We treated a 3-year-old boy with neurofibromatosis who had an optic glioma, intraocular extension with seeding, and iris tumors. On the basis of results of ultrasonography, computed tomography, magnetic resonance imaging, and fine needle aspiration, other intraocular and orbital tumors were excluded. Because of the malignant intraocular aspect, the optic nerve glioma was extirpated. Histologic examination confirmed the diagnosis of an optic nerve glioma with intraocular extension, seeding, and iris nodules. In this juvenile pilocytic astrocytoma with secondary perineural fibrous hyperplasia, several mitoses were found in the orbital and intraocular parts. In the optic canal, three small islands were found that were compatible with the diagnosis of malignant astrocytoma, grade 3. The iris nevi appeared as iris pits and not like the Lisch nodules typical of neurofibromatosis. Café au lait spots were present on the skin. The family history was positive for neurofibromatosis. The results of this study demonstrate that optic gliomas are true astrocytomas and not hamartomas, and have a continuous scale from benign to malignant differentiation.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/0002-9394(89)90868-4 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Retinoblastoma and beyond: pediatric orbital mass lesions.
Neuroradiology
December 2024
Department of Neuroradiology, Istituto Giannina Gaslini, Genoa, Italy.
Various space occupying lesions can arise in the orbit, ranging from developmental anomalies to malignancies, and many of the diseases occurring in children are different from the pathologies in the adult population. As the clinical presentation is frequently nonspecific, radiologic evaluation is essential for lesion detection and characterization as well as patient management. While orbital masses may in some cases involve multiple compartments, a simple compartmental approach is the key for the diagnosis on imaging studies, and MRI is the modality of choice.
View Article and Find Full Text PDFRetinal Microstructural Changes Reflecting Treatment-Associated Cognitive Dysfunction in Patients with Lower-Grade Gliomas.
Ophthalmol Sci
July 2024
Department of Ophthalmology & Vision Science, University of California Davis, Sacramento, California.
Purpose: To determine whether microstructural retinal changes, tumor features, and polymorphism are correlated with clinically detectable treatment-associated cognitive dysfunction (TACD) in patients with lower-grade gliomas.
Design: Cohort study.
Participants And Controls: Sixteen patients with lower-grade glioma at a United States academic ophthalmology department between January 2021 and November 2023.
Pathological findings in enucleated eyes of patients with neurofibromatosis type 1: report of a case with 15-year follow-up and review of 14 patients in the literature.
BMC Ophthalmol
August 2024
Department of Neurological Surgery, General Medical Center, Kawasaki Medical School, Okayama City, 700-8505, Japan.
Article Synopsis
- Iris nodules are common in neurofibromatosis type 1, but other eye issues are rare; this study highlights a patient with a phthisic eye who had it removed for cosmetic reasons after 15 years.
- The case focuses on a 17-year-old male who underwent multiple surgeries due to complications, including total retinal detachment, with the enucleated eye showing signs of massive retinal gliosis and no apparent neurofibromas in some areas.
- A review of 14 similar cases revealed a pattern of eye conditions like glaucoma and neurofibromas in certain structures, indicating various ocular manifestations of neurofibromatosis type 1.
Ophthalmologic Findings in Children with Neurofibromatosis Type 1.
Neuroophthalmology
February 2024
Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
The purpose of this study was to evaluate the ophthalmologic findings in children with neurofibromatosis type 1 (NF1) and compare these findings in eyes with and without optic pathway gliomas (OPGs). We carried out a retrospective chart review of children with NF1. We recorded demographic characteristics, clinical manifestations of disease, and ophthalmologic findings including visual acuity, intraocular pressure, cup-to-disc ratio, visual field testing, and optical coherence tomography findings.
View Article and Find Full Text PDF[Orbital tumors].
Radiologie (Heidelb)
March 2024
Aretaieion Universitätsklinkum für Radiologie, Universität von Athen, Athen, Griechenland.
Article Synopsis
- Orbital tumors are various diseases affecting the eye area, and early detection is crucial for effective treatment, especially for malignant cases.
- Neuroradiological imaging, particularly MRI, is key for diagnosing these tumors, with adults often experiencing lymphoproliferative diseases and children more frequently dealing with dermoid cysts and optic gliomas.
- Specific tumors like optic glioma, which is a type of pilocytic astrocytoma, and retinoblastoma, an aggressive childhood tumor, have distinct characteristics that help doctors narrow down their diagnoses based on patient age, tumor location, and imaging results.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!