Ocular Surface Squamous Neoplasia Associated with Atopic Keratoconjunctivitis

Abstract

Purpose: To describe 2 cases of invasive squamous cell carcinoma that originated in the setting of severe atopic keratoconjunctivitis (AKC).

Methods: Case one involved a 73-year-old male with atopic eczema and severe AKC who developed a limbal lesion suspicious for ocular surface squamous neoplasia (OSSN). Slit-lamp examination was significant for a new sessile lesion in the temporal limbal region of the left eye. The lesion was treated with excisional biopsy and cryotherapy. Topical therapy with mitomycin C, topical interferon alpha 2b, and topical 5-fluorouracil provided only partial control. Exenteration was eventually needed. Case two involved a 53-year-old male with history of severe AKC and eczema. Computed tomography imaging showed an infiltrative mass of the right orbit. Incisional biopsies confirmed conjunctival squamous cell carcinoma of both sides (invasive in the right eye, in situ in the left eye). Exenteration was needed for control of invasive carcinoma in the right eye.

Results: Squamous cell carcinoma was treated without success in spite of surgical excision and aggressive treatment with multiple topical agents and multiple applications of cryotherapy. Orbital exenteration was needed in both cases.

Conclusion: Chronic inflammation associated with AKC may be a risk factor for the development of bilateral, diffuse, invasive, and recurrent OSSN that may require exenteration.

Keywords: Atopia; Inflammation; Masquerade; Neoplasia; Ocular surface.

Fig. 1

Initial presentation of OSSN. A sessile papillomatous-like lesion was noted in the left eye in an eye with a previous penetrating keratoplasty (a). The lesion was located in the temporal limbal area (b). Excisional biopsy showed dysplastic cells suggestive of intraepithelial neoplasia that did not penetrate basement membrane and was graded as severe dysplasia (c). Dysplastic cells were noted at higher magnification. No keratinization was noted (d).

Fig. 2

Signs of tumor recurrence 3 months after completion of 4 cycles of topical mitomycin C chemotherapy. Three different areas suspicious for tumor recurrence were noted (a-c). Higher-magnification picture shows a papillomatous area that is highly suspicious for recurrence (d). Diffuse areas of recurrence invading into the corneal surface after aggressive treatment with multiple therapeutic modalities (e). Higher magnification shows recurrent frond-like lesions in the inferior corneal surface (f).

Fig. 3

Initial presentation of patient with right eyelid lesion illustrating large papillomatous lesion with erythema, ulceration, and loss of lashes (a). Right eyelid excisional biopsy shows sheets of invasive epithelium and connective tissue typical of invasive squamous cell carcinoma (b).

Fig. 4

Left eye conjunctival lesion located near the perilimbal region at 7 o'clock with mild elevation, vascularity and nodularity (a). Higher-magnification photo confirms raised, friable lesion (b). Histopathology illustrates cellular atypia, and diffuse dysplasia of the surface epithelium without invasion into the basement membrane (c, d). The lesion in the left eye responded well and resolved with the use of topical interferon dosed at 1 million units/ml four times a day for 6 months but recurred 3 months after discontinuation of topical interferon (e, f).