A 3-year-old boy was referred because of the presence of a non-pigmented, highly vascular mass with tapioca appearance involving the superior portion of the left iris. Iris fluorescein angiography revealed early hyperfluorescence of the iris tumor with diffuse, intense late leakage of dye throughout the entire iris, not just in the region of the tumor. Cytopathologic examination revealed Touton giant cells and the presence of histiocytes, confirming the diagnosis of iris juvenile xanthogranuloma. The patient was prescribed topical prednisolone acetate, and the lesion resolved during the next 3 months. Fluorescein angiography may be useful in differentiating iris juvenile xanthogranuloma from malignant or non-inflammatory benign iris lesions.
From the Oncology Service (CJD, CLS, AM, MEM, JAS), Wills Eye Hospital, Thomas Jefferson University; and the Department of Pathology (HE), Fox Chase Cancer Center, Philadelphia, Pennsylvania.
Supported in part by the Pan American Ophthalmology Foundation and Retina Research Foundation (MEM), the Eye Tumor Research Foundation, Philadelphia, Pennsylvania (CLS), the Rosenthal Award of the Macula Society (CLS), and the Paul Kayser International Award of Merit in Retina Research, Houston, Texas (JAS).
Address correspondence to Carol L. Shields, MD, Oncology Service, Suite 1440, Wills Eye Hospital, 840 Walnut Street, Philadelphia, PA 19107.
Received: November 12, 2005
Accepted: March 22, 2006
Juvenile xanthogranuloma is one of the most common forms of non-Langerhans cell histiocytosis in infants and young children and is characterized by transient erythematous nodular cutaneous lesions.1 Iris involvement can manifest as diffuse thickening or a tumorous nodule and can lead to hyphema and secondary glaucoma.2,3 We report our experience with fluorescein angiography of a cytologically proven case of iris juvenile xanthogranuloma.4
A 3-year-old boy was referred to the Oncology Service at Wills Eye Hospital in April 2004 because of redness in the left eye for 2 weeks. The patient was otherwise healthy, with no current or previous skin lesions. On ocular examination, visual acuity was 20/25 in both eyes. The anterior segment of the right eye was normal. Slit-lamp examination of the left eye showed a nonpigmented, highly vascular mass with tapioca appearance involving the superior portion of the iris (Fig. 1). Dilated episcleral vessels were noted in the vicinity of the mass. The anterior chamber was deep and quiet. There was no hyphema, corectopia, iris ectropion, keratitic precipitates, or tumor seeds. Our differential diagnosis included juvenile xanthogranuloma versus atypical iris nevus or melanoma.
Figure 1. Anterior Segment Photograph Shows an Amelanotic, Intensely Vascular Mass Affecting the Superior Portion of the Iris.
Under general anesthesia, Shiøtz tonometry demonstrated intraocular pressures of 14 and 12 mm Hg in the right and left eye, respectively. Intravenous fluorescein angiography of the iris revealed normal iris vasculature of the right eye (Fig. 2A) and early hyperfluorescence of the iris tumor in the left eye (Fig. 2B) with diffuse, intense late leakage of dye, not only from the tumor, but also from the uninvolved inferior iris (Fig. 2C). Fine-needle aspiration biopsy of the iris lesion using a 27-gauge needle was performed. Cytologic examination of the iris mass revealed the presence of histiocytes, spindle and stellate shaped cells, Touton giant cells, and scattered eosinophils and lymphocytes (Fig. 3).
Figure 2. (A) Intravenous Fluorescein Angiography of the Right Eye Shows the Normal Radial Iris Vessels with No Dye Leakage. (B) Early Phase Fluorescein Angiogram of the Left Eye Reveals Diffuse, Intense Hyperfluorescence of the Superior Iris Corresponding to the Clinically Visible Mass. the Inferior Portion of the Iris also Shows Early Hyperfluorescence even Though It Clinically Appeared Normal. (C) Late Phase Fluorescein Angiogram Shows Diffuse Hyperfluorescence of the Entire Iris, Both Superiorly and Inferiorly, with Silhouettes of the Larger Tumor Vessels in the Superior Iris.
Figure 3. Cytopathology Showing Presence of Irregular Histiocytes, Few Lymphocytes, and a Touton Giant Cell (arrow) (papanicolaou Stain).
Immunocytochemical stains were positive for vimentin and histiocyte markers (CD68, HAM 56), and negative for melanoma markers (HMB 45, Melan A) and cytokeratin (AE1/AE3). These were consistent with the diagnosis of juvenile xanthogranuloma. The patient was treated with topical prednisolone acetate with resolution of the iris mass over 3 months.
It has been claimed that intravenous fluorescein angiography may be helpful in differentiating benign and malignant melanocytic iris lesions.5,6 Based on an analysis of 23 eyes, Jakobiec et al. stated that benign melanocytic lesions show no tendency to dye uptake or leakage in any phase of the angiogram, whereas malignant melanocytic lesions show early fluorescence and increasing fluorescence in the late phases with additional features of ectropion and corectopia.6 These observations may not pertain to inflammatory conditions because our patient with a benign iris juvenile xanthogranuloma showed intense leakage of fluorescein on intravenous fluorescein angiography, not just in the region of the tumor but throughout the entire iris.
Hyperfluorescence of iris juvenile xanthogranuloma has been a source of misdiagnosis. In one case reported by Hamburg, an eye of a 22-year-old man harboring iris juvenile xanthogranuloma was enucleated because amelanotic melanoma was suspected based on rapid tumor growth and intense leakage on iris fluorescein angiography.7
Based on the iris fluorescein angiographic pattern seen in our patient, we believe that intravenous fluorescein angiography may be useful in the evaluation of children with amelanotic iris lesions. Iris juvenile xanthogranuloma should be considered in eyes showing hyperfluorescence and late leakage of an iris mass associated with diffuse leakage of the clinically uninvolved iris. Further study of the utility of intravenous fluorescein angiography in the assessment of iris tumors is warranted.
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