On the knowledge of solitary juvenile xanthogranuloma of the eyelid: a case series and literature review

Abstract

Purpose Solitary eyelid juvenile xanthogranuloma (JXG) is extremely rare, and there is limited literature on its clinical features and treatment outcomes. Here, we present a case series and comprehensive review of the literature on patients with isolated eyelid JXG. Methods We systematically extracted data from our institution’s records of isolated eyelid JXG cases and conducted a search for additional cases from the literature utilising the PubMed, Wanfang, and Chinese National Knowledge Infrastructure (CNKI) databases. Patients with JXG were analysed with respect to age, sex, clinical presentation, therapy, and outcome. Group comparisons were performed. Results Thirty-two patients (including 13 at our institution and 19 from prior publications) were identified. The median age at first presentation was higher in current patients than in the patients from the published cases (median 9 years, range 1.2 to 47.0 years; median 2 years, range 0.5 months to 46.0 years, respectively, P = 0.014). Of the patients who had known characteristics, no significant differences were observed between the two groups in terms of sex, affected eye, eyelid site, type of cutaneous involvement, or duration of symptoms (each P > 0.05). Seventeen (54.8%) patients were male. The most common lesion location was the upper eyelid (n = 10, 62.5%). Twenty-four (75.0%) cutaneous lesions had full-thickness skin involvement; 8 (25.0%) subcutaneous masses had a chalazion-like appearance. Histologically, the JXG masses were characterised by Touton giant cells with inflammatory cells. Additionally, there was no significant difference in treatment modalities between the two groups (P = 0.072), and 24 (75.0%) patients underwent surgical excision. The overall recurrence-free survival was 3.6 to 52.8 (median 27.0) months in the current patients. For published cases with available follow-up information, there was no recurrence in 10 cases and improvement in 1 case, with a median follow-up of 9.5 months. Conclusion Solitary eyelid JXG is a rare clinical entity and should be included in the differential diagnosis of eyelid mass lesions in patients of all age groups. Surgical excision is often selected for efficient treatment and to obtain an excisional biopsy.

Adult onset xanthogranuloma of the eyelid

“…Two case series concerning eye involving JXG include patients with eyelid involvement presenting in their third and fifth decades, however these do not elaborate on the clinical features of these presentations. 3 , 22 Several individual case reports have discussed adult-onset JXG arising in other ophthalmic structures including the iris, 23 limbus 24 , 25 , 26 , 27 and orbit. 28 , 29 , 30 …”

“…Recurrence of these lesions is rare, with most series of eye involving JXG reporting no recurrence after 15–27 months. 3 , 16 Recurrence requiring multiple surgical excisions to achieve regional tumor control has been reported. Nishina et al 31 describe excision of a mass of JXG and associated subcutaneous tissue, including orbital septum, and reconstruction with a pre-auricular skin graft resulting in two year recurrence free follow up.…”

“… 2 Ophthalmic involvement can occur at the orbit, eyelid, iris, retina, choroid and the optic nerve, with the majority of cases being diagnosed within the first year of life. 3 Surgical excision is often successful at producing recurrence free outcomes, however cases have been reported of multiple resection attempts for recurrent disease. 4 Ocular JXG-like lesions rarely occur in the third decade or later in life and are defined as adult onset xanthogranuloma (AXG).…”