MULTIPLE METACHRONOUS MALIGNANCIES IN XERODERMA PIGMENTOSA: TOO MANY CANCERS IN ONE BODY

*Dr. Chanmiki Sayoo

ABSTRACT

Introduction: Xeroderma Pigmentosa is a rare autosomal recessive genetic disorder characterised by defective DNA repair. Clinical features are skin photosensitivity, xerosios, hyperpigmented lentiginous macule. Patient with this condition are at higher risk of developing malignancies throughout their life as compared to general population. Case Report: we report a case of a 21 year old boy suffering from Xeroderma Pigmentosa who developed multiple malignancies over time and was treated with multiple surgeries and radiation therapy. Till the last follow up the patient is doing well and tolerated well to radiation therapy. Conclusion: At present there is no permanent cure for Xeroderma Pigmentosa. Avoiding sun exposure by chemical or physical sunscreen is important. Early detection of premalignant and malignat lesions is important to avoid morbid surgery and radiotherapy. Though literature report different reaction to radiation therapy but our patient tolerated well to radiation therapy twice radiated.

Keywords: Xeroderma pigmentosa; head and neck cancer; multiple malignancies.