Ocular Surface Squamous Neoplasia Following Keratoplasty in Xeroderma Pigmentosa: A Series of Seven Cases

Abstract

Purpose: To describe the clinical features and management of post-keratoplasty ocular surface squamous neoplasia (pk-OSSN) in patients with xeroderma pigmentosum (XP).Methods: Retrospective case series of seven patients with XP.Results: The mean age at diagnosis of pk-OSSN was 22 years (median, 21 years; range, 12 to 37 years). There were four males and three females. Full-thickness keratoplasty (n = 5) and Descemet stripping endothelial keratoplasty (n = 2) were performed for optical (n = 6) and therapeutic indications (n = 1). None of the patients had evidence of OSSN before or at the time of keratoplasty. The mean interval between keratoplasty and pk-OSSN was 44 months (median, 14 months; range, 3 to 109 months). The tumor epicenter was located at the limbus (n = 4), bulbar conjunctiva (n = 1) or cornea (n = 2). The mean dimension of the lesions was 12 mm (median, 11 mm; range, 6 to 18 mm). Treatment details included conjunctival/corneal tumor excision (n = 6), plaque brachytherapy (n = 3; 1 primary, 2 adjuvant) and extended enucleation (n = 1). One patient had tumor recurrence 18 months after OSSN treatment, which was treated with wide excisional biopsy. All patients are alive and free of disease over a mean follow-up period of 37 months (median, 37 months; range, 4 to 65 months).Conclusion: XP patients undergoing keratoplasty can develop OSSN several months to years after keratoplasty and require close follow-up for the same. Wide excisional biopsy with/without plaque radiotherapy is effective in the management of pk-OSSN.

Keywords: Eye; OSSN; corneal graft; keratoplasty; ocular surface; ocular surface squamous neoplasia; tumor; xeroderma pigmentosum.