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Pediatric Pleomorphic Xanthoastrocytomas: A Multicenter Neuroradiological and Clinical Correlation Study
Karanjot Chhatwal1, Thomas Hargreaves1, Francesco Pacchiano1
1From the Imperial College School of Medicine (K.C.), Imperial College London, London, UK; Department of Radiology (K.C., T.H., H.M., P.S., F.D., U.L., U.G., S.S., A.B., K.M.), Department of Neurosurgery (K.A.), Department of Histopathology (T.S.J., A. M.), Great Ormond Street Hospital for Children, Great Ormond Street, London; Barts and The London School of Medicine and Dentistry (T.H.), Queen Mary University of London, London, UK; University of Naples (F.P.), Federico II, Naples, Italy; Department of Radiology (C.A., V.R.), Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; Children's Healthcare of Atlanta (A.E.G.- Y.), Emory University School of Medicine, GA, USA; Department of Radiology (A.B., A.K., A.G.), Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Radiology (A.J., K.W.Y.), Phoenix Children's Hospital, Phoenix, AZ, USA; Department of Radiology (J.N.), Seattle Children's Hospital, Seattle, WA, USA; Department of Neuroscience (E.P.), Imaging and Clinical Sciences, University of Chieti-Pescara, Chieti, Italy; Oncological Neuroradiology and Advanced Diagnostics Unit (E.P.), Bambino Gesùu Children's Hospital, IRCCS, Rome, Italy; Department of Radiology (K.H., K.A.), Aga Khan University Hospital, Karachi, Pakistan and UCL GOS Institute of Child Health (T.S.J.), London, UK.
This study reveals that pediatric pleomorphic xanthoastrocytomas (PXAs) have specific imaging and molecular traits linked to outcomes. Early surgical removal of pediatric brain tumors improves survival, guiding personalized treatment strategies.
Area of Science:
- Pediatric neuro-oncology
- Brain tumor imaging
- Molecular neuropathology
Background:
- Pleomorphic xanthoastrocytomas (PXAs) are rare pediatric brain tumors.
- Understanding of PXA imaging characteristics and clinical outcomes requires further investigation.
Purpose of the Study:
- To conduct a large international, multi-institutional analysis of pediatric PXAs.
- To focus on neuroimaging features and clinical outcomes in pediatric PXAs.
Main Methods:
- Retrospective international multi-center study of 63 pediatric PXAs.
- Review of neuroimaging data and molecular analyses (BRAF V600E, CDKN2A/B deletions).
- Analysis of treatment modalities and survival outcomes using statistical models.
Main Results:
- CNS WHO grade 3 PXAs were larger than grade 2. BRAF V600E mutations (78%) and CDKN2A/B deletions (93.5%) were common.
- MRI showed well-defined tumors with cysts; CT revealed isointense tumors. Gross total resection improved progression-free survival (HR=0.39).
- Tumor recurrence occurred in 44.8% at 3 years; 3-year overall survival was 94.7%.
Conclusions:
- Pediatric PXAs have distinct neuroimaging and molecular features correlating with prognosis.
- Integrated evaluation of radiological and clinical data is crucial for risk stratification.
- Personalized therapeutic strategies are needed for this rare pediatric brain tumor population.
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