Severe Hearing Loss in Children With Central Nervous System Tumors: A Population-Based Cancer in Young People in Canada (CYP-C) Report.

Purpose: Children with central nervous system (CNS) tumors are prone to treatment-related hearing loss (HL) and subsequent functional impairment. This study reports a dedicated population-based analysis of CNS tumor-specific rates and predictors of early severe HL.

Methods: A cohort study of children ≤15 years diagnosed with CNS tumors between 2001 and 2019 through the Cancer in Young People in Canada (CYP-C) program.

Barriers and Enablers to Engaging with Long-Term Follow-Up Care Among Canadian Survivors of Pediatric Cancer: A COM-B Analysis.

Survivors of pediatric cancer are at risk for late effects and require risk-adapted long-term follow-up (LTFU) care. Yet less than 50% of survivors attend LTFU care. This study aimed to identify barriers and enablers of engaging with LTFU care as perceived by Canadian survivors of pediatric cancer and healthcare providers (HCPs).

A review of diffuse hemispheric glioma, H3 G34-mutant disease development, DNA repair, microenvironment, and treatments on the horizon.

Diffuse hemispheric glioma H3 G34-mutant is primarily diagnosed in adolescents/young adults. While molecular diagnostics have improved, cellular mechanisms that drive tumor progression and therapy resistance are poorly understood. Combining previous published studies with findings from the 2024 NIH G34-mutant symposium aid in summarizing translational and clinical updates on disease development, cellular repair processes, and the immune microenvironment.

Co-Designing Priority Components of an mHealth Intervention to Enhance Follow-Up Care in Young Adult Survivors of Childhood Cancer and Health Care Providers: Qualitative Descriptive Study.

Background: Survivors of childhood cancer are at risk of medical, psychological, and social late effects. To screen for their risks, receipt of consistent, cancer-specific follow-up care is crucial. However, <50% of survivors attend their aftercare, and only 35% of them recognize that they could have a serious health problem.

Salvage therapies for first relapse of SHH medulloblastoma in early childhood.

Background: Sonic hedgehog (SHH) medulloblastoma is the most common molecular group of infant and early childhood medulloblastoma (iMB) and has no standard of care at relapse. This work aimed to evaluate the post-relapse survival (PRS) and explore prognostic factors of patients with nodular desmoplastic (ND) and/or SHH iMB.

Methods: This international retrospective study included 147 subjects diagnosed with relapsed Nodular Desmoplastic/SHH iMB between 1995 and 2017, < 6 years old at original diagnosis, and treated without initial craniospinal irradiation (CSI).

Intra- and interobserver agreement in evaluation of image-defined risk factors on computed tomography in pediatric neuroblastoma.

Background: Image-defined risk factors (IDRFs) were introduced to provide a consensus approach for pre-treatment risk stratification on computed tomography (CT) and magnetic resonance imaging (MRI) in patients with neuroblastoma.

Objective: To assess the intra- and inter-reader agreement of radiologists in identifying IDRFs on CT.

Materials And Methods: Approval for this retrospective study was granted by our institutional research ethics board with a waiver of consent.

Clinical Characteristics and Outcomes of Central Nervous System Tumors Harboring NTRK Gene Fusions.

Purpose: Tropomyosin receptor kinase (TRK) fusions are detected in less than 2% of central nervous system tumors. There are limited data on the clinical course of affected patients.

Experimental Design: We conducted an international retrospective cohort study of patients with TRK fusion-driven central nervous system tumors.

Canadian Consensus for Treatment of BRAF Mutated Pediatric and AYA Gliomas.

The treatment of BRAF V600E gliomas with BRAF inhibitors (BRAFis) and MEK inhibitors (MEKis) has been increasingly integrated into clinical practice for pediatric low-grade gliomas (PLGGs) and pediatric high-grade gliomas (HGGs). However, some questions remain unanswered, such as the best time to start targeted therapy, duration of treatment, and discontinuation of therapy. Given that no clinical trial has been able to address these critical questions, we developed a Canadian Consensus statement for the treatment of BRAF V600E mutated pediatric as well as adolescent and young adult (AYA) gliomas.

Upfront BRAF/MEK inhibitors for treatment of high-grade glioma: A case report and review of the literature.

Background: High-grade gliomas (HGG) with V600E mutation represent a unique subset of central nervous system tumors. Targeted therapies including BRAF and MEK inhibitors are now being explored as possible new treatment options.

Methods: We report an 18-year-old female with a grade 3 pleomorphic xanthoastrocytoma treated upfront with dabrafenib and trametinib.

Outcomes of Infants and Young Children With Relapsed Medulloblastoma After Initial Craniospinal Irradiation-Sparing Approaches: An International Cohort Study.

Purpose: Infant and young childhood medulloblastoma (iMB) is usually treated without craniospinal irradiation (CSI) to avoid neurocognitive late effects. Unfortunately, many children relapse. The purpose of this study was to assess salvage strategies and prognostic features of patients with iMB who relapse after CSI-sparing therapy.

Systematic review of diffuse hemispheric glioma, H3 G34-mutant: Outcomes and associated clinical factors.

Background: A comprehensive review and description of the clinical features that impact prognosis for patients with diffuse hemispheric glioma, H3 G34-mutant (G34-DHG) is needed. Understanding survival and prognostic features is paramount for clinical advancements and patient care.

Methods: PubMed, Embase, and Google Scholar were searched for English articles published between January 1, 2012 and June 30, 2021.

Characteristics of children ≤36 months of age with DIPG: A report from the international DIPG registry.

Background: Children ≤36 months with diffuse intrinsic pontine glioma (DIPG) have increased long-term survival (LTS, overall survival (OS) ≥24 months). Understanding distinguishing characteristics in this population is critical to improving outcomes.

Methods: Patients ≤36 months at diagnosis enrolled on the International DIPG Registry (IDIPGR) with central imaging confirmation were included.

Impact of Time to Diagnosis on Morbidity and Survival in Children With Malignant Central Nervous System Tumors.

Objective: The aim was to determine the impact of time to diagnosis (TTD) on morbidity and mortality and to identify factors associated with overall survival (OS) in pediatric patients with malignant central nervous system (CNS) tumors.

Methods: This is a retrospective review of all malignant CNS tumors presenting to 2 tertiary care pediatric hospitals from 2000 to 2019. Cox proportional hazard model analysis outcomes included TTD and OS as well as morbidity; stratified by tumor category, age, relapse, and presence of metastatic disease.

Massive Saddle Pulmonary Embolism in a Preterm Neonate With Successful Emergent Open Embolectomy.

Pulmonary embolism in the neonate is a rare, life-threatening emergency. Risk factors for neonatal pulmonary embolism (PE) include sepsis, asphyxia, prematurity, and vascular catheterisation. We report the case of a preterm neonate with a massive saddle pulmonary thrombosis of unidentified etiology.

Characteristics of patients ≥10 years of age with diffuse intrinsic pontine glioma: a report from the International DIPG/DMG Registry.

Background: Diffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this study was to describe clinical, radiological, pathologic, and molecular characteristics in patients ≥10 years of age with DIPG enrolled in the International DIPG Registry (IDIPGR).

Methods: Patients ≥10 years of age at diagnosis enrolled in the IDIPGR with imaging confirmed DIPG diagnosis were included.

Incidence and risk factors of venous thrombotic events in pediatric patients with CNS tumors compared with non-CNS cancer: A population-based cohort study.

Background: Venous thromboembolism (VTE) is a well-recognized complication in pediatric cancer patients. It has been demonstrated that the incidence of VTE in pediatric patients with central nervous system (CNS) tumors is lower than that of patients with other cancers. Risk factors for developing cancer-related thrombosis are numerous and can include patient, disease, or treatment-related influences.

Overcoming barriers to establishing autopsy procurement programs in pediatric patients with central nervous system tumors: a call to develop regional centers.

Background: While autopsy-repository programs with a variety of pediatric central nervous system (CNS) tumor types are a critical resource for preclinical neuro-oncology research, few exist and there is no published guidance on how to develop one. The goal of this prospective Pediatric Brain Tumor Repository (PBTR) study was to develop such a program at Cincinnati Children's Hospital Medical Center (CCHMC) and then publish the quantitative and experiential data as a guide to support the development of similar programs.

Methods: Protocols and infrastructure were established-to educate oncologists and families, establish eligibility, obtain consent, address pre- and post-autopsy logistics (e.