Progress and controversy in diagnosis and treatment of optic nerve gliomas_Chinese Journal of Ocular Fundus Diseases

Authors：

Wang Songze ,  Jiang Libin

Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China;

Corresponding author：

Jiang Libin, Email: jlbjlb@sina.com

Keywords：

Optic nerve glioma/diagnosis; Optic nerve glioma/therapy; Review

DOI：

10.3760/cma.j.cn511434-20200916-00456

Video：

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Optic nerve glioma (ONG) is a rare central nervous system tumor that occurs in children and adolescents. It’s main pathological type is low-grade pilocytic astrocytoma. It is divided into sporadic ONG and neurofibromatosis type 1 (NF-1) related ONG. Due to the close relationship between ONG and the optic nerve, there is its particularity in diagnosis and treatment. The diagnosis of ONG mainly relies on medical history, symptoms and signs, as well as imaging examinations such as MRI and CT. ONG should be differentiated from optic nerve sheath meningioma, optic neuritis, optic nerve metastasis and other diseases. In recent years, newly discovered molecular targeted therapy and anti-vascular endothelial growth factor drugs are a powerful supplement to ONG. When chemotherapy is not sensitive or resistant, radiotherapy can be considered, but it is only recommended for patients over 7 years of age. Surgery can be considered when the patient’s visual impairment is severe and the appearance of the eye is significantly affected. In addition, due to the susceptibility of NF-1 patients to tumors, the chemotherapy regimen should take into account the risk of secondary leukemia caused by the drug, and the timing of radiotherapy should be after the age of 10. We look forward to further ONG clinical research, which will bring more references for future clinical work.

Citation： Wang Songze, Jiang Libin. Progress and controversy in diagnosis and treatment of optic nerve gliomas. Chinese Journal of Ocular Fundus Diseases, 2021, 37(3): 248-251. doi: 10.3760/cma.j.cn511434-20200916-00456 Copy

1.	Robert-Boire V, Rosca L, Samson Y, et al. Clinical presentation and outcome of patients with optic pathway glioma[J]. Pediatr Neurol, 2017, 75: 55-60. DOI: 10.1016/j.pediatrneurol.2017.06.019.
2.	Segal L, Darvish-Zargar M, Dilenge ME, et al. Optic pathway gliomas in patients with neurofibromatosis type 1: follow-up of 44 patients[J]. J JAAPOS, 2010, 14(2): 155-158. DOI: 10.1016/j.jaapos.2009.11.020.
3.	Avery RA, Bouffet E, Packer RJ, et al. Feasibility and comparison of visual acuity testing methods in children with neurofibromatosis type 1 and/or optic pathway gliomas[J]. Invest Ophthalmol Vis Sci, 2013, 54(2): 1034-1038. DOI: 10.1167/iovs.12-11385.
4.	Avery RA, Fisher MJ, Liu GT. Optic pathway gliomas[J]. J Neuroophthalmol, 2011, 31(3): 269-278. DOI: 10.1097/WNO.0b013e31822aef82.
5.	Purohit BS, Vargas MI, Ailianou A, et al. Orbital tumours and tumour-like lesions: exploring the armamentarium of multiparametric imaging[J]. Insights Imaging, 2016, 7(1): 43-68. DOI: 10.1007/s13244-015-0443-8.
6.	Campen CJ, Gutmann DH. Optic pathway gliomas in neurofibromatosis type 1[J]. J Child Neurol, 2018, 33(1): 73-81. DOI: 10.1177/0883073817739509.
7.	Marsault P, Ducassou S, Menut F, et al. Diagnostic performance of an unenhanced MRI exam for tumor follow-up of the optic pathway gliomas in children[J]. Neuroradiology, 2019, 61(6): 711-720. DOI: 10.1007/s00234-019-02198-w.
8.	Binning MJ, Liu JK, Kestle JR, et al. Optic pathway gliomas: a review[J]. Neurosurg Focus, 2007, 23(5): E2. DOI: 10.3171/FOC-07/11/E2.
9.	Shapey J, Danesh-Meyer HV, Kaye AH, et al. Diagnosis and management of optic nerve glioma[J]. J Clin Neurosci, 2011, 18(12): 1585-1591. DOI: 10.1016/j.jocn.2011.09.003.
10.	Narain S, Kini A, Ramasubramanian A. Advancements in the management of optic pathway gliomas[J/OL]. Ocular Oncology, 2019, 2019: 47-60[2019-07-11]. DOI: 10.1007/978-981-13-7538-5_4. https://link.springer.com/chapter/10.1007%2F978-981-13-7538-5_4. /. DOI:10.1007/978-981-13-7538-5_4..
11.	Farazdaghi MK, Katowitz WR, Avery RA. Current treatment of optic nerve gliomas[J]. Curr Opin Ophthalmol, 2019, 30(5): 356-363. DOI: 10.1097/ICU.0000000000000587.
12.	Rasool N, Odel JG, Kazim M. Optic pathway glioma of childhood[J]. Curr Opin Ophthalmol, 2017, 28(3): 289-295. DOI: 10.1097/ICU.0000000000000370.
13.	Minturn JE, Fisher MJ. Gliomas in children[J]. Curr Treat Options Neurol, 2013, 15(3): 316-327. DOI: 10.1007/s11940-013-0225-x.
14.	Gururangan S, Fisher MJ, Allen JC, et al. Temozolomide in children with progressive low-grade glioma[J]. Neuro Oncol, 2007, 9(2): 161-168. DOI: 10.1215/15228517-2006-030.
15.	Azizi AA, Schouten-van Meeteren AYN. Current and emerging treatment strategies for children with progressive chiasmatic-hypothalamic glioma diagnosed as infants: a web-based survey[J]. J Neurooncol, 2018, 136(1): 127-134. DOI: 10.1007/s11060-017-2630-6.
16.	Combs SE, Schulz-Ertner D, Moschos D, et al. Fractionated stereotactic radiotherapy of optic pathway gliomas: tolerance and long-term outcome[J]. Int J Radiat Oncol Biol Phys, 2005, 62(3): 814-819. DOI: 10.1016/j.ijrobp.2004.12.081.
17.	Tsang DS, Murphy ES, Merchant TE. Radiation therapy for optic pathway and hypothalamic low-grade gliomas in children[J]. Int J Radiat Oncol Biol Phys, 2017, 99(3): 642-651. DOI: 10.1016/j.ijrobp.2017.07.023.
18.	Packer RJ, Pfister S, Bouffet E, et al. Pediatric low-grade gliomas: implications of the biologic era[J]. Neuro Oncol, 2017, 19(6): 750-761. DOI: 10.1093/neuonc/now209.
19.	Cheng Y, Tian H. Current development status of MEK inhibitors[J]. Molecules, 2017, 22(10): 1551. DOI: 10.3390/molecules22101551.
20.	Banerjee A, Jakacki RI, Onar-Thomas A, et al. A phase Ⅰ trial of the MEK inhibitor selumetinib (AZD6244) in pediatric patients with recurrent or refractory low-grade glioma: a Pediatric Brain Tumor Consortium (PBTC) study[J]. Neuro Oncol, 2017, 19(8): 1135-1144. DOI: 10.1093/neuonc/now282.
21.	Avery RA, Trimboli-Heidler C, Kilburn LB. Separation of outer retinal layers secondary to selumetinib[J]. J AAPOS, 2016, 20(3): 268-271. DOI: 10.1016/j.jaapos.2016.01.012.
22.	Bartels U, Hawkins C, Jing M, et al. Vascularity and angiogenesis as predictors of growth in optic pathway/hypothalamic gliomas[J]. J Neurosurg, 2006, 104(5): 314-320. DOI: 10.3171/ped.2006.104.5.314.
23.	武万水, 刘晶晶, 孙艳玲, 等. 贝伐珠单抗治疗儿童视路胶质瘤的疗效分析[J]. 中国当代儿科杂志, 2019, 21(12): 1193-1197. DOI: 10.7499/j.issn.1008-8830.2019.12.008.Wu WS, Liu JJ, Sun YL, et al. Effect of bevacizumab in treatment of children with optic pathway glioma[J]. Chinese Journal of Contemporary Pediatrics, 2019, 21(12): 1193-1197. DOI: 10.7499/j.issn.1008-8830.2019.12.008.
24.	Lu VM, Welby JP, Nesvick CL, et al. Efficacy and safety of bevacizumab in progressive pediatric low-grade glioma: a systematic review and meta-analysis of outcome rates[J]. Neurooncol Pract, 2020, 7(4): 359-368. DOI: 10.1093/nop/npz076.
25.	Nair AG, Pathak RS, Iyer VR, et al. Optic nerve glioma: an update[J]. Int Ophthalmol, 2014, 34(4): 999-1005. DOI: 10.1007/s10792-014-9942-8.
26.	Dong Q, Yang B, Han JG, et al. A novel hydrogen sulfide-releasing donor, HA-ADT, suppresses the growth of human breast cancer cells through inhibiting the PI3K/AKT/mTOR and Ras/Raf/MEK/ERK signaling pathways[J]. Cancer Lett, 2019, 455: 60-72. DOI: 10.1016/j.canlet.2019.04.031.

1. Robert-Boire V, Rosca L, Samson Y, et al. Clinical presentation and outcome of patients with optic pathway glioma[J]. Pediatr Neurol, 2017, 75: 55-60. DOI: 10.1016/j.pediatrneurol.2017.06.019.
2. Segal L, Darvish-Zargar M, Dilenge ME, et al. Optic pathway gliomas in patients with neurofibromatosis type 1: follow-up of 44 patients[J]. J JAAPOS, 2010, 14(2): 155-158. DOI: 10.1016/j.jaapos.2009.11.020.
3. Avery RA, Bouffet E, Packer RJ, et al. Feasibility and comparison of visual acuity testing methods in children with neurofibromatosis type 1 and/or optic pathway gliomas[J]. Invest Ophthalmol Vis Sci, 2013, 54(2): 1034-1038. DOI: 10.1167/iovs.12-11385.
4. Avery RA, Fisher MJ, Liu GT. Optic pathway gliomas[J]. J Neuroophthalmol, 2011, 31(3): 269-278. DOI: 10.1097/WNO.0b013e31822aef82.
5. Purohit BS, Vargas MI, Ailianou A, et al. Orbital tumours and tumour-like lesions: exploring the armamentarium of multiparametric imaging[J]. Insights Imaging, 2016, 7(1): 43-68. DOI: 10.1007/s13244-015-0443-8.
6. Campen CJ, Gutmann DH. Optic pathway gliomas in neurofibromatosis type 1[J]. J Child Neurol, 2018, 33(1): 73-81. DOI: 10.1177/0883073817739509.
7. Marsault P, Ducassou S, Menut F, et al. Diagnostic performance of an unenhanced MRI exam for tumor follow-up of the optic pathway gliomas in children[J]. Neuroradiology, 2019, 61(6): 711-720. DOI: 10.1007/s00234-019-02198-w.
8. Binning MJ, Liu JK, Kestle JR, et al. Optic pathway gliomas: a review[J]. Neurosurg Focus, 2007, 23(5): E2. DOI: 10.3171/FOC-07/11/E2.
9. Shapey J, Danesh-Meyer HV, Kaye AH, et al. Diagnosis and management of optic nerve glioma[J]. J Clin Neurosci, 2011, 18(12): 1585-1591. DOI: 10.1016/j.jocn.2011.09.003.
10. Narain S, Kini A, Ramasubramanian A. Advancements in the management of optic pathway gliomas[J/OL]. Ocular Oncology, 2019, 2019: 47-60[2019-07-11]. DOI: 10.1007/978-981-13-7538-5_4. https://link.springer.com/chapter/10.1007%2F978-981-13-7538-5_4. /. DOI:10.1007/978-981-13-7538-5_4..
11. Farazdaghi MK, Katowitz WR, Avery RA. Current treatment of optic nerve gliomas[J]. Curr Opin Ophthalmol, 2019, 30(5): 356-363. DOI: 10.1097/ICU.0000000000000587.
12. Rasool N, Odel JG, Kazim M. Optic pathway glioma of childhood[J]. Curr Opin Ophthalmol, 2017, 28(3): 289-295. DOI: 10.1097/ICU.0000000000000370.
13. Minturn JE, Fisher MJ. Gliomas in children[J]. Curr Treat Options Neurol, 2013, 15(3): 316-327. DOI: 10.1007/s11940-013-0225-x.
14. Gururangan S, Fisher MJ, Allen JC, et al. Temozolomide in children with progressive low-grade glioma[J]. Neuro Oncol, 2007, 9(2): 161-168. DOI: 10.1215/15228517-2006-030.
15. Azizi AA, Schouten-van Meeteren AYN. Current and emerging treatment strategies for children with progressive chiasmatic-hypothalamic glioma diagnosed as infants: a web-based survey[J]. J Neurooncol, 2018, 136(1): 127-134. DOI: 10.1007/s11060-017-2630-6.
16. Combs SE, Schulz-Ertner D, Moschos D, et al. Fractionated stereotactic radiotherapy of optic pathway gliomas: tolerance and long-term outcome[J]. Int J Radiat Oncol Biol Phys, 2005, 62(3): 814-819. DOI: 10.1016/j.ijrobp.2004.12.081.
17. Tsang DS, Murphy ES, Merchant TE. Radiation therapy for optic pathway and hypothalamic low-grade gliomas in children[J]. Int J Radiat Oncol Biol Phys, 2017, 99(3): 642-651. DOI: 10.1016/j.ijrobp.2017.07.023.
18. Packer RJ, Pfister S, Bouffet E, et al. Pediatric low-grade gliomas: implications of the biologic era[J]. Neuro Oncol, 2017, 19(6): 750-761. DOI: 10.1093/neuonc/now209.
19. Cheng Y, Tian H. Current development status of MEK inhibitors[J]. Molecules, 2017, 22(10): 1551. DOI: 10.3390/molecules22101551.
20. Banerjee A, Jakacki RI, Onar-Thomas A, et al. A phase Ⅰ trial of the MEK inhibitor selumetinib (AZD6244) in pediatric patients with recurrent or refractory low-grade glioma: a Pediatric Brain Tumor Consortium (PBTC) study[J]. Neuro Oncol, 2017, 19(8): 1135-1144. DOI: 10.1093/neuonc/now282.
21. Avery RA, Trimboli-Heidler C, Kilburn LB. Separation of outer retinal layers secondary to selumetinib[J]. J AAPOS, 2016, 20(3): 268-271. DOI: 10.1016/j.jaapos.2016.01.012.
22. Bartels U, Hawkins C, Jing M, et al. Vascularity and angiogenesis as predictors of growth in optic pathway/hypothalamic gliomas[J]. J Neurosurg, 2006, 104(5): 314-320. DOI: 10.3171/ped.2006.104.5.314.
23. 武万水, 刘晶晶, 孙艳玲, 等. 贝伐珠单抗治疗儿童视路胶质瘤的疗效分析[J]. 中国当代儿科杂志, 2019, 21(12): 1193-1197. DOI: 10.7499/j.issn.1008-8830.2019.12.008.Wu WS, Liu JJ, Sun YL, et al. Effect of bevacizumab in treatment of children with optic pathway glioma[J]. Chinese Journal of Contemporary Pediatrics, 2019, 21(12): 1193-1197. DOI: 10.7499/j.issn.1008-8830.2019.12.008.
24. Lu VM, Welby JP, Nesvick CL, et al. Efficacy and safety of bevacizumab in progressive pediatric low-grade glioma: a systematic review and meta-analysis of outcome rates[J]. Neurooncol Pract, 2020, 7(4): 359-368. DOI: 10.1093/nop/npz076.
25. Nair AG, Pathak RS, Iyer VR, et al. Optic nerve glioma: an update[J]. Int Ophthalmol, 2014, 34(4): 999-1005. DOI: 10.1007/s10792-014-9942-8.
26. Dong Q, Yang B, Han JG, et al. A novel hydrogen sulfide-releasing donor, HA-ADT, suppresses the growth of human breast cancer cells through inhibiting the PI3K/AKT/mTOR and Ras/Raf/MEK/ERK signaling pathways[J]. Cancer Lett, 2019, 455: 60-72. DOI: 10.1016/j.canlet.2019.04.031.

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