Infundibulocystic basal cell carcinoma of the eyelid in basal cell nevus syndrome.

Purpose: To describe the histopathologic findings in a series of eyelid basal cell carcinomas removed from patients with basal cell nevus syndrome.
Methods: Retrospective case series of 5 patients with basal cell nevus syndrome identified from our oculoplastics service. The systemic and ophthalmic features were reviewed, and a retrospective histopathologic analysis of all available previously excised eyelid lesions was performed. The pertinent published literature on basal cell nevus syndrome and eyelid basal cell carcinoma was reviewed.
Results: A total of 26 eyelid lesions were examined histopathologically. Twenty-three of these lesions were basal cell carcinomas. The infundibulocystic variant of basal cell carcinoma was identified most commonly (57%).
Conclusions: Eyelid basal cell carcinomas in patients with basal cell nevus syndrome were commonly of the infundibulocystic variety in our series. Infundibulocystic basal cell carcinomas, which can be clinically indistinguishable from the more common forms, are thought to be less aggressive than other types of basal cell carcinoma and are a reassuring histopathologic diagnosis. It is important for the ophthalmologist and pathologist to be aware of infundibulocystic basal cell carcinomas, as they are more common in patients with basal cell nevus syndrome and may be a clue to the diagnosis of this autosomal dominant cancer-predisposition syndrome or other associated syndromes. To our knowledge, this variant of basal cell carcinoma has not been previously discussed in the ophthalmic literature.