Long-term outcomes of symptomatic optic pathway glioma: 32-year experience at a single Western Australian tertiary pediatric oncology center

Revathi Rajagopal; Mumtaz Khan; Robert Lethbridge; Gabriel Lee; Sharon Lee; Jason Dyke; Vicki Fabian; Alycea McGrath; Mandy Taylor; Peter Jacoby; Raelene Endersby; Sumanth Nagabushan; Nicholas G Gottardo

doi:10.3389/fonc.2023.1157909

Long-term outcomes of symptomatic optic pathway glioma: 32-year experience at a single Western Australian tertiary pediatric oncology center

Front Oncol. 2023 Jul 14:13:1157909. doi: 10.3389/fonc.2023.1157909. eCollection 2023.

Authors

Revathi Rajagopal¹, Mumtaz Khan², Robert Lethbridge³, Gabriel Lee⁴, Sharon Lee⁵, Jason Dyke⁶, Vicki Fabian⁶, Alycea McGrath⁷, Mandy Taylor⁷, Peter Jacoby⁸, Raelene Endersby⁹, Sumanth Nagabushan^{10

11}, Nicholas G Gottardo^{9

12}

Affiliations

¹ Department of Global Pediatric Medicine, St Jude Children's Research Hospital, Memphis, TN, United States.
² Department of Anesthesia, Sir Charles Gairdner Hospital, Perth, WA, Australia.
³ School of Pediatrics and Child Health, University of Western Australia, Perth, WA, Australia.
⁴ Department of Neurosurgery, Sir Charles Gairdner Hospital, Perth, WA, Australia.
⁵ Department of Neurosurgery, Perth Children's Hospital, Perth, WA, Australia.
⁶ Department of Neuropathology, Royal Perth Hospital, Perth, WA, Australia.
⁷ Department of Radiation Oncology, Sir Charles Gairdner Hospital, Perth, WA, Australia.
⁸ Department of Biostatistics, Telethon Kids Institute, The University of Western Australia, Perth, WA, Australia.
⁹ Brain Tumor Research Program, Telethon Kids Institute, The University of Western Australia, Perth, WA, Australia.
¹⁰ Kids Cancer Centre, Sydney Children's Hospital, Sydney, NSW, Australia.
¹¹ School of Women's and Children's Health, University of New South Wales, Sydney, NSW, Australia.
¹² Department of Pediatric and Adolescent Oncology and Hematology, Perth Children's Hospital, Perth, WA, Australia.

Abstract

Introduction: Optic pathway gliomas (OPGs) are associated with significant risk of visual and endocrine morbidity, but data on long-term outcomes in symptomatic patients is sparse. This study reviews the clinical course, disease progression, survival outcomes and long-term sequelae in pediatric patients with symptomatic OPGs in our institution over three decades.

Methods: Retrospective review of patients with symptomatic OPG treated in a single tertiary pediatric oncology center from 1984 to 2016.

Results: A total of 37 patients were diagnosed with symptomatic OPG. Decreased visual acuity was the commonest presenting symptom (75.7%). Surgical intervention was performed in 62.2%; 56.5% underwent biopsy, 26.1% surgical debulking and 17.4% had orbital decompression with cystic fenestration and cosmetic optic nerve excision at different treatment intervals. CSF diversion was performed in 47.8% patients. Histopathologic examination confirmed 86% to be pilocytic astrocytoma and 1 ganglioglioma. 46% received chemotherapy and 48% had radiotherapy, at different intervals. Median follow-up was 13.74 years. In NF1 patients, overall survival (OS) was 100% at 5 years and 55.6 ± 24.8% at 25 years while progression-free-survival (PFS) was 50 ± 15.8% at 5 and 20 years. In non-NF1 patients, OS was 96.2 ± 3.8% at 5 years and 87.4 ± 9% at 25-years. 5-year PFS was 53.8 ± 9.8% and 25-year PFS was 49.0 ± 10%. Cumulative PFS was 53 ± 8.3% at 5 years and 49.7 ± 8.4% at 20 years while cumulative OS was 97.2 ± 2.7% at 5 years and 77.5 ± 10.8% at 25 years. 59.5% patients developed post-operative endocrinopathy. Long-term vision was normal in 8.1%, improved in 13.5%, stabilized in 40.5% but worsened in 37.8% patients. Three patients treated with radiotherapy developed second brain tumors.

Conclusion: 25-year OS in this cohort was 77.5% but survivorship carried significant long-term morbidities including radiation-induced second malignant brain tumors.

Keywords: endocrine; long-term; optic glioma; outcomes; second malignancies; symptomatic; visual.