Original articleJuvenile Xanthogranuloma Involving the Eye and Ocular Adnexa: Tumor Control, Visual Outcomes, and Globe Salvage in 30 Patients
Section snippets
Methods
This retrospective interventional case series included 30 patients diagnosed with ocular JXG at the Ocular Oncology Service of Wills Eye Hospital between December 1981 and November 2014. Institutional review board approval was obtained at Wills Eye Hospital. Four of the 30 patients have been reported.17, 18, 19, 20
Patient data were extracted retrospectively from medical records and included patient age at diagnosis (months), gender (male or female), race (white, African American, Hispanic, or
Results
Patient demographic data are listed in Table 1. Mean patient age at presentation was 51 months (median, 15 months; range, 1–443 months), and the most common presenting symptom was eye redness (12/30, 41%) followed by iris mass (4/30, 13%), hyphema (4/30, 13%), and conjunctival mass (4/30, 13%). Juvenile xanthogranuloma was unilateral in 29 patients (29/30, 97%), and only 1 patient had presumed bilateral disease, involving the choroid. Three of 30 patients (10%) had concurrent cutaneous lesions
Discussion
Juvenile xanthogranuloma is a rare disorder that predominately affects the skin. Although a minority of patients will have ocular involvement, recognition of this condition is important because treatment delay can lead to potentially blinding complications such as glaucoma and hyphema.9 In a survey of 3680 iris tumors referred to a tertiary care ocular oncology service, 449 tumors (12%) occurred in children (≤20 years old), and of those, 14 (3%) were xanthogranulomatous lesions.21 The diagnosis
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2023, American Journal of Ophthalmology Case ReportsCitation Excerpt :There are rare reports of CNS, liver, spleen and lung involvement.15 In a single centre study of 30 patients, the iris was the most frequently involved ophthalmic structure, accounting for 68% of cases, followed by conjunctival and eyelid lesions contributing to 19% and 6% of cases respectively.16 This has been reflected in other series,17,18 however one study found the eyelid to be the most commonly affected site.2
Developmental Anomalies of the Cornea and Iris in Neonates
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2022, Annals of Diagnostic PathologyCitation Excerpt :Three risk factors for ocular involvement include cutaneous micronodular JXG (lesions ≤10 mm vs. macronodular [>10 mm] lesions), multiple cutaneous lesions, and age of ≤2 years. [34] Often asymptomatic but erythema, followed by hyphema, iris mass and conjunctival mass are the various clinical manifestations. [37] In contrast to cutaneous JXG with the capacity for spontaneous resolution, an intraocular lesion does not have that potential and can lead to secondary glaucoma with loss of vision. [15,34,37]
Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
C.L.S.: Support – Eye Tumor Research Foundation, Philadelphia, Pennsylvania. The funders had no role in the design and conduct of the study; in the collection, analysis, and interpretation of the data; and in the preparation, review, or approval of the manuscript. C.L.S. has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Author Contributions:
Conception and design: Samara, Khoo, Say, Saktanasate, Eagle, J.A. Shields, C.L. Shields
Data collection: Samara, Khoo, Say, Saktanasate, C.L. Shields
Analysis and interpretation: Samara, Khoo, Say, Saktanasate, Eagle, C.L. Shields
Obtained funding: Not applicable
Overall responsibility: Samara, Khoo, Say, Saktanasate, Eagle, J.A. Shields, C.L. Shields