OcularTumors Secondary to Xeroderma Pigmentosum:Squamous Intraepithelial Neoplasia/Squamous Cell Carcinoma and Atypical Fibroxanthoma
Objective:To investigate the clinicopathological features and immunohistochemical expression of ocular surface tumors secondary to xeroderma pigmentosum(XP).Methods:A retrospective analysis was conducted on two XP patients who developed secondary ocular surface tumors and were treated at the Eye and ENT Hospital of Fudan University from 2000 to the present.Additionally,relevant literature was also reviewed.Results:Both patients(one male,one female)were diagnosed with XP in early childhood and presented to the Eye and ENT Hospital of Fudan University with ocular surface neoplasms.The male patient was 10 years old at the initial visit and over the subsequent 17 years,underwent 10 hospital admissions,during which he received 9 surgical resections.Pathological diagnoses included carcinoma in situ with early stromal invasion of the left cornea and conjunctiva(four times),squamous intraepithelial neoplasia of the left cornea and conjunctiva(twice),squamous cell carcinoma of the right eyelid(once),keratoacanthoma of the left eyelid(once),and atypical fibroxanthoma(AFX)of the left cornea(once).The female patient was treated with surgical excision of the left conjunctival lesion at age 19,which was diagnosed as carcinoma in situ with early stromal invasion.Conclusions:XP patients are prone to develop tumors in sun-exposed areas,particularly in the eyelids,cornea,and conjunctiva.In addition to common epithelial tumors,rare AFX can also occur on the ocular surface.Despite the high recurrence rate of ocular tumors in XP patients,early surgical intervention can effectively manage disease progression and improve prognosis.
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