Conjunctival Mucoepidermoid Carcinoma in a Patient with Ocular Cicatricial Pemphigoid and a Review of the Literature

Invasive mucoepidermoid carcinoma of the conjunctiva of the left lower eyelid was diagnosed in an orbital exenteration specimen of a 57-year-old woman, after a biopsy of the same lesion was originally diagnosed as invasive squamous cell carcinoma. The woman was undergoing mitomycin C injections for ocular cicatricial pemphigoid, diagnosed in the same eye 2 years prior to identification of the neoplasm. The tumor invaded the cornea, sclera, lacrimal gland, regional small nerves, and lymphatics, but did not show intraocular involvement. The original biopsy specimen was reassessed with stains for mucin and found to be mucoepidermoid carcinoma of the conjunctiva. We reviewed 21 cases of mucoepidermoid carcinoma of the conjunctiva described to date in the English literature. We believe this number underestimates the true incidence of this condition, as it is frequently misdiagnosed both clinically and histopathologically. Evaluating suspected aggressive squamous cell carcinoma with special stains for mucin generally helps to identify mucoepidermoid carcinoma of the conjunctiva. More extensive surgical excision than that used for squamous cell carcinoma should be implemented in the management of mucoepidermoid carcinoma of the conjunctiva to prevent recurrence.