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Children with Diffuse Intrinsic Pontine Gliomas (DIPG), a malignant brainstem tumor, experience poor prognosis. Because of the disease's rarity and highly aggressive course, there is a dearth of research on cognitive and psychosocial outcomes in this underserved, vulnerable population. However, evaluating effects of the disease and treatment on the cognitive and daily functioning of these patients is important to better understand their specific needs and improve their quality of life. The current longitudinal study administered prospective neuropsychological assessments to children diagnosed with CNS malignancies, including the largest sample of children with DIPG to date (n = 21, mean age = 7.86 years, range = 3-16) in neurocognitive, behavioral, social-emotional, and adaptive functioning at baseline, two weeks post-radiation, and six months later. The results describe population-based, cross-sectional characteristics and within-patient longitudinal changes. Prior to radiation, children with DIPG exhibited significant weaknesses compared to normative samples in both parent-report and performance-based measures of attention, and tests of processing speed and verbal learning/memory. Younger children demonstrated poorer inhibitory control on performance tests and worse parent-reported behavioral regulation, depression, and social withdrawal compared to older children. Six-months post-radiation, older children exhibited poorer socialization than younger children. Longitudinally, children with DIPG exhibited short-term improvements immediately post-radiation in performance-based attention tests and parent-reported behavior, including attention, hyperactivity, behavioral regulation, and executive function. However, these improvements did not persist and significant decline was documented on tests of attention by six months. Clinical implications for professionals working with children with DIPG and recommendations for cognitive remediation and quality of life interventions are provided.
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http://dx.doi.org/10.1080/09297049.2022.2144189 | DOI Listing |
Oncogene
September 2025
Division of Neurosurgery, Children's Hospital Los Angeles, Los Angeles, CA, USA.
It has become evident from decades of clinical trials that multimodal therapeutic approaches with focus on cell intrinsic and microenvironmental cues are needed to improve understanding and treat the rare, inoperable, and ultimately fatal diffuse intrinsic pontine glioma (DIPG), now categorized as a diffuse midline glioma. In this study we report the development and characterization of an in vitro system utilizing 3D Tumor Tissue Analogs (TTA), designed to replicate the intricate DIPG microenvironment. The innate ability of fluorescently labeled human brain endothelial cells, microglia, and patient-derived DIPG cell lines to self-assemble has been exploited to generate multicellular 3D TTAs that mimic tissue-like microstructures, enabling an in- depth exploration of the spatio-temporal dynamics between neoplastic and stromal cells.
View Article and Find Full Text PDFFront Immunol
September 2025
Dmitry Rogachev National Medical Center of Pediatric Hematology, Oncology, and Immunology, Moscow, Russia.
Central nervous system (CNS) tumors are the second most common type of cancer in children and remain the leading cause of mortality in pediatric oncology. For patients with high-risk CNS tumors, standard treatments often prove ineffective, with survival rates being less than 10%. Hence, there is an urgent need to develop alternative treatment strategies for this patient population.
View Article and Find Full Text PDFSci Rep
August 2025
Department of Neurosurgery, Stanford University School of Medicine, Stanford, CA, USA.
Pediatric high-grade gliomas (pHGG) are highly invasive with poor survival outcomes. Timing of Temozolomide administration has been shown to affect survival of adult patients with glioblastoma. We investigated whether pHGGs express circadian genes rhythmically and whether underlying rhythms affect Temozolomide sensitivity.
View Article and Find Full Text PDFMed Oncol
August 2025
Department of Pathology, College of Medicine, Imam Mohammad Ibn Saud Islamic University (IMSIU), Riyadh, Saudi Arabia.
Pediatric central nervous system (CNS) tumors rank second among the fatal childhood malignancies, primarily due to the significant challenge posed by the blood-brain barrier (BBB), which limits the therapeutic delivery and contributes to poor clinical outcomes. This challenge is exacerbated by the distinct developmental traits of pediatric BBB, the immunosuppressive tumor microenvironment (TME), and the inherent diversity of different tumor types such as medulloblastoma and diffuse intrinsic pontine glioma (DIPG). Recent advances at the crossroad of immunotherapy and nanomedicine are paving the way for transformative strategies to penetrate the BBB and modify the immunogenic landscape within pediatric CNS tumors.
View Article and Find Full Text PDFNeurooncol Pract
August 2025
The Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Background: The Response Assessment in Pediatric Neuro-Oncology (RAPNO) working group for diffuse intrinsic pontine glioma (DIPG) recently published its recommendations. We aim to test the operative performance of the RAPNO DIPG criteria imaging component by retrospectively applying it to a patient sample from the International DIPG/DMG Registry (IDIPGR).
Methods: Longitudinal MRIs for 46 patients were independently reviewed by 2 pediatric neuro-radiologists.