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Advanced squamous cell carcinoma of cornea in a child with Xeroderma Pigmentosa

Xeroderma Pigmentosum (XP) presents in early childhood with photophobia, photosensitivity, cutaneous pigmentary changes, and a predisposition for malignancy in sun-exposed mucocutaneous areas and ocular structures. Ocular diseases are evident in at least 40% of XP patients and often cause visual impairment. Eight-year-old male child, a diagnosed case of XP at the age of 6 years, came to ophthalmology outpatient department with watering, redness, photophobia and gradually increasing mass on nasal side in the left eye for the last 6 months. In the left eye, almost the entire cornea was involved with a grayish-white, irregular, and limbal mass of 10 mm × 12 mm in size. Excision biopsy of the growth in the left eye was performed under general anesthesia. Cryotherapy application was done over the excised area. Histopathology report revealed well-differentiated squamous cell carcinoma. Ophthalmologist′s role in early detection and excision of suspected lesions, counseling as well as prompt referral to the dermatologist and oncologist are vital in the management of such cases.