Optic Nerve Hemangioblastoma: Review

Darbari, Shaurya; Meena, Rajesh Kumar; Sawarkar, Dattaraj; Doddamani, Ramesh

doi:10.1016/j.wneu.2019.04.224

Cited by 14 publications

(8 citation statements)

References 16 publications

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“…Retrobulbar hemangioblastomas occur in the intraorbital, intracanalicular, intracranial presellar, or intracranial suprasellar segments of the optic nerve. 6,27 Surgical approach depends on which corridor the tumor is located in and its relationship to the optic nerve. Those in the sellar vicinity or located on the medial aspect of the optic nerve provide an environment for transsphenoidal or an extended transsphenoidal approach.…”

Section: Discussionmentioning

confidence: 99%

“…In accordance to management of most VHL-associated hemangioblastomas, surgical resection is reserved for symptomatic or rapidly progressing lesions; however, there is no consensus on the optimal management of these retrobulbar lesions. 9,[17][18][19][20][21][22][23][24][25][26][27][28] Here, we present 18 patients with VHL-associated retrobulbar hemangioblastomas and their clinical course as well as their management.…”

Section: Discussionmentioning

confidence: 99%

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Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease: Clinical Course and Management

et al. 2021

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BACKGROUND Retrobulbar hemangioblastomas involving the optic apparatus in patients with von Hippel-Lindau disease (VHL) are rare, with only 25 reported cases in the literature. OBJECTIVE To analyze the natural history of retrobulbar hemangioblastomas in a large cohort of VHL patients in order to define presentation, progression, and management. METHODS Clinical history and imaging of 250 patients with VHL in an ongoing natural history trial and 1774 patients in a neurosurgical protocol were reviewed. The clinical course, magnetic resonance images, treatment, and outcomes were reviewed for all included patients. RESULTS A total of 18 patients with retrobulbar hemangioblastoma on surveillance magnetic resonance imaging met the inclusion criteria for this study. Of the 17 for whom clinical information was available, 10 patients presented with symptoms related to the hemangioblastoma, and 7 were asymptomatic. The mean tumor volume was larger for symptomatic (810.6 ± 545.5 mm3) compared to asymptomatic patients (307.6 ± 245.5 mm3; P < .05). A total of 5 of the symptomatic patients were treated surgically and all experienced improvement in their symptoms. All 3 symptomatic patients that did not undergo intervention had continued symptom progression. Long-term serial imaging on asymptomatic patients showed that these tumors can remain radiographically stable and asymptomatic for extended periods of time (101.43 ± 71 mo). CONCLUSION This study suggests that retrobulbar hemangioblastomas may remain stable and clinically asymptomatic for long durations. Recent growth and larger tumor volume were associated with symptom occurrence. Surgical treatment of symptomatic retrobulbar hemangioblastomas can be safe and may reverse the associated symptoms.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease: Clinical Course and Management

et al. 2021

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“…Approximately 30% of HGBs are associated with von Hippel-Lindau (VHL) disease and are often multifocal, situated in the spinal cord, cerebellum and brainstem (1,5,6). HGBs in the sellar region or suprasellar region are relatively uncommon, and optic nerve HGBs are extremely rare, with approximately 40 reports in the literature, including this case (7)(8)(9).…”

Section: Introductionmentioning

confidence: 93%

Central Nervous System Hemangioblastoma in a Pediatric Patient Associated With Von Hippel-Lindau Disease: A Case Report and Literature Review

Yang

Wang

et al. 2021

Front. Oncol.

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BackgroundHemangioblastoma is a benign tumor of the central nervous system and may appear as a component of von Hippel-Lindau (VHL) disease. At present, approximately 40 cases of optic nerve HGBs have been reported in the literature. VHL disease is a rare autosomal-dominant inherited cancer syndrome with different phenotypes caused by variants in the VHL gene. Herein, the authors describe a case of a pediatric patient with VHL disease and with optic nerve HGB, a rare phenotypic expression. The purpose of this study was to explore the genotype-phenotype, clinical features, treatment and follow-up of VHL-associated hemangioblastomas in pediatric patients.Case DescriptionA 12-year-old boy presented with vision loss, headache and dizziness at our hospital. Magnetic resonance imaging (MRI) revealed a large (19.8 mm*18.5 mm*23.5 mm) irregular mass located in the suprasellar region. The mass was successfully removed after craniotomy and microsurgical treatment. The pathological diagnosis was left optic nerve HGB. Genetic analyses showed p.Pro86Leu (c. 257C>T) heterozygous missense mutations in the VHL gene.ConclusionThis is the first reported pediatric case of VHL-associated optic nerve HGB. The genotype-phenotype correlation of VHL disease may provide new evidences for predicting tumor penetrance and survival. Gross tumor resection combined with stereotactic radiosurgery might be the most beneficial treatment.

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“…Optic nerve hemangioblastoma (ONH) is an exceedingly rare type of hemangioblastoma derived from within the optic nerve that invades or compresses the normal tissue, resulting in severe visual dysfunction 1 . It is an uncommonly encountered disorder that has only been reported in < 40 cases worldwide thus far.…”

Section: Introductionmentioning

confidence: 99%

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

Liu

Yang²,

Chen³

et al. 2023

Preprint

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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.

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Optic Nerve Hemangioblastoma: Review

Cited by 14 publications

References 16 publications

Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease: Clinical Course and Management

Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease: Clinical Course and Management

Central Nervous System Hemangioblastoma in a Pediatric Patient Associated With Von Hippel-Lindau Disease: A Case Report and Literature Review

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

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