CNS Embryonal Tumours
Prognosis
This section is being prepared by our editorial process.
🔬 Histological Types
📚 Latest Research
Recurrent C19MC-altered embryonal tumors with multilayered rosettes: outcomes of stereotactic radiosurgery.
Harary PM, et al
A retrospective case series of three pediatric patients with C19MC-altered embryonal tumors with multilayered rosettes (ETMR) suggests that stereotactic radiosurgery (SRS) can serve as a feasible salvage strategy for localized recurrence, with one patient remaining disease-free 16 years after initial diagnosis. All three patients had previously undergone gross total resection and adjuvant chemoradiotherapy before experiencing localized recurrence; case 1 (age 3) achieved stability at 22 months post-diagnosis after multiple SRS courses, case 2 (age 4) obtained local control but succumbed to diffuse leptomeningeal spread at 20.5 months post-diagnosis, and case 3 (age 3) became a long-term survivor. SRS was well-tolerated across all cases with no acute neurotoxicity, suggesting it may minimize the neurocognitive burden associated with large-field re-irradiation in very young children. The authors caution that SRS efficacy cannot be isolated from concurrent salvage therapies and call for prospective studies to define its role within multimodal ETMR protocols.
Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
Source →Molecular profiling of pediatric medulloblastoma in Kazakhstan: Genomic alterations, subgroup distribution, and survival.
Bolatov A, et al
A retrospective analysis of 40 pediatric medulloblastomas diagnosed between 2015 and 2024 at a tertiary center in Kazakhstan found that WNT-subgroup tumors (n=7, 17.5%) carried the most favorable prognosis, while non-WNT/non-SHH tumors (n=23, 57.5%) showed the poorest long-term survival and were the only subgroup linked to metastatic disease at presentation. SHH tumors (n=10, 25.0%) displayed characteristic desmoplastic/nodular histology, YAP1/GAB1 expression, and PTCH1/SUFU alterations, with a subset harboring TP53 and TERT alterations. Using archived formalin-fixed, paraffin-embedded tissue, the team combined immunohistochemistry and whole-exome sequencing to achieve clinically meaningful molecular stratification without advanced classification platforms. The findings demonstrate that resource-adapted molecular profiling is feasible in underrepresented settings and produces subgroup assignments consistent with established global biology.
Brain pathology (Zurich, Switzerland)
Source →Deep Learning Pipeline for Automatic Segmentation, Classification, and Molecular Subtyping of Three Pediatric Posterior Fossa Tumors Using T2-Weighted MRI.
Jin Y, et al
A fully automated deep learning pipeline using standard T2-weighted MRI has been developed and validated to segment, classify, and molecularly subtype three pediatric posterior fossa tumors — medulloblastoma, ependymoma, and pilocytic astrocytoma — achieving tumor segmentation Dice scores of 0.94–0.96 and tumor-type classification accuracy of 0.824–0.918 (Cohen's kappa 0.722–0.873) across internal and external test sets in a multicenter cohort of 1,305 patients. Separate models for molecular subtyping reached an accuracy of 0.794 (Cohen's kappa 0.605) for medulloblastoma and 0.789 (Cohen's kappa 0.538) for ependymoma. This noninvasive, fully automated approach could enable preoperative differentiation of tumor types and molecular subtypes in children without the need for surgical sampling, directly informing treatment planning and prognosis.
Journal of magnetic resonance imaging : JMRI
Source →💊 Therapies
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🧪 Tumor markers
This section is being prepared by our editorial process.