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Case Reports
. 2015 Jun;1(4):254-8.
doi: 10.1159/000377682. Epub 2015 Apr 15.

Atypical Fibroxanthoma of the Conjunctiva in Xeroderma Pigmentosum

Nabeel Shalabi  1 , Anat Galor  2 , Sander R Dubovy  3 , Jordan Thompson  4 , J Antonio Bermudez-Magner  4 , Carol L Karp  4
Affiliations
Case Reports

Atypical Fibroxanthoma of the Conjunctiva in Xeroderma Pigmentosum

Nabeel Shalabi et al. Ocul Oncol Pathol. 2015 Jun.

Abstract

Background: Atypical fibroxanthoma (AFX) is a pleomorphic tumor that most commonly presents in the elderly and arises on sun-exposed skin.

Methods: We report a case of a 24-year-old white male with xeroderma pigmentosum (XP), who developed an AFX of the conjunctiva, and review the literature.

Results: The patient presented with a rapidly growing conjunctival mass which was surgically excised and found to be AFX. The patient has done well with no recurrence. A review of the literature was performed, and we found that only one other case has been published so far.

Conclusion: Patients with XP may present with unusual tumors, including nonepithelial soft-tissue tumors. AFX is a rapidly growing tumor which may rarely present on the ocular surface.

Keywords: Atypical fibroxanthoma; Malignant fibrous histiocytoma; Skin tumor; Xeroderma pigmentosum.

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Figures

Fig. 1
Fig. 1
External photograph of the patient demonstrating multiple skin lesions and exenterated left eye.
Fig. 2
Fig. 2
a Right eye with AFX of the conjunctiva revealing a soft, pink lesion on the lateral limbus. b Nine days after the first presentation, the lesion had rapidly enlarged.
Fig. 3
Fig. 3
a Low-magnification image disclosing conjunctiva that contains a well-circumscribed tumor composed of atypical cells within the substantia propria. b High-magnification image of the conjunctival tumor disclosing foci of cells with variably multinucleated prominent nucleoli and many atypical mitotic figures (arrow). c-f The tumor cells stain positive for SMA (c) and CD10 (d) and negative for melan A (e) and S-100 (f) (all from Dako, Carpinteria, Calif., USA). a, b Hematoxylin and eosin: ×40 (a) and ×400 (b). c-f ×200.
See this image and copyright information in PMC

References

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