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2000
Volume 21, Issue 1
  • ISSN: 1573-4056
  • E-ISSN: 1875-6603
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Abstract

Background

Optic nerve hemangioblastoma (ONH) is a rare benign tumor. It can be sporadic or associated with Von-Hippel Lindau (VHL) syndrome. Magnetic resonance imaging (MRI) is the most commonly used diagnostic technique for the tumor. However, an accurate diagnosis can be challenging due to the rarity of ONH and its similarity to glioma and meningioma.

Case Report

A 49-year-old female experienced progressive vision loss for ten years in the right eye, accompanied by proptosis over two years. The ophthalmological examination found her visual acuity of the right eye to have no light perception. Optical coherence tomography showed decreased thickness of the right retinal ganglion cell layer. MRI revealed an oval solid mass within the right retrobulbar space, with isointensity on T-weighted (TWI) imaging and heterogeneous hyperintensity on T-weighted imaging (TWI). Heterogeneous enhancement was found on gadolinium-enhanced TWI and dynamic contrast-enhanced MRI. At internal and marginal areas of the mass, multiple flow voids were observed on various sequences, especially on TWI. Furthermore, the superior, inferior, medial, and lateral rectus muscles of the right eye distinctly atrophied, showing a lower signal intensity on TWI and less apparent enhancement than the left normal ones. Postoperative pathological diagnosis was hemangioblastoma of the right optic nerve.

Conclusion

Hemangioblastoma should be considered as a differential diagnosis for the space-occupying mass of the optic nerve if there is the presence of flow voids, vivid enhancement, and absence of a dural attachment, regardless of VHL syndrome. Of note, this is the first reported case to consider altered extraocular muscles as a potential point to prompt the diagnosis on MRI.

© 2025 The Author(s).
© 2025 The Author(s). Published by Bentham Science Publishers. This is an open access article published under CC BY 4.0 https://creativecommons.org/licenses/by/4.0/legalcode
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2025-09-04

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References

  1. ZywickeH. PalmerC.A. VaphiadesM.S. RileyK.O. Optic nerve hemangioblastoma: a case report.Case Rep. Pathol.201220121410.1155/2012/91540822953140
     [Google Scholar]
  2. TurelM.K. KucharczykW. GentiliF. Optic nerve hemangioblastomas? a review of visual outcomes.Turk Neurosurg.201727582783127509455
     [Google Scholar]
  3. McGrathL.A. MudharH.S. SalviS.M. Optic nerve haemangioblastoma: signs of chronicity.Ocul. Oncol. Pathol.20184637037410.1159/00048686330574489
     [Google Scholar]
  4. GinzburgB.M. MontaneraW.J. TyndelF.J. GriesmanJ.A. McLennanM.K. TerBruggeK.G. WillinskyR.A. Diagnosis of von Hippel-Lindau disease in a patient with blindness resulting from bilateral optic nerve hemangioblastomas.AJR Am. J. Roentgenol.1992159240340510.2214/ajr.159.2.16323661632366
     [Google Scholar]
  5. MiyagamiM. MiyagiA. KidoG. SatohK. TsubokawaT. [The two familial occurrence of von Hippel-Lindau disease].No To Shinkei1994467683689[The two familial occurrence of von Hippel-Lindau disease].7946625
     [Google Scholar]
  6. RubioA. MeyersS.P. PowersJ.M. NelsonC.N. de PappE.W. Hemangioblastoma of the optic nerve.Hum. Pathol.199425111249125110.1016/0046‑8177(94)90044‑27959672
     [Google Scholar]
  7. WirtschafterJ. BalcerL.J. GalettaS.L. CurtisM. MaguireA. JudyK. von Hippel-Lindau disease manifesting as a chiasmal syndrome.Surv. Ophthalmol.199539430230610.1016/S0039‑6257(05)80107‑97725229
     [Google Scholar]
  8. KerrD.J. ScheithauerB.W. MillerG.M. EbersoldM.J. McPheeT.J. Hemangioblastoma of the optic nerve: case report.Neurosurgery199536357358110.1227/00006123‑199503000‑000177753357
     [Google Scholar]
  9. RailaF.A. ZimmermanJ. AzordeganP. FratkinJ. ParentA.D. Successful surgical removal of an asymptomatic optic nerve hemangioblastoma in von Hippel-Lindau disease.J. Neuroimaging199771485010.1111/jon199771489038433
     [Google Scholar]
  10. KouriJ.G. ChenM.Y. WatsonJ.C. OldfieldE.H. Resection of suprasellar tumors by using a modified transsphenoidal approach.J. Neurosurg.20009261028103510.3171/jns.2000.92.6.102810839266
     [Google Scholar]
  11. KatoK. UtsunomiyaA. UenoharaH. SuzukiS. TakahashiN. SuzukiH. NishinoA. NishimuraS. SakuraiY. [Hemangioblastoma of the optic nerve growing like a dumbbell through the optic canal: case report].No To Shinkei2004568711716[Hemangioblastoma of the optic nerve growing like a dumbbell through the optic canal: case report].15508741
     [Google Scholar]
  12. Fons MartínezM.R. España GregoriE. Aviñó MartínezJ.A. Hernández PardinesF. [An optic nerve tumor in von Hippel-Lindau disease, masquerading as a retinal hemangioma].Arch. Soc. Esp. Oftalmol.2006815293296[An optic nerve tumor in von Hippel-Lindau disease, masquerading as a retinal hemangioma].16752322
     [Google Scholar]
  13. HigashidaT. SakataK. KannoH. KawasakiT. TanabeY. YamamotoI. Hemangioblastoma of the optic nerve--case report.Neurol. Med. Chir. (Tokyo)200747521521810.2176/nmc.47.21517527048
     [Google Scholar]
  14. BaggenstosM. ChewE. ButmanJ.A. OldfieldE.H. LonserR.R. Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss.J. Neurosurg.2008109231331710.3171/JNS/2008/109/8/031318671645
     [Google Scholar]
  15. BarrettR. MeyerD. BoulosA. EamesF. Torres-MoraJ. Optic nerve hemangioblastoma.Ophthalmology20081151120952095.e210.1016/j.ophtha.2008.06.00319068378
     [Google Scholar]
  16. MeyerleC.B. DahrS.S. WetjenN.M. JirawuthiworavongG.V. ButmanJ.A. LonserR.R. OldfieldE. Rodriguez-ColemanH. WongW.T. ChewE.Y. Clinical course of retrobulbar hemangioblastomas in von Hippel-Lindau disease.Ophthalmology200811581382138910.1016/j.ophtha.2008.01.02718395800
     [Google Scholar]
  17. PrabhuK. DanielR.T. ChackoG. ChackoA.G. Optic nerve haemangioblastoma mimicking a planum sphenoidale meningioma.Br. J. Neurosurg.200923556156310.1080/0268869090296596419718547
     [Google Scholar]
  18. ShimaHO EguchiY AndoA SetoguchiM OhtaH Optic nerve hemangioblastoma removed after embolization of the ophthalmic artery: a case report.Jpn J Neurosurg2011202133e8
     [Google Scholar]
  19. FardM.A. HassanpoorN. ParsaR. Bilateral optic nerve head angiomas and retrobulbar haemangioblastomas in von Hippel-Lindau disease.Neuroophthalmology201438525425610.3109/01658107.2014.94462227928308
     [Google Scholar]
  20. StaubB. LivingstonA. Chévez-BarriosP. BaskinD. Hemangioblastoma of the optic nerve producing bilateral optic tract edema in a patient with von Hippel-Lindau disease.Surg. Neurol. Int.2014513310.4103/2152‑7806.12843024778921
     [Google Scholar]
  21. KannoH. OsanoS. ShinonagaM. VHL-associated optic nerve hemangioblastoma treated with stereotactic radiosurgery.J. Kidney Cancer VHL2018521610.15586/jkcvhl.2018.10429911000
     [Google Scholar]
  22. DarbariS. MeenaR.K. SawarkarD. DoddamaniR.S. Optic nerve hemangioblastoma.World Neurosurg.201912821121510.1016/j.wneu.2019.04.22431054346
     [Google Scholar]
  23. BorattoS.D.F. CardosoP.A.S. PriolliD.G. BotelhoR.V. GoldenbergA. BiancoB. WaisbergJ. von Hippel-Lindau syndrome: genetic study of case with a rare pathogenic variant with optic nerve hemangioblastoma, a rare phenotypic expression.Front. Oncol.20201013910.3389/fonc.2020.0013932117777
     [Google Scholar]
  24. XuS. LiQ. BianB. ZhouH. LiD. Optic nerve Hemangioblastoma with bilateral frontal lobe Oedema: a case report.BMC Ophthalmol.202020143710.1186/s12886‑020‑01706‑433143685
     [Google Scholar]
  25. AlvarezR. MastorakosP. HoganE. ScottG. LonserR.R. WileyH.E. ChewE.Y. ChittiboinaP. Retrobulbar hemangioblastomas in von Hippel-Lindau disease: clinical course and management.Neurosurgery20218851012102010.1093/neuros/nyaa56533442737
     [Google Scholar]
  26. DuanM. YangL. KangJ. WangR. YouH. FengM. Neuroimaging features of optic nerve hemangioblastoma identified by conventional and advanced magnetic resonance techniques: a case report and literature review.Front. Oncol.20211176369610.3389/fonc.2021.76369634868983
     [Google Scholar]
  27. YangB. LiZ. WangY. ZhangC. ZhangZ. ZhangX. Central nervous system hemangioblastoma in a pediatric patient associated with von Hippel-Lindau disease: a case report and literature review.Front. Oncol.20211168302110.3389/fonc.2021.68302134109129
     [Google Scholar]
  28. CapitanioJ.F. MazzaE. MottaM. MortiniP. ReniM. Mechanisms, indications and results of salvage systemic therapy for sporadic and von Hippel–Lindau related hemangioblastomas of the central nervous system.Crit. Rev. Oncol. Hematol.2013861698410.1016/j.critrevonc.2012.10.00123148943
     [Google Scholar]
  29. Vicente LacerdaR.A. Teixeira JúniorA.G. Sauaia FilhoE.N. de Macêdo FilhoL.J.M. AntônioA.S. CabralJ.A. Ramos JúniorF. Valença JúniorJ.T. Dural-based frontal lobe hemangioblastoma.World Neurosurg.2019129182310.1016/j.wneu.2019.05.17531150863
     [Google Scholar]
  30. RojianiA.M. OwenD.A. BerryK. WoodhurstB. ScudamoreC.H. ScudamoreC.H. ErbS. Hepatic Hemangioblastoma.Am. J. Surg. Pathol.1991151818610.1097/00000478‑199101000‑000101898683
     [Google Scholar]
  31. InS. MiyagiJ. KojhoN. KuramotoS. UeharaM. Intraorbital optic nerve hemangioblastoma with von Hippel-Lindau disease.J. Neurosurg.198256342642910.3171/jns.1982.56.3.04267199079
     [Google Scholar]
  32. McGrathL.A. MudharH.S. SalviS.M. Hemangioblastoma of the optic nerve.Surv. Ophthalmol.201964217518410.1016/j.survophthal.2018.10.00230321570
     [Google Scholar]
  33. LonserR.R. GlennG.M. WaltherM. ChewE.Y. LibuttiS.K. LinehanW.M. OldfieldE.H. von Hippel-Lindau disease.Lancet200336193742059206710.1016/S0140‑6736(03)13643‑412814730
     [Google Scholar]
  34. BaggenstosM.A. ButmanJ.A. OldfieldE.H. LonserR.R. Role of edema in peritumoral cyst formation.Neurosurg. Focus20072251710.3171/foc.2007.22.5.1017613240
     [Google Scholar]
  35. PierpaoliC. JezzardP. BasserP.J. BarnettA. Di ChiroG. Diffusion tensor MR imaging of the human brain.Radiology1996201363764810.1148/radiology.201.3.89392098939209
     [Google Scholar]
  36. BasserP.J. PierpaoliC. A simplified method to measure the diffusion tensor from seven MR images.Magn. Reson. Med.199839692893410.1002/mrm.19103906109621916
     [Google Scholar]
  37. WitwerB.P. MoftakharR. HasanK.M. DeshmukhP. HaughtonV. FieldA. ArfanakisK. NoyesJ. MoritzC.H. MeyerandM.E. RowleyH.A. AlexanderA.L. BadieB. Diffusion-tensor imaging of white matter tracts in patients with cerebral neoplasm.J. Neurosurg.200297356857510.3171/jns.2002.97.3.056812296640
     [Google Scholar]
  38. HandaJ. NakazawaT. WatanabeK. SuzukiF. Haemangioblastoma with multiple dural arterial supply.Acta Neurochir. (Wien)1984733-419319910.1007/BF014008526542743
     [Google Scholar]
  39. SkucasJ. BrinkerR.A. Cerebellar hemangioblastoma with a tentorial artery supply: Report of a case.Neuroradiology19713211311510.1007/BF003399065170810
     [Google Scholar]
  40. KeeneK.R. NottingI.C. VerschuurenJ.J.G.M. VoermansN. de KeizerR.O.B. BeenakkerJ.W.M. TannemaatM.R. KanH.E. Eye muscle MRI in myasthenia gravis and other neuromuscular disorders.J. Neuromuscul. Dis.202310586988310.3233/JND‑23002337182896
     [Google Scholar]
  41. GlassL.R.D. CanollP. LignelliA. LigonA.H. KazimM. Optic nerve glioma: case series with review of clinical, radiologic, molecular, and histopathologic characteristics.Ophthal. Plast. Reconstr. Surg.201430537237610.1097/IOP.000000000000010624762952
     [Google Scholar]
  42. FarazdaghiM.K. KatowitzW.R. AveryR.A. Current treatment of optic nerve gliomas.Curr. Opin. Ophthalmol.201930535636310.1097/ICU.000000000000058731246635
     [Google Scholar]
  43. RenT.T. MaJ.M. [Clinical diagnosis and treatment of optic nerve glioma: a review].Zhonghua Yan Ke Za Zhi2023595415419[Clinical diagnosis and treatment of optic nerve glioma: a review].37151013
     [Google Scholar]
  44. ShapeyJ. Danesh-MeyerH.V. KayeA.H. Diagnosis and management of optic nerve glioma.J. Clin. Neurosci.201118121585159110.1016/j.jocn.2011.09.00322071462
     [Google Scholar]
  45. NairA.G. PathakR.S. IyerV.R. GandhiR.A. Optic nerve glioma: an update.Int. Ophthalmol.2014344999100510.1007/s10792‑014‑9942‑824736941
     [Google Scholar]
  46. TailorT.D. GuptaD. DalleyR.W. KeeneC.D. AnzaiY. Orbital neoplasms in adults: clinical, radiologic, and pathologic review.Radiographics20133361739175810.1148/rg.33613550224108560
     [Google Scholar]
  47. BeckerM. MastersonK. DelavelleJ. ViallonM. VargasM.I. BeckerC.D. Imaging of the optic nerve.Eur. J. Radiol.201074229931310.1016/j.ejrad.2009.09.02920413240
     [Google Scholar]
  48. GaudinoS. MartucciM. RussoR. ViscontiE. GangemiE. D’ArgentoF. VerdolottiT. LauriolaL. ColosimoC. MR imaging of brain pilocytic astrocytoma: beyond the stereotype of benign astrocytoma.Childs Nerv. Syst.2017331355410.1007/s00381‑016‑3262‑427757570
     [Google Scholar]
  49. OrtizO. SchochetS.S. KotzanJ.M. KostickD. Radiologic-pathologic correlation: meningioma of the optic nerve sheath.AJNR Am. J. Neuroradiol.19961759019068733965
     [Google Scholar]
  50. SaeedP. RootmanJ. NugentR.A. WhiteV.A. MackenzieI.R. KoornneefL. Optic nerve sheath meningiomas.Ophthalmology2003110102019203010.1016/S0161‑6420(03)00787‑514522782
     [Google Scholar]
  51. DouglasV.P. DouglasK.A.A. CestariD.M. Optic nerve sheath meningioma.Curr. Opin. Ophthalmol.202031645546110.1097/ICU.000000000000070033009076
     [Google Scholar]
  52. SibonyP.A. KraussH.R. KennerdellJ.S. MaroonJ.C. SlamovitsT.L. Optic nerve sheath meningiomas. Clinical manifestations.Ophthalmology198491111313132610.1016/S0161‑6420(84)34148‑36514296
     [Google Scholar]
  53. KanamallaU.S. The optic nerve tram-track sign.Radiology2003227371871910.1148/radiol.227301075812773677
     [Google Scholar]
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Diagnostic Challenges and Insights in Optic Nerve Hemangioblastoma using Magnetic Resonance Imaging: A Case Report
Curr. Med. Imaging 21, E15734056359483 (2025); https://doi.org/10.2174/0115734056359483250502063550
/content/journals/cmir/10.2174/0115734056359483250502063550
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  • Article Type:     Case Report
Keyword(s): Case report; Differential diagnosis; Hemangioblastoma; Heterogeneous hyperintensity; Magnetic resonance imaging; Optic nerve; Von-Hippel Lindau

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