Juvenile xanthogranuloma is a benign histiocytic inflammatory condition occurring in infants and young children.1 Usually, the skin lesions are slightly elevated, yellowish orange in color, and undergo spontaneous regression in the majority of cases.2 Ocular involvement may include discrete or diffuse lesions of the iris, ciliary body, epibulbar tissues, or orbit. A rare case of retinal, choroidal, and optic nerve involvement has been reported.3 The vast majority of cases are unilateral.
Limbal xanthogranulomas are rare. To date, we are aware of five reported cases of histologically proven xanthogranulomas occurring at the limbus.4"6 This is in addition to other yellow lesions at the limbus with lipidbearing histiocytes without giant cells.7"11 We now present three cases of limbal xanthogranulomas, all of which were studied histologically.
Case 1: A 16-year-old female complained of a month-long foreign body sensation in her left eye. On examination there was a solid, fleshy tumor, measuring 2x2 mm, present at the limbus in the inferotemporal region (Fig 1). Visual acuity and ocular examination were otherwise normal, as were routine biochemical investigations. The lesion was completely excised.
Histological examination showed a granulomatous lesion, with numerous giant cells present, some of which were of Touton type, thus confirming the diagnosis of xanthogranuloma (Fig 2).
After a 2-year follow-up there has been no recurrence.
Case 2: A 16-year-old male presented to the clinic with a 2-month history of a painless swelling present at the lower limbus of the right eye (Fig 3). Systemic and ocular findings were otherwise normal. The lesion measured 6x3x5 mm and was yellowish orange in color. It extended to approximately 4 mm onto the cornea and a similar distance onto the sclera. It was excised flush with the corneal surface and was submitted for histological examination. Microscopy showed the presence of numerous histiocytes and Touton giant ceils. Electron microscopy and fat stains of the specimen confirmed the presence of large amounts of fat.
During the ensuing 6 months, the lesion recurred and, although smaller than the original tumor, it was not cosmetically acceptable. As there was considerable fat deposition in the anterior stroma of the involved cornea, it was thought unlikely that a course of systemic steroids would clear the affected area. Topical steroids had been prescribed, but with no effect. The patient, therefore had a superficial keratectomy with insertion of a lamellar graft. Histological examination of the recurrence once again confirmed the diagnosis of xanthogranuloma. After a 5year follow-up the graft has remained clear, with no evidence of recurrence (Fig 4).
FIGURE 1: Limbol xanthogranuloma.
FIGURE 2: Touton giant cell (arrowed) beneath corneal epithelium. (Hematoxylin-eosin X 79).
FIGURE 3: Large limbal xanthogranuloma.
FIGURE 4: Case 2, 5 years after lamellar keratoplasty.
Case 3: A 5-year-old female had a 1 -month history of a painless swelling at the upper limbus of her right eye. On examination, a pink fleshy tumor, measuring 3x4 mm was noted. Once again, complete ocular examination was otherwise normal. The lesion was excised and microscopic examination showed numerous histiocytes and giant cells, including Teuton cells.
One year following excision, the lesion recurred and is cosmetically unacceptable. The patient is expected to have further excision of the lesion with insertion of a lamellar graft.
Each of the corneal lesions demonstrated a similar histological appearance and were characterized by extensive stromal replacement by numerous multi-nucleate giant cells. Many of the giant cells were of the Touton type, having centrally located nuclei. Numerous lymphocytes were also present elsewhere. Capillary endothelial cells were extremely prominent. There was marked proliferation of stromal fibroblasts and the overlying epithelium demonstrated well marked squamatization with spongiosis. Numerous giant cells were also present within the overlying epithelial layer. Eosinophils were not prominent.
Ultra-structural examination demonstrated extensive intracytoplasmic lipid droplets, but no evidence of birbeck granules. Examination of the biopsies under polarized light failed to reveal any birefringement material. The appearances were those of typical xanthogranulomata.
The ophthalmic manifestations of xanthogranuloma are quite varied. Zimmerman has provided the most comprehensive review of the topic to date and has stressed that unless the condition is recognized early and appropriate treatment commenced, lesions involving the iris will result in progressive loss of vision by way of secondary glaucoma from recurrent hyphema.2
Xanthogranulomas occurring at the limbus are far less common and should not pose a threat to vision. However, they can be troublesome both from a cosmetic point of view and from the associated foreign body sensation which they produce. Typically, they are said to occur in the very young.2 Our patients, however, were older, one patient being 5 years of age, while the other two were 16 years of age at presentation.
These three cases were cases of localized xanthogranulomatous disease, with no evidence of any systemic disorder. The majority of previously reported cases of limbal xanthogranulomata were also unassociated with systemic evidence of xanthogranulomatous disease. This would suggest that limbal xanthogranuloma is indeed a truly localized process. This is one of the reasons why we feel a local form of treatment is best suited for these patients.
Previously reported cases of limbal xanthogranuloma have all been treated either by simple excision or excision combined with radiotherapy.4"6 Those with extension onto the cornea, which were treated with simple excision, tended to recur.5 We recommend that cases should have a superficial keratectomy performed, with insertion of a lamellar graft. This allows complete excision, thereby decreasing the chance of recurrence and the avoidance of pseudo-pterygia and unsightly scars.
1. Helwig EB, Hackney VC. Juvenile xanthogranuloma (neoxanthoendothelioma). Am J Pathol. 1954;30:625-626.
2. Lever WF, Schaumburg-Lever G. Histopathology of the Skin, 6th ed. Philadelphia, PA: JB Lippincott; 1963:398-399.
3. Wertz FD, Zimmerman LE, McKeown CA, et al. Juvenile xanthogranuloma of the optic nerve, disc, retina and choroid. Ophthalmology. 1982;89:1331-1335.
4. Zimmerman LE. Ocular lesions of juvenile xanthogranuloma. Am J Ophthalmol. 1965;60:1011-1035.
5. Cogan DG, Kuwabara T, Parke D. Epibulbar nevoxanthoendothelioma. Arch Ophthalmol. 1958;59:717-721.
6. Nordentoft B, Anderson SR. Juvenile xanthogranuloma of the cornea and conjunctiva. Acta Opkt halmol. 1967;45:720-726.
7. Paludi JE, Kenyon K, Green WR. Fibrous histiocytoma of the corneoscleral limbus. Am J Ophthalmol. 1975;80:619-624.
8. Grayson M, Pieroni D. Solitary xanthoma of the limbus. Br J Ophthalmol. 1970;54:562-564.
9. Jacobiec FA. Fibrous histiocytoma of the corneoscleral limbus. Am J Ophthalmol. 1974;78:700-706.
10. Liebman SD, Crocker AC, Geiser CF. Corneal xanthomas in childhood. ArcA Ophthalmol. 1966;76:221-229.
11. Litricin O. Fibrous histiocytoma of the corneosclera. ArcA Ophthalmol. 1983;101:426-429.