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Benjamin Peter Erickson, Anat Galor, Thomas Johnson, Wendy Wong Lee, Carol L Karp; Orbital Invasion From Ocular Surface Squamous Neoplasia . Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):3424.
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© ARVO (1962-2015); The Authors (2016-present)
Orbital invasion from ocular surface squamous neoplasia (OSSN) is a rare and potentially devastating complication. The majority of studies arise from Sub-Saharan Africa and the Middle East, where HIV and poor access to care are significant factors. The North American literature primarily consists of isolated reports. We present our experience with 7 cases of OSSN invading the orbit.
Retrospective review of 500 consecutive patients with biopsy-confirmed OSSN and radiologic and/or histologic evidence of orbital invasion.
Seven patients with orbital extension of OSSN were identified. Average age was 59.7±15.8 years old; 5 patients were male and 2 female. 5/7 were black and the remainder Hispanic (1/7) or non-Hispanic Caucasian (1/7). 4/7 were current or former smokers. 3/7 patients (age 48±10 years old) were HIV positive. None had a known history of hematogenous malignancy, head & neck radiation, immunosuppressive medication use, skin cancer, or HPV. Five of the lesions were primary, while the remaining 2 represented recurrence of previously treated OSSN. Lesions occupied 6.8±5.0 clock hours, with 5 of 7 cases involving the perilimbal conjunctiva. The one case with multiple recurrences had only a single clock hour of superficial involvement. Orbital invasion was clinically detected in the form of significant motility restriction in 4/7 patients, diplopia in 2/7, profound vision loss (light projection or worse) in 2/7, and epiphora in 2/7. Only the patient with multiple recurrences lacked clear external stigmata of invasion, which was identified by computed tomography (CT). Scans revealed involvement of the extraconal fat in 6/7, intraconal fat in 2/7, lacrimal drainage system in 2/7, and optic nerve in 1/7. Four of the 7 patients underwent total, and 2/7 lid sparing, exenteration; the remaining patient (age 88) refused surgery and died of unrelated causes. 2/7 underwent negative sentinel node biopsy. One patient each was treated with adjuvant chemotherapy and radiation. A single patient had disease specific mortality.
OSSN is statistically a disease of older Caucasian men with history of sun exposure, but the majority of patients in our study were black with delayed primary diagnosis and/or underlying immunosuppression. Orbital invasion appears rare in cases of appropriately treated but recurrent OSSN, but associated external findings can be minimal and close surveillance is essential.
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