Hemangioblastoma of the optic nerve producing bilateral optic tract edema in a patient with von Hippel-Lindau disease

Staub, Blake N.; Livingston, Andrew D.; Chévez-Barrios, Patricia; Baskin, David S.

doi:10.4103/2152-7806.128430

Cited by 14 publications

(15 citation statements)

References 14 publications

(26 reference statements)

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“…While most of these lesions are cerebral in location, a cranial nerve origin of these tumors is infrequent (8). Only 22 cases of HBL (including our case) arising from the optic nerve have been described, 18 of which are in the English literature and 4 reports are in Japanese (9). HBLs arising from the sella, stalk and hypothalamus has also been reported but are excluded from this review, which attempts to focus primarily on visual outcomes of pure ONHs.…”

Section: █ Discussionmentioning

confidence: 94%

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Optic nerve hemangioblastomas – a review of visual outcomes

Turel

Kucharczyk²,

Gentili³

2016

Turkish Neurosurgery

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ABSTRACThalos around objects as well as headaches. His visual acuity was 20/60 in the left eye and 20/20 in the right eye and visual field testing revealed concentric narrowing. Fundoscopic examination revealed left primary optic atrophy. Endocrine examination was normal.The magnetic resonance imaging (MRI) ( Figure 1A-D) showed a 1.8 cm extra-axial, well-defined, slightly lobulated lesion adjacent to the left anterior clinoid process and planum sphenoidale, compressing the optic nerve and the chiasm. The anterior cerebral artery (A1) was abutting the posterior surface of the tumor but was not encased. Most of the tumor was hypointense on T1-weighted images, enhanced uniformly, and showed heterogeneous intensity on T2-weighted images. There was a small cystic component to the tumor postero-inferiorly. The tumor did not have an obvious dural attachment and a dural-tail could not be identified. It was clearly separate from the pituitary gland and pituitary stalk. There was compression of the left optic nerve with no clear plane of separation. Likewise there was edema along the optic tracts bilaterally, more so on the left side. The preoperative █ INTRODUCTION H emangioblastomas (HBLs) are benign vascular tumors of the central nervous system accounting for approximately 2% of all intracranial neoplasms (3). They are often associated with von Hippel-Lindau (VHL) disease (1). Only 22 cases of optic nerve hemangioblastoma (ONH) have been reported in the literature. They often mimic meningiomas or optic nerve gliomas and are frequently misdiagnosed preoperatively. There has been only one other case of a HBL of the optic nerve producing optic tract oedema (9). We describe a case of an ONH without VHL, mimicking a meningioma preoperatively with optic tract edema. We review the visual outcomes of these patients as they have significant clinical implications. Such a review has not been done before in the literature. █ CASE REPORTA 67-year-old man presented with a 1-year history of episodic blurring of vision in the left eye. He also complained of seeing Supratentorial hemangioblastomas (HBLs) are exceedingly uncommon. They account for less than 5% of all central nervous system HBLs. The commonest location is the cerebral cortex. Rarely, they can involve the visual pathway. Most of the reports have focused on the differential diagnosis, since depending on the location they can often resemble optic nerve gliomas or meningiomas of the optic nerve sheath. In this study, we describe a 67-year-old man with progressive diminution of vision in the left eye with an optic nerve hemangioblastoma. There was no history of von Hippel-Lindau disease. We discuss the diagnostic challenges, and review the visual presentation and visual outcomes in these patients. This report, we believe, may add further clarity in counseling patients with an optic nerve lesion and the factors in surgical decision-making.

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Section: █ Discussionmentioning

confidence: 94%

“…They often mimic meningiomas or optic nerve gliomas and are frequently misdiagnosed preoperatively. There has been only one other case of a HBL of the optic nerve producing optic tract oedema (9). We describe a case of an ONH without VHL, mimicking a meningioma preoperatively with optic tract edema.…”

mentioning

confidence: 99%

Optic nerve hemangioblastomas – a review of visual outcomes

Turel

Kucharczyk²,

Gentili³

2016

Turkish Neurosurgery

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“…Vision loss in VHL may also be due to retinal lesions, but neuro-ophthalmological evaluation may raise suspicion for optic nerve haemangioblastoma. 6 As in our patient, contrast enhanced MRI of the orbit and the brain is helpful in these cases.…”

Section: Commentsmentioning

confidence: 52%

“…Ipsilateral optic nerve haemangiblastoma produced optic tract oedema, which resolved after resection of the prechiasmal tumour. 6 The preoperative differential diagnosis for this tumour based on imaging studies has included glioma, meningioma, and neurilemoma (''neurinoma''). Unlike gliomas, haemangioblastomas enhance brightly with gadolinium as in our case, allowing differentiation of these tumours.…”

Section: Commentsmentioning

confidence: 99%

Bilateral Optic Nerve Head Angiomas and Retrobulbar Haemangioblastomas in von Hippel-Lindau Disease

Fard

Hassanpoor

Parsa

2014

Neuro-Ophthalmology

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A 39-year-old man presented with progressive visual loss in both eyes. His past medical history was remarkable for biopsy-proven bilateral adrenal phaeochromocytomas. Ophthalmoscopy revealed an optic nerve head angioma and macular lipid exudates in the right eye and a regressed optic nerve head angioma in the left eye. A fluorescein angiogram revealed a lobulated pattern of leakage and venous staining from the optic nerve head angioma. Brain and orbital magnetic resonance imaging showed haemangioblastomas of the right intraorbital and left paraclinoid optic nerves and in the left cerebellopontine angle area. These findings were consistent with a clinical diagnosis of von Hippel-Lindau disease.

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“…Until now, 20 cases of optic nerve hemangioblastoma including this present case have been reported in the English literature ( 29 – 40 ). Fourteen of the 20 cases (70%) were associated with VHL.…”

Section: Discussionmentioning

confidence: 88%

VHL-Associated Optic Nerve Hemangioblastoma Treated with Stereotactic Radiosurgery

Kanno

Osano²,

Shinonaga

2018

jkcvhl

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Central nervous system hemangioblastomas are generally restricted to the cerebellum, spinal cord, and brainstem. Supratentorial hemangioblastomas are uncommon, and optic nerve hemangioblastomas are extremely rare, with fewer than 25 reports including this case. In this report, we present the case of a 36-year-old woman with von Hippel–Lindau (VHL) disease who presented with progressive diminution of vision in the left eye due to a retrobulbar optic nerve hemangioblastoma. The patient had a history of cerebellar/spinal hemangioblastomas and pancreatic cysts, and her father and brother were patients with VHL disease. Gadolinium-enhanced magnetic resonance imaging showed intraorbital retrobulbar–enhanced mass on the left optic nerve. The optic nerve hemangioblastoma was treated with fractionated stereotactic radiosurgery using Novalis. Eighteen months after the stereotactic radiosurgery, the tumor volume decreased although the patient lost vision. This report presents an extremely rare case of optic nerve hemangioblastoma, which is the first case treated with stereotactic radiosurgery.

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Hemangioblastoma of the optic nerve producing bilateral optic tract edema in a patient with von Hippel-Lindau disease

Cited by 14 publications

References 14 publications

Optic nerve hemangioblastomas – a review of visual outcomes

Optic nerve hemangioblastomas – a review of visual outcomes

Bilateral Optic Nerve Head Angiomas and Retrobulbar Haemangioblastomas in von Hippel-Lindau Disease

VHL-Associated Optic Nerve Hemangioblastoma Treated with Stereotactic Radiosurgery

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