Treatment during a developmental window prevents NF1-associated optic pathway gliomas by targeting Erk-dependent migrating glial progenitors

2021 ◽  
Author(s):  
Emmanuelle S. Jecrois ◽  
Wang Zheng ◽  
Miriam Bornhorst ◽  
Yinghua Li ◽  
Daniel M. Treisman ◽  
...  
Keyword(s):  
Optic Pathway ◽  
Glial Progenitors ◽  
Optic Pathway Gliomas ◽  
Developmental Window

scholarly journals Neurosurgical experience of managing optic pathway gliomas

2021 ◽  
Author(s):  
Ciaran Scott Hill ◽  
Mehdi Khan ◽  
Kim Phipps ◽  
Katherine Green ◽  
Darren Hargrave ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Surgical Resection ◽  
Visual Function ◽  
Optic Pathway ◽  
Who Grade ◽  
Radiographic Findings ◽  
Surgical Interventions ◽  
Hypothalamic Dysfunction ◽  
Cerebrospinal Fluid Diversion ◽  
Optic Pathway Gliomas

Abstract Background Optic pathway gliomas (OPGs), also known as visual pathway gliomas, are debilitating tumors that account for 3–5% of all pediatric brain tumors. They are most commonly WHO grade 1 pilocytic astrocytomas and frequently occur in patients with neurofibromatosis type 1. The location of these tumors results in visual loss and blindness, endocrine and hypothalamic dysfunction, hydrocephalus, and premature death. Their involvement of the visual pathways and proximity to other eloquent brain structures typically precludes complete resection or optimal radiation dosing without incurring significant neurological injury. There are various surgical interventions that can be performed in relation to these lesions including biopsy, cerebrospinal fluid diversion, and partial or radical resection, but their role is a source of debate. This study catalogues our surgical experience and patient outcomes in order to support decision-making in this challenging pathology. Methods A retrospective review of all cases of OPGs treated in a single center from July 1990 to July 2020. Data was collected on patient demographics, radiographic findings, pathology, and management including surgical interventions. Outcome data included survival, visual function, endocrine, and hypothalamic dysfunction. Results One hundred twenty-one patients with OPG were identified, and 50 of these patients underwent a total of 104 surgical procedures. These included biopsy (31), subtotal or gross total resection (20 operations in 17 patients), cyst drainage (17), Ommaya reservoir insertion (9), or cerebrospinal fluid diversion (27). During the study period, there was 6% overall mortality, 18% hypothalamic dysfunction, 20% endocrine dysfunction, and 42% had some cognitive dysfunction. At diagnosis 75% of patients had good or moderate visual function in at least one eye, and overall, this improved to 83% at the end of the study period. In comparison the worst eye had good or moderate visual function in 56%, and this reduced to 53%. Baseline and final visual function were poorer in patients who had a surgical resection, but improvements in vision were still found—particularly in the best eye. Discussion/conclusion OPG are debilitating childhood tumor that have lifelong consequences in terms of visual function and endocrinopathies/hypothalamic dysfunction; this can result in substantial patient morbidity. Decisions regarding management and the role of surgery in this condition are challenging and include cerebrospinal fluid diversion, biopsy, and in highly select cases cystic decompression or surgical resection. In this paper, we review our own experience, outcomes, and surgical philosophy.

Follow-Up of Optic Pathway Gliomas in Children with Neurofibromatosis Type 1

1994 ◽  
Vol 25 (06) ◽  
pp. 295-300 ◽  
Author(s):  
Ch. Kuenzle ◽  
M. Weissert ◽  
E. Roulet ◽  
H. Bode ◽  
S. Schefer ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Optic Pathway ◽  
Optic Pathway Gliomas

Optic Pathway Gliomas in Neurofibromatosis

2008 ◽  
Vol 32 (9) ◽  
pp. 820-824 ◽  
Author(s):  
David W. Dunn ◽  
VALERIE PURVIN
Keyword(s):  
Optic Pathway ◽  
Optic Pathway Gliomas

Non-inferiority of a non-gadolinium-enhanced magnetic resonance imaging follow-up protocol for isolated optic pathway gliomas

2021 ◽  
Author(s):  
Ezekiel Maloney ◽  
Francisco A. Perez ◽  
Ramesh S. Iyer ◽  
Randolph K. Otto ◽  
Jason N. Wright ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Optic Pathway ◽  
Resonance Imaging ◽  
Optic Pathway Gliomas

scholarly journals Longitudinal Change of Circumpapillary Retinal Nerve Fiber Layer Thickness in Children With Optic Pathway Gliomas

2015 ◽  
Vol 160 (5) ◽  
pp. 944-952.e1 ◽  
Author(s):  
Robert A. Avery ◽  
Avital Cnaan ◽  
Joel S. Schuman ◽  
Carmelina Trimboli-Heidler ◽  
Chieh-Li Chen ◽  
...  
Keyword(s):  
Nerve Fiber ◽  
Layer Thickness ◽  
Retinal Nerve Fiber Layer ◽  
Longitudinal Change ◽  
Optic Pathway ◽  
Fiber Layer ◽  
Nerve Fiber Layer ◽  
Nerve Fiber Layer Thickness ◽  
Optic Pathway Gliomas ◽  
Retinal Nerve Fiber

Advancements in the Management of Optic Pathway Gliomas

2019 ◽  
pp. 47-60
Author(s):  
Sahas Narain ◽  
Ashwini Kini ◽  
Aparna Ramasubramanian
Keyword(s):  
Optic Pathway ◽  
Optic Pathway Gliomas

Optic Pathway Gliomas

2005 ◽  
pp. 579-586 ◽  
Author(s):  
Cian J. O'Kelly ◽  
James T. Rutka
Keyword(s):  
Optic Pathway ◽  
Optic Pathway Gliomas
Sign in / Sign up

Export Citation Format

Share Document