenter protocol to standardize treatment for newly diagnosed metastatic Ewing sarcoma (ES) in South America.</p><p><strong>Methods: </strong>Prospective trial. Induction chemotherapy consisted of 9 alternating interval-compressed cycles (every 14 days) of vincristine, doxorubicin, cyclophosphamide, and ifosfamide-etoposide; local and metastatic site control; and 5 consolidation cycles (every 21 days), followed by MCT with cyclophosphamide and vinblastine for 1 year.</p><p><strong>Results: </strong>Between 2011 and 2019, 198 patients were recruited from 34 centers in Argentina, Brazil, Chile, and Uruguay. Characteristics include: male patients (60.6%), median age of 12.3 years (range, 0.8-31.1); axial primary localization (62.1%), size >8 cm (70.2%); and bone origin (71.2%). Metastatic sites were lung, extra-lung, and combined in 43.4%, 31.3%, and 25.3%, respectively. The overall response rate was 79.3%, and local treatment was performed in 85.3% of patients. With a median follow-up of 65.1 months (95% CI: 53.9-76.4), the 5-year overall survival (OS) and event-free survival (EFS) were 33.1% (95% CI: 25.9-40.4) and 27.8% (95% CI: 21.5-34.3), respectively. The 5-year OS was 44.9%, 31.3%, and 15.6% for lung, extra-lung, and combined, respectively (p < 0.001). The median interval between induction chemotherapy cycles was 17 days, with a febrile neutropenia rate of 19.3%. Metronomic chemotherapy (MCT) was administered to 100 patients (50.5%), demonstrating good tolerability, with 58 patients completing at least 75% of the scheduled cycles.</p><p><strong>Conclusion: </strong>The implementation of a multicenter protocol incorporating MCT for metastatic ES proved feasible across Latin America.</p>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31707"},"PeriodicalIF":2.4,"publicationDate":"2025-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144037723","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
MRI-Based Score to Recognize Thalamic Glioma Grade in Children: Morphology, Diffusion, and Arterial-Spin-Labeling Perfusion. 基于mri评分识别儿童丘脑胶质瘤分级:形态学、扩散和动脉自旋标记灌注。
IF 2.4 3区 医学
Pediatric Blood & Cancer Pub Date : 2025-04-11 DOI: 10.1002/pbc.31704
Alexis Plasse, Raphael Levy, Arnault Tauziède-Espariat, Thibault Agripnidis, Jean-François Hak, Charles-Joris Roux, Thomas Samoyeau, Alexis Ollitrault, Kevin Beccaria, Lelio Guida, Thomas Blauwblomme, Jacques Grill, Christelle Dufour, Léa Guerrini-Rousseau, Franck Bourdeaut, François Doz, Pascale Varlet, Nathalie Boddaert, Volodia Dangouloff-Ros
{"title":"MRI-Based Score to Recognize Thalamic Glioma Grade in Children: Morphology, Diffusion, and Arterial-Spin-Labeling Perfusion.","authors":"Alexis Plasse, Raphael Levy, Arnault Tauziède-Espariat, Thibault Agripnidis, Jean-François Hak, Charles-Joris Roux, Thomas Samoyeau, Alexis Ollitrault, Kevin Beccaria, Lelio Guida, Thomas Blauwblomme, Jacques Grill, Christelle Dufour, Léa Guerrini-Rousseau, Franck Bourdeaut, François Doz, Pascale Varlet, Nathalie Boddaert, Volodia Dangouloff-Ros","doi":"10.1002/pbc.31704","DOIUrl":"https://doi.org/10.1002/pbc.31704","url":null,"abstract":"<p><strong>Purpose: </strong>Thalamic gliomas are found predominantly in children and can be classified into two main types with different prognoses and management: diffuse midline glioma (DMG) H3K27-altered and low-grade glioma (LGG). Our aim was to find imaging features distinguishing these tumors and to develop a diagnostic score.</p><p><strong>Patients and methods: </strong>A retrospective study spanning September 1999 to May 2021 involved pediatric patients with thalamic gliomas, categorized into H3K27-altered DMG and LGG groups. Preoperative imaging, including morphology, diffusion, and arterial-spin-labeling perfusion, was reviewed blindly and compared between the two groups. A diagnostic score was formulated based on significant findings. Results were validated using an internal and external validation cohort.</p><p><strong>Results: </strong>Sixty-six patients were included (median age, 9 years; interquartile range [IQR] [5-13]; 38 girls) with 37 DMG H3K27-altered and 29 LGG. DMG H3K27-altered tumors exhibited larger volumes (median 64 cc, IQR [36-88] vs 26 cc, IQR [15-37], p < 0.001), greater heterogeneity in T2-weighted signal and enhancement (62% [22/37] vs 31% [9/29], p = 0.04, and 97% [32/33] vs 56% [15/27], p = 0.0008, respectively), lower minimum relative apparent diffusion coefficient (ADC) (0.92 (IQR [0.76-1.23]) vs 1.55 (IQR [1.40-1.72]), p < 0.001), and higher relative maximum cerebral blood flow (CBF) levels (2.08 (IQR [1.48-3.05]) vs 0.84 (IQR [0.45-1]), p < 0.001). A diagnostic score integrating tumor diameter, solid content predominance, relative ADC, and relative CBF achieved 100% sensitivity and specificity in distinguishing DMG H3K27-altered tumors from LGG (full score available for 36 patients), with good results in external and internal validation cohorts (12 patients).</p><p><strong>Conclusion: </strong>The morphological, diffusion, and arterial spin labeling imaging characteristics of pediatric thalamic tumors enable excellent differentiation of DMG H3K27-altered and LGG.</p>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31704"},"PeriodicalIF":2.4,"publicationDate":"2025-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144016506","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Prognostic Role of Systemic Inflammatory Markers in Pediatric Soft-Tissue Sarcoma: A Multicenter Study of 213 Patients. 全身性炎症标志物在小儿软组织肉瘤中的预后作用:213例患者的多中心研究
IF 2.4 3区 医学
Pediatric Blood & Cancer Pub Date : 2025-04-11 DOI: 10.1002/pbc.31701
Anna Gabrych, Malgorzata A Krawczyk, Malgorzata Styczewska, Weronika Lyzinska, Jadwiga Weclawek-Tompol, Iwona Dachowska-Kalwak, Bernarda Kazanowska, Malgorzata Sawicka-Zukowska, Maryna Krawczuk-Rybak, Monika Richert-Przygonska, Jan Styczynski, Julia Geisler-Paliwoda, Tomasz Szczepanski, Wioletta Bal, Radoslaw Chaber, Michal Kunc, Ewa Bien
{"title":"Prognostic Role of Systemic Inflammatory Markers in Pediatric Soft-Tissue Sarcoma: A Multicenter Study of 213 Patients.","authors":"Anna Gabrych, Malgorzata A Krawczyk, Malgorzata Styczewska, Weronika Lyzinska, Jadwiga Weclawek-Tompol, Iwona Dachowska-Kalwak, Bernarda Kazanowska, Malgorzata Sawicka-Zukowska, Maryna Krawczuk-Rybak, Monika Richert-Przygonska, Jan Styczynski, Julia Geisler-Paliwoda, Tomasz Szczepanski, Wioletta Bal, Radoslaw Chaber, Michal Kunc, Ewa Bien","doi":"10.1002/pbc.31701","DOIUrl":"https://doi.org/10.1002/pbc.31701","url":null,"abstract":"<p><strong>Background: </strong>The prognostic role of systemic inflammation markers in pediatric soft-tissue sarcomas (STS) remains unclear.</p><p><strong>Procedure: </strong>This multicenter study investigated the prognostic significance of neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), lymphocyte-to-monocyte ratio (LMR), systemic immune-inflammation index (SII), systemic inflammatory response index (SIRI), C-reactive protein (CRP), and lactate dehydrogenase (LDH) in 213 pediatric patients diagnosed with STS in years 2002-2023. Patients were categorized into groups: rhabdomyosarcoma (RMS, n = 126), RMS-like (n = 57), and non-RMS (n = 30). Clinicopathological data, including complete blood counts (CBCs), CRP, and LDH levels, were collected and age-adjusted. Optimal cutoffs for predicting outcomes were determined, and the prognostic value of the inflammatory markers was assessed using Kaplan-Meier survival analysis, log-rank tests, and Cox regression models.</p><p><strong>Results: </strong>No significant differences in NLR, PLR, LMR, and CRP levels were observed between RMS, RMS-like, and non-RMS groups. However, LDH levels were significantly elevated in the RMS group compared with the RMS-like group. A consistent trend toward higher NLR, PLR, and CRP values was noted in patients with more advanced disease stages. Multivariate Cox regression analysis across the entire cohort identified CRP (HR 3.39, 95% CI 1.55-7.4, p = 0.002), NLR (HR 2.06, 95% CI 1.07-3.99, p = 0.03), and disease stage (HR = 0.49, 95% CI 0.26-0.95, p = 0.035) as independent prognostic factors for survival. Subgroup analyses revealed that the prognostic impact of these markers varied across histopathological subtypes, with limited utility in the RMS-like group.</p><p><strong>Conclusions: </strong>These findings highlight the prognostic value of systemic inflammatory markers in pediatric STS, emphasizing their potential to refine risk assessment and guide treatment.</p>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31701"},"PeriodicalIF":2.4,"publicationDate":"2025-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144049609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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