Ipsilateral presentation of ocular surface squamous neoplasia and conjunctival melanoma in xeroderma pigmentosum: A rare occurrence

Abstract

A case of a 16-year-old male with xeroderma pigmentosum (XP) who presented with multiple pigmented and nonpigmented conjunctival lesions in both eyes is reported. He had a keratinized lesion at the limbus and a pigmented lesion in bulbar conjunctiva in the left eye and multiple pigmented bulbar conjunctival lesions and a keratinized limbal nodule in the right eye. Excision biopsy confirmed the diagnosis of ocular surface squamous neoplasia (OSSN) and conjunctival intraepithelial melanocytic neoplasia-2 (CIMN-2) in the right eye and OSSN and conjunctival melanoma in situ (CIMN-5) in the left eye. Two malignant conjunctival lesions occurring simultaneously in the same eye of a patient with XP have not been reported earlier.

Keywords: Conjunctival melanoma; ocular surface squamous neoplasia; xeroderma pigmentosum.

Figure 1

Clinical photograph showing OSSN and conjunctival melanocytic lesion in the (a) right eye and (b) left eye. High-magnification photograph of the right eye showing (c) placoid area (yellow arrowhead) with surface keratin at temporal limbus and (d) areas of flat brown conjunctival melanocytic lesion of the nasal bulbar conjunctiva. High-magnification photographs of the left eye showing (e) OSSN at the temporal limbus and (e) dark brown thickened pigmented conjunctival lesion in nasal bulbar conjunctiva

Figure 2

Histopathology of the excised conjunctival lesions. Hematoxylin and eosin (H and E, ×40)-stained tissue from the (a) right eye nonpigmented temporal bulbar conjunctiva showing stratified squamous epithelium with mild to moderate dysplasia and (b) nonpigmented temporal lesion in the left eye showing moderate squamous dysplasia. Histopathology (H and E, ×40) (c) from the right eye flat pigmented nasal bulbar conjunctival lesion showing CMIN with grade 2 atypia and (d) from pigmented left eye nasal bulbar conjunctival lesion showing CMIN with grade 5 atypia (melanoma in situ)

Figure 3

Postoperative photographs. (a) Slit-lamp photograph of the left eye at 2 weeks postoperative period showing a gelatinous nodule at the edge of conjunctival excision (yellow arrowhead) suggestive of OSSN recurrence. (b) AS-OCT through nodule showing localized thickened hyper-reflective epithelium with abrupt transition between the normal and thickened epithelium (white arrowhead). Postoperative photograph of the (c) right and (d) left eye at 1 year follow-up showing conjunctival scarring with no evidence of recurrence of OSSN or melanoma