Abstract

A 14 YEAR FEMALE DIAGNOSED WITH XERODERMA PIGMENTOSA PRESENTED WITH FLESHY MASS IN RIGHT EYE SINCE 6 DAYS ASSOCIATED WITH PAIN AND DISCHARGE .SHE ALSO HAS DIMINUTION OF VISION IN BE SINCE AGE OF 6 YEARS.SHE WAS MANAGED CONSERVATIVELY. NO H/O OCULAR TRAUMA/OCULAR SURGERY OR SIMILAR COMPLAINTS IN THE FAMILY. CECT ORBIT & PNS SHOWED RE OCULAR SURFACE DIFFUSE ABNORMAL ENHANCING SOFT TISSUE LESION INFERO MEDIALLY, INFILTRATING THE EYELID, EXTENDING AND PROTRUDING TOWARDS SURFACE OF INFRAORBITAL REGION. RE PARTIAL EXENTRATION DONE. HISTOPATHOLOGY OF TISSUE SHOWED FEATURES SUGGESTIVE OF MALIGNANT MELANOMA.
CONCLUSION – MALIGNANT MELANOMA IN XERODERMA PIGMENTOSA IS A RARE PRESENTATION. MANAGEMENT OF MALIGNANT MELANOMA IN XERODEMA PIGMENTOSA POSES A GREAT CHALLENGE TO OCULOPLASTY SURGEONS AND IT REQUIRES A MULITI MODAL INTERVENTION BY PATHOLOGIST, MAXILLOFACIAL SURGEONS AND PLASTIC SURGEONS. EXENTERATION IN SUCH CASES HELPS IN IMPROVING THE SURVIVAL OF THE PATIENT