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BRIEF COMMUNICATION
Year : 2008  |  Volume : 56  |  Issue : 5  |  Page : 421-423

Orbital amelanotic melanoma in xeroderma pigmentosum: A rare association


1 Institute of Ophthalmology, Jawaharlal Nehru Medical College, Aligarh, UP, India
2 Department of Pathology, Jawaharlal Nehru Medical College, Aligarh, UP, India
3 Bishen Skin Center, M-69, Marris Road, Aligarh, UP, India

Correspondence Address:
Syed AR Rizvi
52, Alig Apartments, Shamshad Market, Aligarh - 202 002, UP
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0301-4738.42423

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Xeroderma pigmentosum (XP) is an autosomal recessive genetic disorder of DNA repair in which the body's normal ability to repair damage caused by ultraviolet light is deficient. This leads to a 1000-fold increased risk of cutaneous and ocular neoplasms. Ocular neoplasms occurring in XP in order of frequency are squamous cell carcinoma, basal cell carcinoma and melanoma. Malignant melanomas occur at an early age in patients with XP. We report a case of XP with massive orbital melanoma in an eight-year-old boy which is unique due to its amelanotic presentation confirmed histopathologically.


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