LGG-05. OPTIC PATHWAY GLIOMA VOLUME PREDICTS VISUAL ACUITY LOSS AND AXONAL LOSS IN CHILDREN WITH NEUROFIBROMATOSIS TYPE 1. Issue 2 (22nd June 2018)

• Record Type: Journal Article
• Authors: Avery, Robert; Mansoor, Awais; Liu, Grant; Trimboli-Heidler, Carmelina; Ying, Gui-Shuang; Eltoukhy, Nadine; Packer, Roger; Fisher, Michael; Linguraru, Marius
• Is Part Of: Neuro-oncology. Volume 20:Issue 2(2018)supplement 2
• Page Start: i105
• Page End: i105
• Publication Date: 2018-06-22
• Subjects: Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noy059.348
• Languages: English
• ISSNs: 1522-8517

Abstract

Abstract: OBJECTIVE: To determine whether MRI measures of tumor volume can identify children who are at the greatest risk of visual acuity (VA) loss and axonal loss from their optic pathway glioma secondary to Neurofibromatosis type 1 (NF1-OPG). METHODS: Children with NF1-OPGs enrolled in an ongoing prospective study of VA were eligible if they had undergone 3-Tesla MRI that included a T1-weighted volumetric sequence and concurrent optical coherence tomography (OCT). The volume (ml) of proximal anterior visual pathway (AVP) and its individual components (optic nerve, chiasm and proximal tract) were measured from the T1 sequence using our semi-automated algorithm. OCT measured the circumpapillary retinal nerve fiber layer (cpRNFL) thickness, a biomarker of axon integrity. VA and OCT were reported on a per-eye basis. RESULTS: Twenty-eight study eyes (14 children) with NF1-OPGs met inclusion criteria, of which 57% (N=16) had abnormal VA and 43% (N = 12) had normal VA. Total AVP volume > 1.75 ml detected vision loss with 87% sensitivity and 90% specificity. Linear regression demonstrated that for every 1 ml increase in optic chiasm volume or AVP volume, the cpRNFL declined by 6 or 10 microns, respectively. CONCLUSION: Volumetric measures of NF1-OPGs accurately identified children with VA loss. Larger tumor volumes were associated with more severe axonal injury and greater VA loss. Both tumor volume and cpRNFL thickness may be helpful in making treatment decisions in children with NF1-OPGs that present with abnormal VA loss or are uncooperative with VA testing.