Abstract ID: 26-404
Survival into the sixth decade: A case of bilateral periocular basal cell carcinoma and ocular surface squamous neoplasia in Xeroderma Pigmentosum
Author: Zeynep Bilge Kozanoğlu Yılmaz Base Hospital / Institution: Istanbul University, Istanbul Faculty of Medicine
Presentation Type: ePoster Presentation
Purpose
Xseroderma pigmentosum is an autosomal recessive disorder characterized by a defective DNA repair mechanism in response to UV radiation-induced damage. In this report, we discuss a case of well-managed 52-year-old xeroderma pigmentosum with a previously diagnosed bilateral basal cell carcinoma (BCC) and a recent diagnosis of ocular surface squamous neoplasia.
Methods
A 52-year-old male patient with a clinically established diagnosis of Xeroderma Pigmentosum, followed in our ocular oncology unit, underwent local excisions for bilateral inferior eyelid BCC 18 years ago and for right-sided BCC 11 years ago in our clinic, and also has a history of multiple excisions in other clinics. After 11 years of follow-up without any ocular neoplasias, the patient presented with hyperemia in the left eye for two days. On ocular examination, the best corrected visual acuity was 0.9 in the right eye and 0.7 in the left eye. The left eye showed a localized inferior conjunctival lesion involving up to four clock hours, with a dilated feeder vessel. Other findings included ectropion and madarosis of the eyelids, and nuclear sclerosis of the lens in both eyes.
Results
An excisional biopsy was performed with a pre-diagnosis of carcinoma in situ in the left eye. Histopathological analysis of the conjunctival lesion confirmed the presence of carcinoma in situ.
Conclusion
Patients with xeroderma pigmentosum face a higher risk of developing oculocutaneous cancers like BCC and SCC; thus, any suspicious lesions must be carefully examined and managed. Early detection is crucial for a longer lifespan. The case presented here was managed effectively, and patient’s survival into the sixth decade is quite remarkable. To our knowledge, it is one of the eldest reported cases of xeroderma pigmentosum with ocular malignancy.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Gonca | İnci | Istanbul University, Istanbul Faculty of Medicine |
| Mehmet Serhat | Mangan | Istanbul University, Istanbul Faculty of Medicine |