Neuroimaging Features of Optic Nerve Hemangioblastoma Identified by Conventional and Advanced Magnetic Resonance Techniques: A Case Report and Literature Review

Abstract

Optic nerve hemangioblastoma is a very rare benign tumor with only 39 reported cases by now. It appears to be hyperintense on T2-weighted images with a significant enhancement on contrast scans, which are similar to glioma and meningioma. Due to the lack of specificity in MRI manifestations, optic nerve hemangioblastoma is often misdiagnosed. To provide new insights into differential diagnosis of optic nerve hemangioblastoma, we report for the first time an optic nerve hemangioblastoma case employing advanced magnetic resonance techniques including diffusion-weighted imaging (DWI), apparent diffusion coefficient (ADC) maps, and magnetic resonance angiography (MRA). In addition, we have collected all reported optic nerve hemangioblastoma cases and reviewed their neuroimaging findings by MRI and angiography. Our results show that solid-type tumor is the dominant form of optic nerve hemangioblastoma and extensive edema is widely observed. These findings are surprisingly contrary to manifestations of cerebellar hemangioblastoma. Besides the structural features, quantitative indexes including ADC and relative cerebral blood volume (rCBV) ratio, which are significantly elevated in cerebellar hemangioblastoma, may also shed a light on the preoperative diagnosis of hemangioblastoma of optic nerve. Finally, we discuss the critical neuroimaging features in the differential diagnosis between optic nerve hemangioblastoma from optic pathway glioma and optic nerve sheath meningioma.

Imaging features of retinal hemangioblastoma: A case report

“…In addition, hemangioblastoma has different imaging features based on different sites of occurrence. Solid tumors are also the main form of optic nerve hemangioblastoma, but most of the lesions are surrounded by edema[ 16 ]. Large cysts with small nodules are characteristic of cerebellar and spinal hemangioblastomas, and are mainly associated with intratumoral and peritumoral flow void effects[ 17 ].…”

Supratentorial hemangioblastoma at the anterior skull base: A case report

“…In 2021, the WHO categorized HB as grade I “mesenchymal, non-meningothelial tumor.” HB accounts for approximately 2% of all intracranial tumors and 7%-12% of all posterior fossa tumors. HB is divided into sporadic and familial hereditary types, with the latter accounting for approximately 20% of HBs[ 1 ]. HB is often diagnosed between 35 years and 45 years of age, and men are more likely to be diagnosed than women[ 2 ].…”

“…If there was fresh bleeding from the tumor, T1WI and T2WI would display a hyperintense signal. It is associated with no or mild peritumoral edema[ 1 , 7 ].…”

“…Infratentorial and supratentorial HBs often differ from each other, as the former are primarily cystic, whereas the latter are cystic-solid mixed, with a few cystic components. The reason for this difference might be that the brain is believed to be less resistant to fluid diffusion than the cerebellum[ 1 , 7 ].…”

“…Hemangioblastoma (HB) is a rare tumor, comprising about 2% of all intracranial tumors[ 1 ]. HB occurs most commonly in the cerebellar hemispheres, followed by the brainstem and spinal cord, and is least common in the supratentorial region[ 2 ].…”