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Unique properties of clinical manifestation and magnetic resonance imaging for differential diagnosis of optic nerve hemangioblastoma

Eye volume 38, pages 3562–3568 (2024)Cite this article

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Abstract

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 was older than that of adult ONG patients (p = 0.007). There were 42.9% (3/7) of ONH patients closely associated with the diagnosis of Von Hippel-Lindau (VHL) syndrome. Notably in Magnetic Resonance Imaging (MRI), the retrobulbar hemangioblastomas in all ONH patients were primarily intraorbital (100%, 8/8), canalicular (87.5%, 7/8), and anterior intracranial (12.5%, 1/8), especially concentrated in the orbital apex, with little posterior optic pathway involvement. Nearly all affected parts of optic nerve in the ONH group (75.0%, 6/8) presented with circumscribed cystic-solid components, characterized by cystic lesions (peritumoral oedema) anteriorly and solid tumours posteriorly, with significant heterogeneous enhancement. Adult ONG lesions prior to extend from the anterior portion to the whole length of the optic nerve, with relatively innocent enlargement in the postcontrast study.

Conclusion

Optic nerve tumours in those with older ages at diagnosis (>30 years) or those diagnosed with VHL syndrome are more likely to be indicative of ONH. In the absence of associated VHL syndrome, a distinguishing MRI feature is the concentration of tumours in the orbital apex, characterized by circumscribed cystic-solid components and heterogeneous enhancement of the solid portion.

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Fig. 1: Orbital MRI images of ONH patients.
Fig. 2: Orbital MRI, fundus and pathological images of ONH patients.
Fig. 3: MRI Images of two adult ONG patients.

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Data availability

The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request.

References

  1. Darbari S, Meena RK, Sawarkar D, Doddamani RS. Optic Nerve Hemangioblastoma: review. World Neurosurg. 2019;128:211–5.

    Article  PubMed  Google Scholar 

  2. Staub B, Livingston A, Chévez-Barrios P, Baskin D. Hemangioblastoma of the optic nerve producing bilateral optic tract edema in a patient with von Hippel-Lindau disease. Surg Neurol Int. 2014;5:33.

    Article  PubMed  PubMed Central  Google Scholar 

  3. McGrath LA, Mudhar HS, Salvi SM. Optic nerve haemangioblastoma: signs of chronicity. Ocul Oncol Pathol. 2018;4:370–4.

    Article  PubMed  PubMed Central  Google Scholar 

  4. McGrath LA, Mudhar HS, Salvi SM. Hemangioblastoma of the optic nerve. Surv Ophthalmol. 2019;64:175–84.

    Article  PubMed  Google Scholar 

  5. Alvarez R, Mastorakos P, Hogan E, Scott G, Lonser RR, Wiley HE, et al. Retrobulbar hemangioblastomas in von hippel-lindau disease: clinical course and management. NEUROSURGERY. 2021;88:1012–20.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Chittiboina P, Lonser RR. Von Hippel-Lindau disease. Handb Clin Neurol. 2015;132:139–56.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Meyerle CB, Dahr SS, Wetjen NM, Jirawuthiworavong GV, Butman JA, Lonser RR, et al. Clinical course of retrobulbar hemangioblastomas in von Hippel-Lindau disease. OPHTHALMOLOGY. 2008;115:1382–9.

    Article  PubMed  Google Scholar 

  8. Fard MA, Hassanpoor N, Parsa R. Bilateral optic nerve head angiomas and retrobulbar haemangioblastomas in von Hippel-Lindau disease. Neuroophthalmology. 2014;38:254–6.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Duan M, Yang L, Kang J, Wang R, You H, Feng M. Neuroimaging features of optic nerve hemangioblastoma identified by conventional and advanced magnetic resonance techniques: a case report and literature review. Front Oncol. 2021;11:763696.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Turel MK, Kucharczyk W, Gentili F. Optic nerve hemangioblastomas?a review of visual outcomes. Turk Neurosurg. 2017;27:827–31.

    PubMed  Google Scholar 

  11. Higashida T, Sakata K, Kanno H, Kawasaki T, Tanabe Y, Yamamoto I. Hemangioblastoma of the optic nerve-case report. Neurol Med Chir (Tokyo). 2007;47:215–8.

    Article  PubMed  Google Scholar 

  12. Xu S, Li Q, Bian B, Zhou H, Li D. Optic nerve Hemangioblastoma with bilateral frontal lobe oedema: a case report. BMC Ophthalmol. 2020;20:437.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Barrett R, Meyer D, Boulos A, Eames F, Torres-Mora J. Optic nerve hemangioblastoma. Ophthalmology. 2008;115:2095.

    Article  PubMed  Google Scholar 

  14. Wladis EJ, Adamo MA, Weintraub L. Optic nerve gliomas. J Neurol Surg B Skull Base. 2021;82:91–5.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Louise MBM, Smerdel M, Borgwadt L, Beck Nielsen SS, Madsen MG, Moller HU, et al. von Hippel-Lindau disease: updated guideline for diagnosis and surveillance. Eur J Med Genet. 2022;65:104538.

    Article  Google Scholar 

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Funding

Open access funding provided by Natural Science Foundation of Beijing Municipality (No.7222028).

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Authors and Affiliations

  1. Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University; Beijing Ophthalmology and Visual Sciences Key Laboratory, Beijing, China

    Hongjuan Liu, Yue Chen, Sitong Guo & Libin Jiang

  2. Department of Radiology, Beijing Tongren Hospital, Capital Medical University, Beijing, China

    Bentao Yang

Authors
  1. Hongjuan Liu
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  2. Bentao Yang
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  3. Yue Chen
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  4. Sitong Guo
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  5. Libin Jiang
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Contributions

HL, draft of the paper, statistical analysis and collection of cases. BY, analysis of radiological data. YC and SG, Data collection. LJ, study design, correspondence and manuscript revision.

Corresponding author

Correspondence to Libin Jiang.

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The authors declare no competing interests.

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The Ethics committee of Beijing Tongren Hospital affiliated with Capital Medical University.

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Liu, H., Yang, B., Chen, Y. et al. Unique properties of clinical manifestation and magnetic resonance imaging for differential diagnosis of optic nerve hemangioblastoma. Eye 38, 3562–3568 (2024). https://doi.org/10.1038/s41433-024-03363-z

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