Orbital Neurogenic Tumors: An Eye Care Service Experience in Turkey

Öz

Introduction:This study aimed to evaluate the clinical and pathological features and treatment outcomes of neurogenic tumors developing in the orbit. Materials and Methods: A retrospective study was conducted on the medical records of 23 patients diagnosed with orbital neurogenic tumors between 2008 and 2020 in the ophthalmology clinic. Clinicopathologic features and treatment results of this patient group were evaluated. Results: Twenty-three patients, mean (SD) age 33.34±20.18 (min-max 4-60) years, were included in this study. Median follow-up time was 56.7±42.7 (min-max 9-120) months. At the time of presentation, 15 (65%) patients had proptosis, 8 (35%) patients had strabismus and reduced vision. Histopathologic diagnosis was made after lateral orbitotomy through the skin in 16 patients (69.5%) and medial orbitotomy through the conjunctiva in 4 patients (17.4%). The diagnosis was established clinically in three (13%) cases. Ten patients (43.4%) underwent whole excisional biopsy, nine patients (391.1%) underwent incisional biopsy, and one patient (4.3%) underwent subtotal excisional biopsy. Nine (39.1%) of the orbital neurogenic tumors were diagnosed as meningiomas based on histological and clinical findings, eight (34.8%) as optic nerve gliomas, and the other six (26%) as peripheral nerve origin tumors. Four (44.5%) of the meningiomas originated from the sphenoid wing, and five (55.5%) from the optic nerve sheath. As a treatment modality, external radiotherapy was administered to fifteen patients (65.2%), cyberknife radiosurgery to one patient (4.3%), chemotherapy to one patient (4.3%), and exenteration surgery to one patient (4.3%). Discussion: According to our study, meningioma, optic nerve glioma, and peripheral nerve sheath tumors were the most frequent neurogenic tumors of the orbit. Gliomas and meningiomas of the optic nerve sheath were treated with external radiation. Total excision was performed for schwannoma and solitary neurofibroma among the peripheral nerve sheath tumors, while subtotal excision was performed for infiltrative plexiform neurofibroma. With the treatments applied, survival and the visual prognosis were satisfactory.

Anahtar Kelimeler

• Meningioma
• orbit
• schwannoma
• neurofibroma
• optic nerve glioma

Kaynakça

1. 1.Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology.2004;111:997‒1008.
2. 2.Ghassibi MP,Ulloa-Padilla JP,Dubovy SR. Neural Tumors of the Orbit—What Is New? Asia-Pac J Ophthalmol 2017;6:273–82
3. 3.Kızıltunç PB, Gündüz K,Erden E. Orbita Nörojenik Tümörlerinin Klinik ve Tedavi Özellikleri.Turk J Ophthalmol 2013 ;43:335-9
4. 4.Cantore WA. Neural orbital tumors.Curr Opin Ophthalmol. 2000;11:367‒71
5. 5. Robert-Boire V, Rosca L, Samson Y, et al. Clinical Presentation and Outcome of Patients With Optic Pathway Glioma. Pediatr Neurol. 2017 Oct;75:55-60.
6. 6. Beres SJ, Avery RA. Optic pathway gliomas.J Pediatr Neurol 2017; 15:15–24.
7. 7. Campen CJ, Gutmann DH. Optic Pathway Gliomas in Neurofibromatosis Type 1. J Child Neurol. 2018 Jan;33(1):73-81.
8. 8.Siwá A, Autrata R, Vejmělková K, et al. Neurofibromatosis Type 1 And Optic Pathway Glioma. Cesk Slov Oftalmol 2019 Winter;75(4):200-08.

9. 9. de Blank PMK, Fisher MJ, Liu GT, et al. Optic pathway gliomas in neurofibromatosis type 1: an update: surveillance, treatment indications, and biomarkers of vision.J Neuroophthalmol 2017;37(suppl. 1):23–32.
10. 10.Wan MJ,Ullrich NJ,Manley PE,et al.Long-term visual outcomes of optic pathway gliomas in pediatric patients without neurofibramatosis type 1.J Neurooncol.2016 Aug;129(1):173-8
11. 11.Kinori M ,Armarnik S, Listernick R, et al.Neurofibromatosis Type 1-Associated Optic Pathway Glioma in Children: A Follow-Up of 10 Years or More Am J Ophthalmol. 2021 Jan:221:91-96.
12. 12. Irshad K,Huang YK,Rodriguez P,et al. The Neuroimmune Regulation and Potential Therapeutic Strategies of Optic Pathway Glioma. Brain Sci.2023 Oct;13(10):1424
13. 13. Bello SD,Martinuzzi D,Tereshko Y,et al. The Present and Future of Optic Pathway Glioma Therapy. Cells.2023 Oct;12(19):2380.
14. 14.Farazdaghi MK,Katowitz WR, Avery RA.Current treatment of optic nerve gliomas Curr Opin Ophthalmol.2019 Sep; 30(5): 356–63.
15. 15. Hwang EI, Jakacki RI, Fisher MJ, et al. Long-term efficacy and toxicity of bevacizumab-based therapy in children with recurrent low-grade gliomas. Pediatr Blood Cancer. 2013 May;60(5):776-82
16. 16.Shaikh M, Khera P, Yadav T, et al. Dural Ectasia of the Optic Nerve:A Rare Presentation in Neurofibromatosis Type I. J Neurosci Rural Pract. 2019 Apr-Jun;10(2):349-51
17. 17. Kheir V, Faouzi M, Borruat FX. Visual Outcomes of Fractionated Radiotherapy in Optic Nerve Sheath Meningioma: A Retrospective Study. Klin Monbl Augenheilkd. 2019 Apr;236(4):526-29
18. 18. Pandit R, Paris L, Rudich DS, et al. Long-term efficacy of fractionated conformal radiotherapy for the management of primary optic nerve sheath meningioma. Br J Ophthalmol. 2019 Oct;103(10):1436-40
19. 19. Furdová A, Babál P, Kobzová D. Optic nerve orbital meningioma. Cesk Slov Oftalmol. 2018 Spring;74(1):23-30.
20. 20. Parker RT, Ovens CA, Fraser CL, et al. Optic nerve sheath meningiomas: prevalence, impact, and management strategies. Eye Brain. 2018;10:85-99
21. 21. Sibony P, Strachovsky M, Honkanen R, et al. Optical coherence tomography shape analysis of the peripapillary retinal pigment epithelium layer in presumed optic nerve sheath meningiomas. J Neuroophthalmol. 2014 Jun;34(2):130-6
22. 22.Loo JL, Tian J, Miller NR, et al. Use of optical coherence tomography in predicting post-treatment visual outcome in anterior visual pathway meningiomas. Br J Ophthalmol. 2013 Nov;97(11):1455-8.
23. 23. Hénaux PL, Bretonnier M, Le Reste PJ, et al. Modern Management of Meningiomas Compressing the Optic Nerve: A Systematic Review. World Neurosurg. 2018 Oct;118:e677-86
24. 24. Rassi MS, Prasad S, Can A, et al. Prognostic factors in the surgical treatment of intracanalicular primary optic nerve sheath meningiomas. J Neurosurg. 2018 Sep 21;131(2):481-88.
25. 25. Maza G, Subramaniam S, Yanez-Siller JC, et al. The Role of Endonasal Endoscopic Optic Nerve Decompression as the Initial Management of Primary Optic Nerve Sheath Meningiomas. J Neurol Surg B Skull Base. 2019 Dec;80(6):568-76
26. 26. Hunt PJ, DeMonte F, Tang RA,et al. Surgical Resection of an Optic Nerve Sheath Meningioma: Relevance of Endoscopic Endonasal Approaches to the Optic Canal. J Neurol Surg Rep. 2017 Apr;78(2):e81-e85.
27. 27. Sasano H, Shikishima K, Aoki M, et al. Efficacy of intensity-modulated radiation therapy for optic nerve sheath meningioma. Graefes Arch Clin Exp Ophthalmol. 2019 Oct;257(10):2297-306.
28. 28. Ratnayake G, Oh T, Mehta R, et al. Long-term treatment outcomes of patients with primary optic nerve sheath meningioma treated with stereotactic radiotherapy. J Clin Neurosci. 2019 Oct;68:162-67.
29. 29. Honig S, Trantakis C, Frerich B, et al .Meningiomas involving the sphenoid wing outcome after microsurgical treatment--a clinical review of 73 cases. Cent Eur Neurosurg 2010; 71 : 189-98,
30. 30. Talacchi A, Hasanbelliu A, D’Amico A,et al. Long-term follow-up after surgical removal of meningioma of the inner third of the sphenoidal wing: outcome determinants and different strategies. Neurosurg Rev 2020;43 : 109-17
31. 31. Gonen L, Nov E, Shimony N, et al. Sphenoorbital meningioma: surgical series and design of an intraoperative management algorithm. Neurosurg Rev 2018;41 : 291-301
32. 32. Samadian M, Sharifi G, Mousavinejad SA, et al : Surgical outcomes of sphenoorbital en plaque meningioma: a 10-year experience in 57 consecutive cases. World Neurosurg 2020; 144 : e576-81
33. 33. Masalha W,Heiland DH,Steiert C,et.al.Management of Medial Sphenoid Wing Meningioma Involving the Cavernous Sinus. A Single-Center Series of 105 Cases Cancers (Basel) 2022 Apr 28;14(9):2201
34. 34. Puchner MJA, Hans VH, Harati A, et al. Bevacizumab-induced regression of anaplastic meningioma. Ann Oncol. 2010 Dec;21(12):2445-46
35. 35. Chen MH, Yan JH.Imaging characteristics and surgical management of orbital neurilemmomas.Int J Ophthalmol.2019; 12(7): 1108–15.
36. 36.Chaskes MB,Rabinowitz MR.Orbital Schwannoma.J Neurol Surg B Skull Base. 2020 Aug; 81(4): 376–80.
37. 37. Sweeney AR,Gupta D,Keene CD,et al.Orbital peripheral nerve sheath tumors.Surv Ophthalmol.2017;62:43‒57