A Case of Adult Limbal Xanthogranuloma

Abstract

When the tumor gradually became larger, we excised it for a precise diagnosis, which showed that it was JXG.The present case is rare because (1) only six cases of JXG involving the corneoscleral limbus have been previously reported 5 as occurring in patients at less than 5 years of age; 5 and (2) the extensive time course of the lesion was photographed.Of the 18 reported patients with JXG involving the corneoscleral limbus, three patients underwent partial excision. 5 In all three cases, the tumor recurred. In these recurring cases, all tumors were located on the lower side of the limbus. Recurrence may be related to the choice of surgery and the location of the tumor. It has been the main choice of therapy to totally excise the tumor, since complete excision decreases the chance of limbal JXG recurrence. 3,5 In our case, observation 12 months after the surgery has not shown any evidence of tumor recurrence, but we will have to continue to follow this patient.Solitary JXG affecting the corneoscleral limbus is a rare form of limbal lesion. If such a lesion becomes enlarged and extends to the stromal cornea rapidly, total excision is required, and occasionally, lamellar keratoplasty.JXG should be considered in the differential diagnosis of any lesions occurring in the limbus.Juvenile xanthogranuloma (JXG) is an uncommon benign skin disorder usually seen in infants and children, occurring a b c Figure 2. a The tumor is composed of a mixture of acute and chronic infl ammatory cells. Lipid-laden vacuoles are present. H&E staining, bar = 200 µm. b High-power fi eld of the tumor. Lipid-laden vacuoles are intermixed with acute and chronic infl ammatory cells. Arrows indicate Touton giant cells. H&E staining, bar = 50 µm. c Immunohistochemical staining for CD68. Positively stained cells are dispersed throughout the tumor. Bar = 50 µm.lymphoproliferative disorders, primary xanthomatous or histiocytic disorders, and malignant neoplasms such as squamous cell carcinoma. 5 Our fi rst impression was that this tumor was a dermoid cyst, because the surface was smooth.

New Treatment Option for Adult-onset Limbal Xanthogranuloma

“…In the 22 documented cases of limbal JXG in children or adults, 1,7,12-14 only 3 have been treated with topical corticosteroids, one infant case resulting in slow regression 15 the other 2 eventually requiring excision. 7,16 Irradiation without short-term recurrence was used in 2 cases, one combined with resection. 17 Surgical removal of the lesion was the only curative option in 20 of the 22 patients 15,7 requiring lamellar grafting in a number of cases.…”

“…7,16 Irradiation without short-term recurrence was used in 2 cases, one combined with resection. 17 Surgical removal of the lesion was the only curative option in 20 of the 22 patients 15,7 requiring lamellar grafting in a number of cases. Because incomplete excision often ended in a recurrence, 2,6,18 total resection was considered the only viable treatment option to date, 1 and routine combination with a graft has been suggested.…”

“…To our knowledge, 6 such cases have been documented. [2][3][4][5][6][7] Our case is unique as a result of the vision-threatening growth toward the center of the cornea and the successful implementation of a new, minimally invasive treatment option.…”

The epidemiology, clinical characteristics, histopathology and management of juvenile- and adult-onset corneoscleral limbus xanthogranuloma

“…2). Some authors have relied on establishing a diagnosis of adult AOX based solely on the haematoxylin-eosin stain [8,16,20,21] (Table 2). The recognition of inflammatory cell types within the area of the xanthogranulomatous inflammation is adequate to provide a diagnosis of AOX.…”

“…Ocular JXG mainly involves the iris [5,6], and can present with spontaneous hyphema [7]; JXG is the most common cause of hyphema in children [8]. Infiltration of the anterior chamber angle and trabecular meshwork can lead to secondary unilateral glaucoma [7] and potentially to blindness [9].…”