Optic nerve hemangioblastoma mimicking optic pathway glioma: A case report and literature review

Optic nerve hemangioblastomas (ONH) are rare tumors frequently associated with Von Hippel-Lindau (VHL) syndrome. They may mimic optic pathway gliomas (OPG), posing diagnostic and surgical challenges. We report a case of a 36-year-old male presenting with progressive blurry vision in the right eye over three months. Initial examination revealed unilateral axial proptosis, a positive relative afferent pupillary defect, and optic disc swelling. Initial magnetic resonance imaging revealed a lobulated, enhancing lesion involving the intra-orbital segment of the right optic nerve, which was presumed to be an OPG. Over five years of surveillance, the tumor progressed, causing new-onset headaches, worsening proptosis, and mass effect. Surgical debulking was performed, revealing a hemangioblastoma histologically. ONH should be considered in the differential diagnosis of optic pathway tumors, particularly in cases with atypical presentation or progression. Surgical intervention may be necessary for definitive diagnosis and management, and an association with VHL must be considered. • Optic nerve hemangioblastomas (ONH) lead to profound visual impairment. • Diagnosing ONH could be challenging. • Surgical intervention is required for definitive diagnosis and management.