3
Xanthogranuloma of the corneosclerallimbus in an adult Jason J. Wang, MD; Deepak P. Edward, MD; Elmer Tu, MD E pibulbar xanthogranulomas are uncommon lesions that occur most frequently in children under 5 years of age.l They are very rare in adults, with only four reports in teenagers2-4 and one report in a 30-year-old; 5 in all cases the xanthogranuloma pre- sented as a solitary limbal lesion. These observations concur with the dermatologic literature, which sug- gests that adult xanthogranulomas are unusual head and neck lesions that, as a rule, present as a solitary, slow-growing mass.6 We describe a 45-year-old man with a xanthogranuloma of the corneosclerallimbus. CASE REPORT A 45-year-old white man presented with a painless mass at the right inferior limbus that had been noticed 1 week before presentation. His medical history and past ocular history were noncontributory. His best cor- rected visual acuity was 20/20 in either eye. No skin lesions were noted on external examination. Slit-lamp examination showed a yellow-pink mass measuring 6 mm by 4 mm by 3 mm straddling the inferior limbus (Fig. 1 ). The surface and edge of the lesion had a crys- talline appearance. Ultrasound biomicroscopy demon- strated extension into the corneal stroma with areas of peripheral high reflectance and central lower reflec- tance (Fig. 2). Excisional biopsy was performed, with no recurrence over 5 months of follow-up. No sys- temic skin lesions were noted. Microscopic examination showed richly vascular- ized fibrovascular tissue lined by conjunctival epithe- lium. The lesion was highly cellular and was infiltrat- ed by lipid-laden histiocytes and spindle-shaped From the Department of Ophthalmology, University of Illinois at Chicago, Chicago, Ill. Originally received Sept. 12, 2000 Accepted for publication Feb. 20, 2001 Reprint requests to: Dr. Deepak P. Edward, 1855 W Taylor St., Chicago IL 60612-7243, USA; fax (312) 243-3937; [email protected] Can j Ophthalmol 200 I;36:275-7 Adult xanthogranuloma-Wang et al fibroblasts with scattered aggregates of lymphocytes and plasma cells (Fig. 3). It extended into the deep corneal stroma. The deeper portions contained numer- ous Touton giant cells (Fig. 3). Stains for bacteria, fungi and acid-fast bacilli were negative. The histio- cytes stained strongly with macrophage marker CD-68 and were negative for S-100 (Fig. 4). COMMENTS The ophthalmic presentation was quite typical in our patient and appears to be similar in children and adults.l-5 Systemic disease has been reported only in very young children and is not seen with adult epibulbar xanthogranulomas with skin involvementP Clinically, the differential diagnosis of this limbal mass in an adult includes dermoid, dermolipoma, fibrous histiocytoma, Langerhans cell histiocytosis, amelanotic nevus or mel- anoma, lymphoproliferative disorders, fibroma, myxo- ma and neurofibroma. 4 Histologically, the lesion contains large numbers of lipid-containing histiocytes and Touton giant cells. Giant cells are typically more abundant in the deeper parts of the lesion. 6 Xanthogranulomas are a form of non-Langerhans cell histiocytosis, and the cells are monocyte-macrophage in origin. They label strongly with macrophage markers, such as CD-68 and HAM- 56;6 S-100 immunoreactivity, which is helpful in the diagnosis of Langerhans cell histiocytosis, is typically absent.6 A number of entities are described that share clinical and histologic features with juvenile xan- thogranulomas. Entities that are included in the histo- logic differential diagnosis relevant to our case include fibrous histiocytoma, necrobiotic xanthogranuloma and Erdheim-Chester disease. Lipid-laden Touton giant cells may be seen in all these lesions. Fibrous histiocy- tomas may in some instances be difficult to distinguish from xanthogranulomas. However, the former tend to have larger numbers of spindle-shaped fibrocytes that demonstrate a storiform pattern. Also, more extensive infiltrate of chronic inflammatory cells, as seen in our case, is more typical of xanthogranuloma. 8 Necrobiotic xanthogranuloma, unlike juvenile xanthogranuloma, is 275
