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Year : 1994  |  Volume : 60  |  Issue : 1  |  Page : 57

Squamous cell carcinoma from limbus of eye in xeroderma pigmentosum

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How to cite this article:
Udagani, MM, Govekar V G. Squamous cell carcinoma from limbus of eye in xeroderma pigmentosum. Indian J Dermatol Venereol Leprol 1994;60:57

How to cite this URL:
Udagani, MM, Govekar V G. Squamous cell carcinoma from limbus of eye in xeroderma pigmentosum. Indian J Dermatol Venereol Leprol [serial online] 1994 [cited 2020 Oct 22];60:57. Available from:

Ocular neoplasms arising from the eye, excluding those of eyelids constitutes 11% Xeroderma pigmentosurn (XP) patients. [1] They are most frequently arising from the limbus and are predominently sqamous cell carcinomas (SCC). Recently there was a case report of malignant melanoma of skin and SCC of the eye arising from limbus in an adult XP patient . [2]

A 6-year-old male, youngest child of a consanguineous parents had multiple freckles and hypopigmented atrophic macules on sun exposed parts of the body since 4 years of age. He had photophobia, blepharospasm and increased lacrimation. Developmental mile stones were normal and no neurological manifestations were noticed.

Both the sisters of the patient developed XP, while his only brother was healthy.

Patient developed a small nodular growth 1 month back, situated at 5 O'clock position at the limbus of left eye. During I month, it attained the size of 1.5cm X 1cm grayish brown raised growth encroached upon cornea completely and growth was protruding out about 0.5cm. Child had pain, irritation and could not close the eye. There were no metastases.

Routine investigations were normal including LET. X-ray chest found normal, skin biopsy confirmed the diagnosis of XP. Enucleation of eyeball was inevitable. Histopathology of the growth revealed as well differentiated SCC.

Neoplasm of the eye in XP confined almost exclusively to the conjuctiva, cornea and eyelids, those portions of the eye exposed to ultraviolet radiation. These tissue sheild the iris, lens and retina from ultraviolet radiation.

Unique review of 830 published cases of XP in a span of 108 years by Kraemer et all revealed that neoplasms occured most frequently at the limbus followed by the cornea and conjuctiva. The most frequent histologic type reported was SCC.

  References Top

1.Kraemer K H, Lee M M, Scotto J. Xeroderma pigmentosum, cutaneous ocular and neurologic abnormalities in 830 published cases. Arch Dermatol 1987; 123: 241-50.  Back to cited text no. 1    
2.Nagabhushan N R, Satish D A, Sumathy T K, et al. Xeroderma pigmentosum with malignant melanoma and squamous cell carcinoma. ind J Dermatol Venereol Leprol 1989; 55: 248-50.  Back to cited text no. 2    


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