Publications by authors named "Sylvia Cheng"

Purpose: 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 CNS tumors.

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  • This study examines how the Wim Hof Method (WHM) compares to slow breathing in managing stress and depressive symptoms in women with high stress levels.
  • 84 midlife women were randomly assigned to either the WHM group, which involved specific breathing techniques and cold exposure, or a control group practicing slow-paced breathing and warm showers over three weeks during the COVID-19 pandemic.
  • Both groups reported significant reductions in depressive and anxiety symptoms immediately after the intervention and at a 3-month follow-up, but participants in the WHM group had lower retention rates, suggesting differences in perceived credibility and expected benefits of the interventions.
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  • Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is a diverse and challenging type of cancer that often has poor outcomes, especially in younger patients lacking the SMARCB1 protein.
  • Research indicated that human and mouse PTCL-NOS exhibit similar DNA changes, including the hypermethylation of T-cell genes and the hypomethylation of myeloid development genes, contributing to a complicated tumor ecosystem.
  • A study found that histone deacetylase inhibitors (HDACi), like SAHA, can effectively treat PTCL-NOS by modifying the tumor's microenvironment and improving immune function, paving the way for potential combination therapies.
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  • Successful treatment of acute lymphoblastic leukemia (ALL) involves multiagent chemotherapy and CNS prophylaxis, but a minority of patients can develop drug resistant epilepsy (DRE) and epileptic encephalopathies (EE) like Lennox-Gastaut syndrome (LGS).
  • A study of four ALL remission patients revealed that all patients faced significant challenges, including DRE, moderate intellectual disability, and neuropsychiatric issues, with symptoms often beginning around the age of 5.6 years.
  • The findings suggest that younger age at diagnosis (under 3 years) may increase the risk of developing DRE and EEs, while common palliative treatments like the ketogenic diet and corpus callosotomy show limited effectiveness.
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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.

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Background: Although CNS tumors are the most common pediatric cancer in the United States, most physicians caring for these patients are not formally certified in the subspecialty. To determine support for developing a formal certification process in pediatric neuro-oncology, the Society for Neuro-Oncology's Pediatrics Special Interest Track Training and Credentialing working group performed a cross-sectional survey-based study of physicians and patients/caregivers of children with a CNS tumor history.

Methods: Surveys were built in Survey Monkey and were available for 3 months.

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Objectives: We conducted a retrospective multi-centre study to assess the real-world outcome of regorafenib (REGO) and cabozantinib (CABO) in recurrent/refractory bone tumours (BTs) including osteosarcoma (OST), Ewing sarcoma (EWS) and chondrosarcoma (CS)/extra-skeletal mesenchymal CS (ESMC).

Methods: After regulatory approval, data from patients with recurrent BT (11 institutions) were extracted from CanSaRCC (Canadian Sarcoma Research and Clinical Collaboration) database. Patient characteristics, treatment and outcomes were collected.

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Objective: To describe disease outcomes including overall survival and relapse patterns by subgroup in young pediatric patients treated for medulloblastoma with a radiation-sparing approach.

Methods: Retrospective analysis of clinical outcomes includes treatment, relapse, and salvage therapy and late effects in children treated for medulloblastoma with a radiation-sparing approach at British Columbia Children's Hospital (BCCH) between 2000 and 2020.

Results: There were 30 patients (median age 2.

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Question: Headache, vomiting, lethargy, and seizures are common symptoms in healthy children with benign viral illnesses, but they are also signs that could represent a central nervous system (CNS) tumour. Primary care providers and guardians are hesitant to expose children to radiation associated with computed tomography scans or take on risks associated with the sedation frequently needed for magnetic resonance imaging. When should primary care providers order radiologic head imaging for children with common symptoms to identify those with a CNS tumour?

Answer: Central nervous system tumours have no pathognomonic features, which often results in delays in diagnosis.

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Introduction: Craniopharyngioma is a rare, low-grade tumor located in the suprasellar region of the brain, near critical structures like the pituitary gland. Here, we concurrently investigate the status of clinical and genomic data in a retrospective craniopharyngioma cohort and survey-based data to better understand patient-relevant outcomes associated with existing therapies and provide a foundation to inform new treatment strategies.

Methods: Clinical, genomic, and outcome data for a retrospective cohort of patients with craniopharyngioma were collected and reviewed through the Children's Brain Tumor Network (CBTN) database.

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  • This study identifies the first human case of complete deficiency in NFAT1, a protein crucial for immune cell activation, linked to a specific genetic mutation in the NFATC2 gene.* -
  • The patient exhibited severe health issues including joint contractures, bone growths, and recurrent B-cell lymphoma, suggesting NFAT1's role in both immune response and cancer development.* -
  • The findings also reveal how the lack of NFAT1 affects immune cell distribution and function, indicating the need for better diagnosis and caution regarding the long-term use of calcineurin inhibitors.*
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Accumulating evidence suggests that maternal exposure to objectively stressful events and subjective distress during pregnancy may have intergenerational impacts on children's mental health, yet evidence is limited. In a multisite longitudinal cohort (N = 454), we used multi-variable linear regression models to evaluate the predictive value of exposure to stressful events and perceived distress in pregnancy for children's internalizing problems, externalizing problems, and adaptive skills at age 4. We also explored two- and three-way interactions between stressful events, distress, and child sex.

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Low-grade diffusely infiltrative tumour (LGDIT), SMARCB1-mutant, is a histopathological distinct low-grade lesion encountered in older children and young adults that shows epigenetic similarity with ATRT-MYC and has the potential for malignant progression.

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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.

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  • Diffuse intrinsic pontine glioma (DIPG) is primarily diagnosed through imaging rather than tissue samples, highlighting the need for accurate imaging to differentiate it from other pontine tumors.
  • Analysis of 286 patients revealed that 8% had diagnoses inconsistent with DIPG, with many identified as lower-grade gliomas or embryonal tumors, illustrating the potential misclassification.
  • The study found a significant correlation between neuro-imaging reviews and histopathologic diagnoses, with imaging insights being crucial for confirming DIPG and predicting patient survival.
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Purpose: To describe a series of children with extensive PNF or treatment refractory PLGG treated on a compassionate basis with trametinib.

Methods: We report on six patients with NF-1 treated with trametinib on a compassionate basis at British Columbia Children's Hospital since 2017. Data were collected retrospectively from the patient record.

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This is the first report of a NACC2-NTRK2 fusion in a histological glioblastoma. Oncogenomic analysis revealed this actionable fusion oncogene in a pediatric cerebellar glioblastoma, which would not have been identified through routine diagnostics, demonstrating the value of clinical genome profiling in cancer care.

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Background: Anaplastic pleomorphic xanthoastrocytoma (APXA) is a rare subtype of CNS astrocytoma. They are generally treated as high-grade gliomas; however, uncertainty exists regarding the optimal therapy. Here, we report on 3 pediatric cases of APXA.

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Primary CNS tumors are the leading cause of cancer-related death in pediatrics. It is essential to understand treatment trends to interpret national survival data. In Canada, children with CNS tumors are treated at one of 16 tertiary care centers.

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