Unusual Basal Cell Carcinoma in an Adult Woman With Gorlin-Goltz Syndrome

Abstract

A woman aged 66 years presented to the ophthalmology clinic for evaluation of an asymptomatic medial canthus mass of the left eye (Figure , A). Her medical history was significant for Gorlin-Goltz syndrome. Her best-corrected visual acuity was 6/9 OD and 6/7.5 OS. On examination, the medial canthus mass was noted to be noncontiguous with the periorbital skin. Biopsy of the lesion revealed conjunctival basal cell carcinoma (BCC) (Figure , B). The lesion was managed with 2 excisions and conjunctival patch grafting.BCCs originate from pilosebaceous structures, which are only found in the caruncle of the conjunctiva. 1 Some ophthalmologists believe that there must be pluripotent basal cells within the deeper stroma capable of producing BCCs. 2 This patient had Gorlin-Goltz syndrome, which is an inherited autosomal-dominant syndrome resulting in multiple nevoid BCCs owing to an abnormal PTCH1 gene. 3 Conjunctival BCCs are rare. Of 5002 cases of conjunctival tumors, Shields et al 4 found only 4 BCCs, all caruncular in origin. Only 2 published cases describe temporal conjunctival BCCs, 1 pigmented and the other invasive. 5 This seems vanishingly rare, prompting us to raise the question: do all conjunctival BCCs arise from the caruncle?