A Case of Juvenile Xanthogranuloma of the Corneoscleral Limbus

ABSTRACT

PURPOSE: Juvenile xanthogranuloma usually affects the anterior uvea before the age of 1 year, and it may less frequently involve the corneoscleral limbus. We report a case of juvenile xanthogranuloma of the corneoscleral limbus in a 7-year-old boy. METHODS: A 7-year-old boy was referred for the evaluation of a painless limbal mass in the left eye, noticed 1 month previously. On examination, the visual acuity was 16/20 (OD) and 10/20 (OS) without correction, and the corrected visual acuity was 20/20 in both eyes. The other ocular findings were normal, except for an elevated, well circumscribed, smooth surfaced mass measured 9x6 mm at the upper temporal corneoscleral limbus of the left eye. After the excision of the mass, the frozen biopsy and the immunohistochemical staining for CD68 and S100 protein were performed. RESULTS: The lesion consisted of a red-brown, vascular, and raised mass. Histopathologically, there was a chronic granulomatous inflammation with stromal fibrosis, which included many giant cells immunoreactive for CD68 and S100 protein. There was no evidence of recurrence during follow-up period of 5 months and 20 months. CONCLUSIONS: The case was diagnosed as juvenile xanthogranuloma by the clinical and histopathologic findings. The juvenile xanthogranuloma of the corneoscleral limbal involvement is very rare and it can be treated with the surgical excision for the purpose of improvement of cosmetic problems without any recurrence. It seems to have very good prognosis when completely excised.