Early radiotherapy preserves vision in sporadic optic pathway glioma. Issue 13 (19th March 2021)

• Record Type: Journal Article
• Authors: Hanania, Alexander N.; Paulino, Arnold C.; Ludmir, Ethan B.; Shah, Veeral S.; Su, Jack M.; McGovern, Susan L.; Baxter, Patricia A.; McAleer, Mary Frances; Grosshans, David R.; Okcu, M. Fatih; Chintagumpala, Murali M.
• Is Part Of: Cancer. Volume 127:Issue 13(2021)
• Page Start: 2358
• Page End: 2367
• Publication Date: 2021-03-19
• Subjects: blindness‐free survival -- early radiation therapy -- optic pathway hypothalamic glioma -- proton beam therapy -- visual acuity; Cancer -- Periodicals; Cancer -- Cytopathology -- Periodicals; 616.99405
• DOI: 10.1002/cncr.33497
• Languages: English
• ISSNs: 0008-543X

Abstract

Abstract : BACKGROUND: Sporadic optic pathway/hypothalamic gliomas represent a unique entity within pediatric low‐grade glioma. Despite favorable survival, location makes treatment difficult and local progression debilitating. This study is a longitudinal assessment of visual acuity (VA) among children treated within the last 2 decades. METHODS: Clinical characteristics were abstracted for patients treated from 2000 to 2018 at Texas Children's Cancer Center in Houston. Ophthalmologic data taken at 3‐ to 6‐month intervals were examined with age‐appropriate VA metrics converted to the LogMAR (logarithm of the minimum angle of resolution) scale. Kaplan‐Meier blindness‐free survival (BFS) curves, calculated as time‐to‐bilateral functional blindness (LogMAR ≥0.8 in both eyes), were calculated for patients receiving early radiation therapy (RT; upfront or as first‐line salvage treatment) or chemotherapy (CT) and evaluated using the log‐rank test. RESULTS: Thirty‐eight patients with a median follow‐up of 8.5 years (range, 2‐17 years) were identified. Median age at diagnosis was 3 years (interquartile range, <1‐6 years). Early RT was administered in 11 patients (29%). Twenty‐seven patients (71%) were treated primarily with CT, initiated at a median age of 3.5 years (range, <1‐11 years). Eight patients in the CT group did eventually require RT secondary to VA loss and following multiple lines of CT. Median age at RT for all patients was 11 years (range, 3‐17 years). BFS rates were 81% at 5 years and 60% at 8 years for CT and 100% at 5 and 8 years for early RT ( P = .017). CONCLUSIONS: In a contemporary cohort, early RT, defined as initial or first‐line salvage therapy, was found to have superior BFS for appropriately selected patients with sporadic optic pathway/hypothalamic gliomas. LAY SUMMARY: Children with low‐grade brain tumors of the optic pathway generally have excellent long‐term survival; however, given the location of these tumors, there can commonly be threatened vision if the tumor grows. Although radiation is generally deferred in children on the basis of legitimate concerns regarding the effects on the developing brain, it may represent a vision‐preserving therapy for well‐selected older patients. Abstract : The treatment paradigm for pediatric optic pathway glioma has mostly shifted from using radiation therapy (RT) as the definitive therapy to favor postponement of RT for secondary and tertiary lines of systemic therapy. Despite excellent patient survival rates, long‐term vision outcomes with chemotherapy are poor. Well‐selected older patients who receive early RT as upfront or first‐line salvage therapy retain meaningful long‐term visual acuity.