A potential VEGF-driven hypothesis of calvarial centripetal proliferation in cerebral proliferative angiopathy

Abstract Cerebral proliferative angiopathy (CPA) is a rare subtype of cerebral arteriovenous malformation, characterized by unique angiographic features and clinical presentations. Although the clinical and angiographic characteristics of CPA have been well described, their impact on the surrounding...

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Main Authors: Shigeta Miyake, Timo Krings, Tze Phei Kee, Thierry A. G. M. Huisman
Format: Article
Language:English
Published: Nature Portfolio 2025-01-01
Series:Scientific Reports
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Online Access:https://doi.org/10.1038/s41598-025-86525-7
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author Shigeta Miyake
Timo Krings
Tze Phei Kee
Thierry A. G. M. Huisman
author_facet Shigeta Miyake
Timo Krings
Tze Phei Kee
Thierry A. G. M. Huisman
author_sort Shigeta Miyake
collection DOAJ
description Abstract Cerebral proliferative angiopathy (CPA) is a rare subtype of cerebral arteriovenous malformation, characterized by unique angiographic features and clinical presentations. Although the clinical and angiographic characteristics of CPA have been well described, their impact on the surrounding tissues remains underexplored. Herein, we investigated the presence of calvarial thickening in patients with CPA, and discuss its potential pathogenesis. This retrospective multicenter cohort study enrolled 16 CPA patients from our institutions. Patients were identified by a search of the hospital medical records for key words related to “cerebral proliferative angiopathy.” Data on demographics, CPA characteristics, clinical symptoms, and calvarial thickening were collected and analyzed using CT and MRI. Statistical analyses were performed using t-tests and Fisher’s exact tests, with significance set at P < 0.05. Patients with CPA ranged from 4 to 69 years of age and underwent skull evaluation at a median age of 26.5 years. Calvarial thickening was present in 43.8% of cases, predominantly affecting the frontal bone, and extending bilaterally. A trend toward calvarial thickening was observed in patients with concomitant signs of cerebral venous congestion (p = 0.06), while no significant association with age, sex, or symptoms was found. Calvarial thickening displayed centripetal proliferation with trabecular formation extending from the inner table. This study identified Calvarial centripetal proliferation in a significant proportion of patients with CPA. We hypothesized that this may be related to elevated vascular endothelial growth factor levels due to the proangiogenic nature of CPA.
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spelling doaj-art-b7e63e69cd6d4c688fa96c76adc67eb02025-01-26T12:25:26ZengNature PortfolioScientific Reports2045-23222025-01-011511810.1038/s41598-025-86525-7A potential VEGF-driven hypothesis of calvarial centripetal proliferation in cerebral proliferative angiopathyShigeta Miyake0Timo Krings1Tze Phei Kee2Thierry A. G. M. Huisman3Department of Neurosurgery, Yokohama City University Graduate School of MedicineDivision of Neurointerventional Radiology, UMass Chan School of Medicine, Lahey Hospital, Beth Israel Lahey HealthUniversity Health Network and Toronto Western Hospital, University of TorontoEdward B. Singleton Department of Radiology, Texas Children’s Hospital, Baylor College of MedicineAbstract Cerebral proliferative angiopathy (CPA) is a rare subtype of cerebral arteriovenous malformation, characterized by unique angiographic features and clinical presentations. Although the clinical and angiographic characteristics of CPA have been well described, their impact on the surrounding tissues remains underexplored. Herein, we investigated the presence of calvarial thickening in patients with CPA, and discuss its potential pathogenesis. This retrospective multicenter cohort study enrolled 16 CPA patients from our institutions. Patients were identified by a search of the hospital medical records for key words related to “cerebral proliferative angiopathy.” Data on demographics, CPA characteristics, clinical symptoms, and calvarial thickening were collected and analyzed using CT and MRI. Statistical analyses were performed using t-tests and Fisher’s exact tests, with significance set at P < 0.05. Patients with CPA ranged from 4 to 69 years of age and underwent skull evaluation at a median age of 26.5 years. Calvarial thickening was present in 43.8% of cases, predominantly affecting the frontal bone, and extending bilaterally. A trend toward calvarial thickening was observed in patients with concomitant signs of cerebral venous congestion (p = 0.06), while no significant association with age, sex, or symptoms was found. Calvarial thickening displayed centripetal proliferation with trabecular formation extending from the inner table. This study identified Calvarial centripetal proliferation in a significant proportion of patients with CPA. We hypothesized that this may be related to elevated vascular endothelial growth factor levels due to the proangiogenic nature of CPA.https://doi.org/10.1038/s41598-025-86525-7Brain arteriovenous fistulaCerebral proliferative angiopathyCalvarial thickeningVEGF
spellingShingle Shigeta Miyake
Timo Krings
Tze Phei Kee
Thierry A. G. M. Huisman
A potential VEGF-driven hypothesis of calvarial centripetal proliferation in cerebral proliferative angiopathy
Scientific Reports
Brain arteriovenous fistula
Cerebral proliferative angiopathy
Calvarial thickening
VEGF
title A potential VEGF-driven hypothesis of calvarial centripetal proliferation in cerebral proliferative angiopathy
title_full A potential VEGF-driven hypothesis of calvarial centripetal proliferation in cerebral proliferative angiopathy
title_fullStr A potential VEGF-driven hypothesis of calvarial centripetal proliferation in cerebral proliferative angiopathy
title_full_unstemmed A potential VEGF-driven hypothesis of calvarial centripetal proliferation in cerebral proliferative angiopathy
title_short A potential VEGF-driven hypothesis of calvarial centripetal proliferation in cerebral proliferative angiopathy
title_sort potential vegf driven hypothesis of calvarial centripetal proliferation in cerebral proliferative angiopathy
topic Brain arteriovenous fistula
Cerebral proliferative angiopathy
Calvarial thickening
VEGF
url https://doi.org/10.1038/s41598-025-86525-7
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