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
• tumor
• ocular surface
• ocular surface squamous neoplasia
• OSSN
• keratoplasty
• xeroderma pigmentosum
• corneal graft

Authors’ contributions

VVS was involved with data collection, data analysis and first draft preparation, and final approval of the manuscript; AGK was involved with patient data and revision of the manuscript; SK was involved with planning of the study, patient data, and revision of the manuscript.

Declaration of interest

No conflicting relationship exists for any author.

Ethics approval

Ethics Committee of LV Prasad Eye Institute, Hyderabad, India approved the study

Additional information

Funding

Support provided by The Operation Eyesight Universal Institute for Eye Cancer (SK) and Hyderabad Eye Research Foundation (SK), Hyderabad, India. The funders had no role in the preparation, review or approval of the manuscript.