Unique Properties of Clinical Manifestation and Magnetic Resonance Imaging for Differential Diagnosis of Optic Nerve Hemangioblastoma

Abstract Background To investigate the unique properties of clinical manifestation and radiological imaging for differential diagnosis of optic nerve hemangioblastoma (ONH) from adult optic nerve glioma (ONG) prior to surgical resection. Methods ONH and adult ONG patients were recruited from 2012 to 2022. Results A total of seven ONH patients (8 eyes) and 23 adult ONG patients (24 eyes) were assessed. The median onset age of ONH patients (39 years) was older than that of adult ONG patients (27 years) (p = 0.007). 42.9% of ONH patients were closely associated with the diagnosis of VHL syndrome. Notably in MRI, the retrobulbar hemangioblastomas in all ONH patients were primarily intraorbital (100%) and canalicular (87.5%), and anterior intracranial (12.5%), especially concentrated in the orbital apex, with little posterior optic pathway involvement. Nearly all affected parts of optic nerve in the ONH group (6/8, 75.0%) presented with circumscribed cyst-solid components, characterized by cystic lesions in the front (peritumoral edema) and solid tumors in the back. The tumor presented typically with hypo- or isointense on the non-contrast T1-weighted images, and hyper- or isointense on the T2-weighted images (hyperintense pretumor cystic lesion, slight hyper-/isointense solid lesion), and the solid part appeared with significant heterogeneous enhancement. Conclusion Optic nerve tumors in those with older ages (> 30 years) or those diagnosed with VHL syndrome are more likely to be indicative of ONH. In the absence of associated VHL syndrome, MRI images presenting with circumscribed cyst-solid components (peritumoral edema and posterior solid tumor) especially concentrated in the orbital apex, with a heterogeneous enhancement of the solid part in the postcontrast study, is the unique property of ONH for differentiation.