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Optic Pathway Glioma
Daniel E. Meltzer, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Optic pathway glioma (OPG)
            • Synonyms

              • Optic nerve (ON) glioma; anterior visual pathway glioma
            • Definitions

              • Primary neuroglial tumor of optic pathway
              • 3 broad subtypes
                • Childhood benign tumors: With neurofibromatosis type 1 (NF1), syndromic 30-40%
                • Childhood benign tumors: Without NF1 (sporadic)
                • Adult tumors: Typically malignant

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Boonzaier NR et al: Quantitative MRI demonstrates abnormalities of the third ventricle subventricular zone in neurofibromatosis type-1 and sporadic paediatric optic pathway glioma. Neuroimage Clin. 28:102447, 2020
                                            2. Pisapia JM et al: Predicting pediatric optic pathway glioma progression using advanced magnetic resonance image analysis and machine learning. Neurooncol Adv. 2(1):vdaa090, 2020
                                            3. de Blank PMK et al: Optic pathway gliomas in neurofibromatosis type 1: an update: surveillance, treatment indications, and biomarkers of vision. J Neuroophthalmol. 37 Suppl 1:S23-32, 2017
                                            4. Friedrich RE et al: Optic pathway glioma and cerebral focal abnormal signal intensity in patients with neurofibromatosis type 1: characteristics, treatment choices and follow-up in 134 affected individuals and a brief review of the literature. Anticancer Res. 36(8):4095-121, 2016
                                            5. Aquilina K et al: Optic pathway glioma in children: does visual deficit correlate with radiology in focal exophytic lesions? Childs Nerv Syst. 31(11):2041-9, 2015
                                            6. Shofty B et al: The effect of chemotherapy on optic pathway gliomas and their sub-components: a volumetric MR analysis study. Pediatr Blood Cancer. 62(8):1353-9, 2015
                                            7. Rodriguez FJ et al: BRAF duplications and MAPK pathway activation are frequent in gliomas of the optic nerve proper. J Neuropathol Exp Neurol. 71(9):789-94, 2012
                                            8. Nicolin G et al: Natural history and outcome of optic pathway gliomas in children. Pediatr Blood Cancer. 53(7):1231-7, 2009
                                            9. Schupper A et al: Optic-pathway glioma: natural history demonstrated by a new empirical score. Pediatr Neurol. 40(6):432-6, 2009
                                            10. Jost SC et al: Diffusion-weighted and dynamic contrast-enhanced imaging as markers of clinical behavior in children with optic pathway glioma. Pediatr Radiol. 38(12):1293-9, 2008
                                            11. Pereira LS et al: Perineural arachnoidal gliomatosis: case report. Arq Bras Oftalmol. 71(4):595-8, 2008
                                            12. Taylor T et al: Radiological classification of optic pathway gliomas: experience of a modified functional classification system. Br J Radiol. 81(970):761-6, 2008
                                            13. Walrath JD et al: Magnetic resonance imaging evidence of optic nerve glioma progression into and beyond the optic chiasm. Ophthal Plast Reconstr Surg. 24(6):473-5, 2008
                                            14. Zeid JL et al: Orbital optic nerve gliomas in children with neurofibromatosis type 1. J AAPOS. 10(6):534-9, 2006
                                            15. Chateil JF et al: MRI and clinical differences between optic pathway tumours in children with and without neurofibromatosis. Br J Radiol. 74(877):24-31, 2001
                                            16. Parsa CF et al: Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging. Arch Ophthalmol. 119(4):516-29, 2001
                                            17. Friedman JM et al: An association between optic glioma and other tumours of the central nervous system in neurofibromatosis type 1. Neuropediatrics. 28(2):131-2, 1997
                                            18. Listernick R et al: Optic pathway tumors in children: the effect of neurofibromatosis type 1 on clinical manifestations and natural history. J Pediatr. 127(5):718-22, 1995
                                            19. Millar WS et al: MR of malignant optic glioma of adulthood. AJNR Am J Neuroradiol. 16(8):1673-6, 1995
                                            20. Parazzini C et al: Spontaneous involution of optic pathway lesions in neurofibromatosis type 1: serial contrast MR evaluation. AJNR Am J Neuroradiol. 16(8):1711-8, 1995
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Optic pathway glioma (OPG)
                                                      • Synonyms

                                                        • Optic nerve (ON) glioma; anterior visual pathway glioma
                                                      • Definitions

                                                        • Primary neuroglial tumor of optic pathway
                                                        • 3 broad subtypes
                                                          • Childhood benign tumors: With neurofibromatosis type 1 (NF1), syndromic 30-40%
                                                          • Childhood benign tumors: Without NF1 (sporadic)
                                                          • Adult tumors: Typically malignant

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR Findings

                                                            • Ultrasonographic Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Boonzaier NR et al: Quantitative MRI demonstrates abnormalities of the third ventricle subventricular zone in neurofibromatosis type-1 and sporadic paediatric optic pathway glioma. Neuroimage Clin. 28:102447, 2020
                                                                                      2. Pisapia JM et al: Predicting pediatric optic pathway glioma progression using advanced magnetic resonance image analysis and machine learning. Neurooncol Adv. 2(1):vdaa090, 2020
                                                                                      3. de Blank PMK et al: Optic pathway gliomas in neurofibromatosis type 1: an update: surveillance, treatment indications, and biomarkers of vision. J Neuroophthalmol. 37 Suppl 1:S23-32, 2017
                                                                                      4. Friedrich RE et al: Optic pathway glioma and cerebral focal abnormal signal intensity in patients with neurofibromatosis type 1: characteristics, treatment choices and follow-up in 134 affected individuals and a brief review of the literature. Anticancer Res. 36(8):4095-121, 2016
                                                                                      5. Aquilina K et al: Optic pathway glioma in children: does visual deficit correlate with radiology in focal exophytic lesions? Childs Nerv Syst. 31(11):2041-9, 2015
                                                                                      6. Shofty B et al: The effect of chemotherapy on optic pathway gliomas and their sub-components: a volumetric MR analysis study. Pediatr Blood Cancer. 62(8):1353-9, 2015
                                                                                      7. Rodriguez FJ et al: BRAF duplications and MAPK pathway activation are frequent in gliomas of the optic nerve proper. J Neuropathol Exp Neurol. 71(9):789-94, 2012
                                                                                      8. Nicolin G et al: Natural history and outcome of optic pathway gliomas in children. Pediatr Blood Cancer. 53(7):1231-7, 2009
                                                                                      9. Schupper A et al: Optic-pathway glioma: natural history demonstrated by a new empirical score. Pediatr Neurol. 40(6):432-6, 2009
                                                                                      10. Jost SC et al: Diffusion-weighted and dynamic contrast-enhanced imaging as markers of clinical behavior in children with optic pathway glioma. Pediatr Radiol. 38(12):1293-9, 2008
                                                                                      11. Pereira LS et al: Perineural arachnoidal gliomatosis: case report. Arq Bras Oftalmol. 71(4):595-8, 2008
                                                                                      12. Taylor T et al: Radiological classification of optic pathway gliomas: experience of a modified functional classification system. Br J Radiol. 81(970):761-6, 2008
                                                                                      13. Walrath JD et al: Magnetic resonance imaging evidence of optic nerve glioma progression into and beyond the optic chiasm. Ophthal Plast Reconstr Surg. 24(6):473-5, 2008
                                                                                      14. Zeid JL et al: Orbital optic nerve gliomas in children with neurofibromatosis type 1. J AAPOS. 10(6):534-9, 2006
                                                                                      15. Chateil JF et al: MRI and clinical differences between optic pathway tumours in children with and without neurofibromatosis. Br J Radiol. 74(877):24-31, 2001
                                                                                      16. Parsa CF et al: Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging. Arch Ophthalmol. 119(4):516-29, 2001
                                                                                      17. Friedman JM et al: An association between optic glioma and other tumours of the central nervous system in neurofibromatosis type 1. Neuropediatrics. 28(2):131-2, 1997
                                                                                      18. Listernick R et al: Optic pathway tumors in children: the effect of neurofibromatosis type 1 on clinical manifestations and natural history. J Pediatr. 127(5):718-22, 1995
                                                                                      19. Millar WS et al: MR of malignant optic glioma of adulthood. AJNR Am J Neuroradiol. 16(8):1673-6, 1995
                                                                                      20. Parazzini C et al: Spontaneous involution of optic pathway lesions in neurofibromatosis type 1: serial contrast MR evaluation. AJNR Am J Neuroradiol. 16(8):1711-8, 1995