Pediatric Pleomorphic Xanthoastrocytomas: A Multicenter Neuroradiologic and Clinical Correlation Study
15 affiliations
- 1From the Imperial College School of Medicine (K.C.), Imperial College London, London, UK
- 2Department of Radiology (K.C., T.H., H.M., P.S., F.D., P.G., U.L., U.G., S.S., A.B., K.M.), Great Ormond Street Hospital, London, UK
- 3Barts and The London School of Medicine and Dentistry (T.H.), Queen Mary University of London, London, UK
- 4University of Naples (F.P.), Federico II, Naples, Italy
- 5Department of Radiology (C.A., V.R.), Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts
- 6Children’s Healthcare of Atlanta (A.E.G.-Y.), Emory University School of Medicine, Atlanta, Georgia
- 7Department of Radiology (A.B., A.K., A.G.), Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania
- 8Department of Radiology (A.J., K.W.Y.), Phoenix Children’s Hospital, Phoenix, Arizona
- 9Department of Radiology (J.N.), Seattle Children's Hospital, Seattle, Washington
- 10Department of Neuroscience (E.P.), Imaging and Clinical Sciences, University of Chieti-Pescara, Chieti, Italy
- 11Oncological Neuroradiology and Advanced Diagnostics Unit (E.P.), Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
- 12Department of Radiology (K.H.), Aga Khan University Hospital, Karachi, Pakistan
- 13Department of Neurosurgery (K.A.), Great Ormond Street Hospital for Children, London
- 14Department of Histopathology (T.S.J., A.M.), Great Ormond Street Hospital for Children, London, UK
- 15the University College London Great Ormond Street Institute of Child Health (T.S.J.), London, UK.
Abstract
BACKGROUND AND PURPOSE: Pleomorphic Xanthoastrocytomas (PXAs) are rare pediatric brain tumors accounting for approximately 1% of primary brain neoplasms in children and young adults. Despite recent imaging and molecular advances, gaps remain in our understanding of their diverse imaging characteristics and clinical outcomes. This study provides a large international, multi-institutional analysis of pediatric PXAs focusing on neuroimaging and clinical outcomes. MATERIALS AND METHODS: We conducted a retrospective international multi-center study including 63 pediatric patients with histologically confirmed PXAs. Neuroimaging data were reviewed, and molecular analyses were recorded with emphasis on BRAF V600E mutations and CDKN2A/B deletions. Treatment modalities and clinical outcomes, including progression-free and overall survival, were analyzed using statistical survival models. RESULTS: 73.3% of tumors were CNS WHO grade 2 and 26.7% were CNS WHO grade 3. The median age at diagnosis was 10.7 (IQR 6.9) years. CNS WHO grade 3 tumors were significantly larger at diagnosis, with a median volume of 87,920 mm³ compared with 14,925 mm³ for grade 2 tumors (p = 0.03). BRAF V600E mutations were identified in 78% of cases and CDKN2A/B deletions in 93.5%. On MRI, PXAs typically appeared well-defined, with cortical and leptomeningeal contact (86.0% and 56.4%, respectively), frequent cysts (59.6%), and intermediate diffusivity (mean ADC 1005 × 10−6 mm2/s). CT imaging showed most tumors were isointense to gray matter (52.0%), with hydrocephalus more common in grade 3 tumors (71.4% vs. 26.3%, p = 0.07). Gross total resection was achieved in 73.7% of cases and was associated with improved progression-free survival, independent of age at diagnosis and tumor grade (HR = 0.39; 95% CI, 0.16–0.96; p = 0.041). Larger tumor volume correlated with poorer survival outcomes (HR = 3.47; 95% CI, 0.83–14.4; p = 0.087), also independent of tumor grade and age at diagnosis. Tumor recurrence occurred in 44.8% of patients at three years. The estimated three-year overall survival rate was 94.7%. CONCLUSIONS: Pediatric PXAs exhibit distinct neuroimaging and molecular features correlating with prognosis. Integrated evaluation of radiological and clinical features is critical to improve risk stratification and guide personalized therapeutic strategies in this rare tumor population.
Related
- Incremental Anatomical Value of CT Angiography Beyond Echocardiography in the Preoperative Assessment of Pediatric Conotruncal Anomalies: A Surgical Correlation Study
- Temporal drift, calibration monitoring, and adaptive updating of an IVIG resistance prediction model in Kawasaki disease: a multicenter pediatric cohort study
- Organ-Specific Clinical Patterns of Pediatric Cystic Echinococcosis: A Multicenter Cohort from an Endemic Region
- Development and Validation of Temperature Trajectory-Based Phenotypes in Pediatric Sepsis: A Multicenter Retrospective Cohort Study, 2012–2018
- Differences in practices for surgical site infection prevention in neonatal surgery between neonatologists and pediatric surgeons: a multicenter survey in Japan
BibTeX
@article{chhatwal2026pediatric,
title = {{Pediatric Pleomorphic Xanthoastrocytomas: A Multicenter Neuroradiologic and Clinical Correlation Study}},
author = {Karanjot Chhatwal and Thomas Hargreaves and Francesco Pacchiano and Cesar Alves and Vanessa Rameh and Adam Ezra Goldman-Yassen and Aashim Bhatia and Alok Jaju and Kristen W. Yeom and Jason Nixon and Eleonora Piccirilli and Kiran Hilal and Apoorva Kotha and Alka Gupta and Hannah Morley and Kristian Aquilina and Thomas S. Jacques and Ashirwad Merve and Patricia Svrckova and Felice D’Arco and Pritika Gaur and Ulrike Löbel and Utsav Ganguly and Sniya Sudhakar and Asthik Biswas and Kshitij Mankad},
journal = {AJNR: American Journal of Neuroradiology},
year = {2026},
month = nov,
doi = {10.3174/ajnr.a9340},
url = {https://doi.org/10.3174/ajnr.a9340},
note = {CC BY},
}
RIS
TY - JOUR
TI - Pediatric Pleomorphic Xanthoastrocytomas: A Multicenter Neuroradiologic and Clinical Correlation Study
AU - Karanjot Chhatwal
AU - Thomas Hargreaves
AU - Francesco Pacchiano
AU - Cesar Alves
AU - Vanessa Rameh
AU - Adam Ezra Goldman-Yassen
AU - Aashim Bhatia
AU - Alok Jaju
AU - Kristen W. Yeom
AU - Jason Nixon
AU - Eleonora Piccirilli
AU - Kiran Hilal
AU - Apoorva Kotha
AU - Alka Gupta
AU - Hannah Morley
AU - Kristian Aquilina
AU - Thomas S. Jacques
AU - Ashirwad Merve
AU - Patricia Svrckova
AU - Felice D’Arco
AU - Pritika Gaur
AU - Ulrike Löbel
AU - Utsav Ganguly
AU - Sniya Sudhakar
AU - Asthik Biswas
AU - Kshitij Mankad
PY - 2026
DA - 2026/11/06
JO - AJNR: American Journal of Neuroradiology
DO - 10.3174/ajnr.a9340
UR - https://doi.org/10.3174/ajnr.a9340
AB - BACKGROUND AND PURPOSE: Pleomorphic Xanthoastrocytomas (PXAs) are rare pediatric brain tumors accounting for approximately 1% of primary brain neoplasms in children and young adults. Despite recent imaging and molecular advances, gaps remain in our understanding of their diverse imaging characteristics and clinical outcomes. This study provides a large international, multi-institutional analysis of pediatric PXAs focusing on neuroimaging and clinical outcomes. MATERIALS AND METHODS: We conducted a retrospective international multi-center study including 63 pediatric patients with histologically confirmed PXAs. Neuroimaging data were reviewed, and molecular analyses were recorded with emphasis on BRAF V600E mutations and CDKN2A/B deletions. Treatment modalities and clinical outcomes, including progression-free and overall survival, were analyzed using statistical survival models. RESULTS: 73.3% of tumors were CNS WHO grade 2 and 26.7% were CNS WHO grade 3. The median age at diagnosis was 10.7 (IQR 6.9) years. CNS WHO grade 3 tumors were significantly larger at diagnosis, with a median volume of 87,920 mm³ compared with 14,925 mm³ for grade 2 tumors (p = 0.03). BRAF V600E mutations were identified in 78% of cases and CDKN2A/B deletions in 93.5%. On MRI, PXAs typically appeared well-defined, with cortical and leptomeningeal contact (86.0% and 56.4%, respectively), frequent cysts (59.6%), and intermediate diffusivity (mean ADC 1005 × 10−6 mm2/s). CT imaging showed most tumors were isointense to gray matter (52.0%), with hydrocephalus more common in grade 3 tumors (71.4% vs. 26.3%, p = 0.07). Gross total resection was achieved in 73.7% of cases and was associated with improved progression-free survival, independent of age at diagnosis and tumor grade (HR = 0.39; 95% CI, 0.16–0.96; p = 0.041). Larger tumor volume correlated with poorer survival outcomes (HR = 3.47; 95% CI, 0.83–14.4; p = 0.087), also independent of tumor grade and age at diagnosis. Tumor recurrence occurred in 44.8% of patients at three years. The estimated three-year overall survival rate was 94.7%. CONCLUSIONS: Pediatric PXAs exhibit distinct neuroimaging and molecular features correlating with prognosis. Integrated evaluation of radiological and clinical features is critical to improve risk stratification and guide personalized therapeutic strategies in this rare tumor population.
SN - 0195-6108
SN - 1936-959X
ER -