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Bilateral invasive HPV-16 positive ocular surface squamous cell carcinoma in an immunocompetent patient

What:
Paper Presentation | Présentation d'article
When:
2:20 PM, Friday 1 Jun 2018 (6 minutes)
How:
Authors: Sara AlShaker, Martin Chang, Curtis Archibald, Godfery Heathcote, Dan DeAngelis
Author Disclosure Block: S. AlShaker: None. M. Chang: None. C. Archibald: None. G. Heathcote: None. D. DeAngelis: None.

Abstract Body:

Purpose: Ocular surface squamous neoplasia (OSSN) is rare; incidence is reported to be 0.02 to 3.5 per 100,000. Human papilloma virus (HPV) has long been implicated in OSSNs. While the finding of an HPV-driven disease in squamous carcinomas of the oropharynx indicates a potentially favourable prognosis, current ophthalmic literature is currently conflicted on the role of HPV in OSSN due to many factors including the rarity of the condition, geographic variations, and differences in sample testing.

Study Design: Case report

Methods: Retrospective review of clinical charts

Results: An otherwise-healthy 52-year old female presented to our centre with bilateral protuberant eyelid masses with crusting and discharge at the eyelid margins. She had originally seen an ophthalmologist 7 years prior to presentation with the finding of a small left followed by a right palpebral conjunctival papillomas. She was prescribed topical chemotherapy but had not been compliant with treatment which led to progression of the disease bilaterally. She had also declined early surgery and was lost to follow up for years. She once again sought medical attention as the masses had accelerated in growth resulting in vision loss in both eyes. On examination, the eyelid masses appeared to contiguously involve the palpebral and bulbar conjunctiva resulting in distortion of the ocular surface. A CT scan of the orbits confirmed anterior segment invasion as well as anterior orbital invasion. An examination under anesthesia and biopsy of the lesions bilaterally revealed an invasive squamous cell carcinoma that was P16 positive. Formalin-fixed paraffin-embedded tissue from both sites was later typed for HPV via the Roche Cobas 4800 Assay for HPV PCR and showed bilateral HPV-16 positivity. There was no distant metastasis and no lymphadenopathy (Stage T4N0M0). She was also evaluated for immunodeficiency and was found to be HIV-negative and hosting a normal level of immunoglobulins deeming her immunocompetent. The patient was counselled on the need for bilateral exenterations, which she initially refused but eventually had underwent. She declined post-surgical radiation.

Conclusions: This is a unique case of bilateral locally invasive OSSN in an immunocompetent host that resulted in bilateral vision loss necessitating the need for bilateral exenteration. The lack of compliance with therapy had led to observation of the natural history of OSSN, which demonstrated slow yet locally destructive progression. The finding of HPV-16 positivity in both sites poses the question whether this is the driving etiology behind the disease.
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