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Optic Pathway Glioma in Type 1 Neurofibromatosis: Review of Its Pathogenesis, Diagnostic Assessment, and Treatment Recommendations

1
Clinical Genetics Unit, Department of Women’s and Children’s Health, University of Padova, 35128 Padova, Italy
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Istituto di Ricerca Pediatrica—IRP, Città della Speranza, 35127 Padova, Italy
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IRCCS-Istituto di Ricovero e Cura a Carattere Scientifico—Fondazione Bietti, 00198 Rome, Italy
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Hematology Oncology Division, Department of Women’s and Children’s Health, University of Padova, 35128 Padova, Italy
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Paediatric Oncology, Blood, Cell, and Cancer Department, Great Ormond Street Hospital, London WC1N 3JH, UK
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Department of Ophthalmology, University of Padova, 35128 Padova, Italy
*
Authors to whom correspondence should be addressed.
Matteo Cassina and Luisa Frizziero contributed equally to this work.
Cancers 2019, 11(11), 1790; https://doi.org/10.3390/cancers11111790
Received: 22 October 2019 / Revised: 12 November 2019 / Accepted: 12 November 2019 / Published: 14 November 2019
Type 1 neurofibromatosis (NF1) is a dominantly inherited condition predisposing to tumor development. Optic pathway glioma (OPG) is the most frequent central nervous system tumor in children with NF1, affecting approximately 15–20% of patients. The lack of well-established prognostic markers and the wide clinical variability with respect to tumor progression and visual outcome make the clinical management of these tumors challenging, with significant differences among distinct centers. We reviewed published articles on OPG diagnostic protocol, follow-up and treatment in NF1. Cohorts of NF1 children with OPG reported in the literature and patients prospectively collected in our center were analyzed with regard to clinical data, tumor anatomical site, diagnostic workflow, treatment and outcome. In addition, we discussed the recent findings on the pathophysiology of OPG development in NF1. This review provides a comprehensive overview about the clinical management of NF1-associated OPG, focusing on the most recent advances from preclinical studies with genetically engineered models and the ongoing clinical trials. View Full-Text
Keywords: type 1 neurofibromatosis; NF1; optic pathway glioma; brain/orbit MRI; clinical trials type 1 neurofibromatosis; NF1; optic pathway glioma; brain/orbit MRI; clinical trials
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Cassina, M.; Frizziero, L.; Opocher, E.; Parrozzani, R.; Sorrentino, U.; Viscardi, E.; Miglionico, G.; Midena, E.; Clementi, M.; Trevisson, E. Optic Pathway Glioma in Type 1 Neurofibromatosis: Review of Its Pathogenesis, Diagnostic Assessment, and Treatment Recommendations. Cancers 2019, 11, 1790.

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