U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Optic pathway glioma

MedGen UID:
162950
•Concept ID:
C0796418
•
Neoplastic Process
Synonyms: glioma of optic tract; glioma of the optic tract; Glioma of the Visual Pathway; glioma of the visual pathway; Glioma of Visual Pathway; glioma of visual pathway; optic pathway glioma; optic tract glioma; Visual Pathway Glioma; visual pathway glioma
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016167
Orphanet: ORPHA2086

Definition

Optic pathway glioma (OPG) is a benign tumor that develop along the optic nerve (chiasm, tracts, and radiations) characterized by impairment or loss of vision and may be accompanied by diencephalic symptoms such as reduced growth and alteration in sleeping patterns. OPG are often linked to neurofibromatosis type 1 (NF1, see this term). [from ORDO]

Professional guidelines

PubMed

Risk factors for treatment-refractory and relapsed optic pathway glioma in children with neurofibromatosis type 1.
Kotch C, Avery R, Getz KD, Bouffet E, de Blank P, Listernick R, Gutmann DH, Bornhorst M, Campen C, Liu GT, Aplenc R, Li Y, Fisher MJ
Neuro Oncol 2022 Aug 1;24(8):1377-1386. doi: 10.1093/neuonc/noac013. PMID: 35018469Free PMC Article
Treatment and Outcome in 65 Children with Optic Pathway Gliomas.
El Beltagy MA, Reda M, Enayet A, Zaghloul MS, Awad M, Zekri W, Taha H, El-Khateeb N
World Neurosurg 2016 May;89:525-34. Epub 2016 Feb 18 doi: 10.1016/j.wneu.2016.02.042. PMID: 26898488
Neurofibromatosis type 1 (NF1): diagnosis and management.
Ferner RE, Gutmann DH
Handb Clin Neurol 2013;115:939-55. doi: 10.1016/B978-0-444-52902-2.00053-9. PMID: 23931823

Recent clinical studies

Etiology

Optic pathway glioma and endocrine disorders in patients with and without NF1.
Gil Margolis M, Yackobovitz-Gavan M, Toledano H, Tenenbaum A, Cohen R, Phillip M, Shalitin S
Pediatr Res 2023 Jan;93(1):233-241. Epub 2022 May 10 doi: 10.1038/s41390-022-02098-5. PMID: 35538247
Regression and pseudoprogression of pediatric optic pathway glioma in patients treated with proton beam therapy.
Youn SH, Kim H, Lee SH, Kim JY
Pediatr Blood Cancer 2022 Mar;69(3):e29434. Epub 2021 Nov 12 doi: 10.1002/pbc.29434. PMID: 34766717
Neurofibromatosis in the Era of Precision Medicine: Development of MEK Inhibitors and Recent Successes with Selumetinib.
Galvin R, Watson AL, Largaespada DA, Ratner N, Osum S, Moertel CL
Curr Oncol Rep 2021 Mar 15;23(4):45. doi: 10.1007/s11912-021-01032-y. PMID: 33721151
NEUROFIBROMATOSIS TYPE 1 AND OPTIC PATHWAY GLIOMA.
Siwá A, Autrata R, Vejmělková K, Pavelka Z, Zitterbart K
Cesk Slov Oftalmol 2019 Winter;75(4):200-208. doi: 10.31348/2019/4/4. PMID: 32397722
Neurofibromatosis type 1.
Anderson JL, Gutmann DH
Handb Clin Neurol 2015;132:75-86. doi: 10.1016/B978-0-444-62702-5.00004-4. PMID: 26564071

Diagnosis

Optic pathway glioma and endocrine disorders in patients with and without NF1.
Gil Margolis M, Yackobovitz-Gavan M, Toledano H, Tenenbaum A, Cohen R, Phillip M, Shalitin S
Pediatr Res 2023 Jan;93(1):233-241. Epub 2022 May 10 doi: 10.1038/s41390-022-02098-5. PMID: 35538247
Neurofibromatosis Type 1: Review and Update on Emerging Therapies.
Karaconji T, Whist E, Jamieson RV, Flaherty MP, Grigg JRB
Asia Pac J Ophthalmol (Phila) 2019 Jan-Feb;8(1):62-72. Epub 2018 Nov 2 doi: 10.22608/APO.2018182. PMID: 30387339
Pediatric Optic Pathway/Hypothalamic Glioma.
Aihara Y, Chiba K, Eguchi S, Amano K, Kawamata T
Neurol Med Chir (Tokyo) 2018 Jan 15;58(1):1-9. Epub 2017 Nov 9 doi: 10.2176/nmc.ra.2017-0081. PMID: 29118304Free PMC Article
Optic pathway glioma of childhood.
Rasool N, Odel JG, Kazim M
Curr Opin Ophthalmol 2017 May;28(3):289-295. doi: 10.1097/ICU.0000000000000370. PMID: 28257299
Neurofibromatosis type 1 (NF1): diagnosis and management.
Ferner RE, Gutmann DH
Handb Clin Neurol 2013;115:939-55. doi: 10.1016/B978-0-444-52902-2.00053-9. PMID: 23931823

Therapy

Novel "T-Dimension" Therapies for Pediatric Optic Pathway Glioma: A Timely, Targeted, and Tailored Treatment Trend.
Giotta Lucifero A, Elbabaa SK, Baldoncini M, Bruno N, Savasta S, Marseglia GL, Luzzi S
Pediatr Neurosurg 2022;57(3):161-174. Epub 2022 May 4 doi: 10.1159/000524873. PMID: 35588700
Neurofibromatosis in the Era of Precision Medicine: Development of MEK Inhibitors and Recent Successes with Selumetinib.
Galvin R, Watson AL, Largaespada DA, Ratner N, Osum S, Moertel CL
Curr Oncol Rep 2021 Mar 15;23(4):45. doi: 10.1007/s11912-021-01032-y. PMID: 33721151
A phase II trial of selumetinib in children with recurrent optic pathway and hypothalamic low-grade glioma without NF1: a Pediatric Brain Tumor Consortium study.
Fangusaro J, Onar-Thomas A, Poussaint TY, Wu S, Ligon AH, Lindeman N, Campagne O, Banerjee A, Gururangan S, Kilburn LB, Goldman S, Qaddoumi I, Baxter P, Vezina G, Bregman C, Patay Z, Jones JY, Stewart CF, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Neuro Oncol 2021 Oct 1;23(10):1777-1788. doi: 10.1093/neuonc/noab047. PMID: 33631016Free PMC Article
NF1 optic pathway glioma: analyzing risk factors for visual outcome and indications to treat.
Azizi AA, Walker DA, Liu JF, Sehested A, Jaspan T, Pemp B, Simmons I, Ferner R, Grill J, Hargrave D, Driever PH, Evans DG, Opocher E; SIOPE NF1 OPG Nottingham, UK, Workshop 2014
Neuro Oncol 2021 Jan 30;23(1):100-111. doi: 10.1093/neuonc/noaa153. PMID: 32628746Free PMC Article
Neurofibromatosis Type 1: Review and Update on Emerging Therapies.
Karaconji T, Whist E, Jamieson RV, Flaherty MP, Grigg JRB
Asia Pac J Ophthalmol (Phila) 2019 Jan-Feb;8(1):62-72. Epub 2018 Nov 2 doi: 10.22608/APO.2018182. PMID: 30387339

Prognosis

A phase II trial of selumetinib in children with recurrent optic pathway and hypothalamic low-grade glioma without NF1: a Pediatric Brain Tumor Consortium study.
Fangusaro J, Onar-Thomas A, Poussaint TY, Wu S, Ligon AH, Lindeman N, Campagne O, Banerjee A, Gururangan S, Kilburn LB, Goldman S, Qaddoumi I, Baxter P, Vezina G, Bregman C, Patay Z, Jones JY, Stewart CF, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Neuro Oncol 2021 Oct 1;23(10):1777-1788. doi: 10.1093/neuonc/noab047. PMID: 33631016Free PMC Article
NEUROFIBROMATOSIS TYPE 1 AND OPTIC PATHWAY GLIOMA.
Siwá A, Autrata R, Vejmělková K, Pavelka Z, Zitterbart K
Cesk Slov Oftalmol 2019 Winter;75(4):200-208. doi: 10.31348/2019/4/4. PMID: 32397722
Pediatric Optic Pathway/Hypothalamic Glioma.
Aihara Y, Chiba K, Eguchi S, Amano K, Kawamata T
Neurol Med Chir (Tokyo) 2018 Jan 15;58(1):1-9. Epub 2017 Nov 9 doi: 10.2176/nmc.ra.2017-0081. PMID: 29118304Free PMC Article
Optic pathway glioma of childhood.
Rasool N, Odel JG, Kazim M
Curr Opin Ophthalmol 2017 May;28(3):289-295. doi: 10.1097/ICU.0000000000000370. PMID: 28257299
Optic pathway gliomas.
Jahraus CD, Tarbell NJ
Pediatr Blood Cancer 2006 May 1;46(5):586-96. doi: 10.1002/pbc.20655. PMID: 16411210

Clinical prediction guides

Visual Outcomes of Optic Pathway Glioma Treated With Chemotherapy in Neurofibromatosis Type 1.
José P, Couceiro R, Passos J, Jorge Teixeira F
J Pediatr Ophthalmol Strabismus 2022 Mar-Apr;59(2):128-135. Epub 2021 Sep 30 doi: 10.3928/01913913-20210720-02. PMID: 34592874
NF1 optic pathway glioma: analyzing risk factors for visual outcome and indications to treat.
Azizi AA, Walker DA, Liu JF, Sehested A, Jaspan T, Pemp B, Simmons I, Ferner R, Grill J, Hargrave D, Driever PH, Evans DG, Opocher E; SIOPE NF1 OPG Nottingham, UK, Workshop 2014
Neuro Oncol 2021 Jan 30;23(1):100-111. doi: 10.1093/neuonc/noaa153. PMID: 32628746Free PMC Article
NEUROFIBROMATOSIS TYPE 1 AND OPTIC PATHWAY GLIOMA.
Siwá A, Autrata R, Vejmělková K, Pavelka Z, Zitterbart K
Cesk Slov Oftalmol 2019 Winter;75(4):200-208. doi: 10.31348/2019/4/4. PMID: 32397722
Neurological malignancies in neurofibromatosis type 1.
Eoli M, Saletti V, Finocchiaro G
Curr Opin Oncol 2019 Nov;31(6):554-561. doi: 10.1097/CCO.0000000000000576. PMID: 31436563
Clinical Presentation and Outcome of Patients With Optic Pathway Glioma.
Robert-Boire V, Rosca L, Samson Y, Ospina LH, Perreault S
Pediatr Neurol 2017 Oct;75:55-60. Epub 2017 Jul 6 doi: 10.1016/j.pediatrneurol.2017.06.019. PMID: 28847625

Recent systematic reviews

Novel "T-Dimension" Therapies for Pediatric Optic Pathway Glioma: A Timely, Targeted, and Tailored Treatment Trend.
Giotta Lucifero A, Elbabaa SK, Baldoncini M, Bruno N, Savasta S, Marseglia GL, Luzzi S
Pediatr Neurosurg 2022;57(3):161-174. Epub 2022 May 4 doi: 10.1159/000524873. PMID: 35588700
Resolving mystery behind autonomous retrogression of low-grade gliomas: A systematic review.
Ahmed SI, Bareeqa SB, Samar SS, Jilanee SDA
J Pak Med Assoc 2020 Dec;70(12(B)):2441-2448. doi: 10.47391/JPMA.581. PMID: 33475560
Café-au-lait Macules and Neurofibromatosis Type 1: A Review of the Literature.
Bernier A, Larbrisseau A, Perreault S
Pediatr Neurol 2016 Jul;60:24-29.e1. Epub 2016 Mar 19 doi: 10.1016/j.pediatrneurol.2016.03.003. PMID: 27212418
Does chemotherapy affect the visual outcome in children with optic pathway glioma? A systematic review of the evidence.
Moreno L, Bautista F, Ashley S, Duncan C, Zacharoulis S
Eur J Cancer 2010 Aug;46(12):2253-9. Epub 2010 Apr 17 doi: 10.1016/j.ejca.2010.03.028. PMID: 20400294
Prognostic factors for progression of childhood optic pathway glioma: a systematic review.
Opocher E, Kremer LC, Da Dalt L, van de Wetering MD, Viscardi E, Caron HN, Perilongo G
Eur J Cancer 2006 Aug;42(12):1807-16. Epub 2006 Jun 30 doi: 10.1016/j.ejca.2006.02.022. PMID: 16809032

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...