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HPV16 conjunctival squamous cell carcinoma

Published:September 18, 2022DOI:https://doi.org/10.1016/j.amjms.2022.08.021

      Case Presentation

      A 38-years-old Nigerian man presented with an enlarged and vascularized conjunctival lesion covering the corneal surface of the left eye, associated with an upper bulbar conjunctival nodule (Fig. 1a). Magnetic resonance imaging (MRI) showed a thickened and contrast-enhancing corneal and conjunctival-scleral lesion, mostly evident in the upper lateral surface, close to the superior and lateral rectus muscles and lacrimal gland, which appeared enlarged (Fig. 1b and 1c). No bone erosion of orbital walls was demonstrated on computed tomography (CT) (Fig. 1d). After surgical evaluation, the patient underwent orbital exenteration and reconstruction using temporalis muscle. At histopathologic examination, the diagnosis was moderately-differentiated squamous cell carcinoma (Fig. 1e and 1f), extended to the conjunctive with infiltration of periocular striated muscles. Immunohistochemical examination revealed the positivity for p16 (Fig. 1g) demonstrating a correlation with HPV, later confirmed by HPV-DNA PCR analysis. Today, 5 years after surgery the patient is in well-health with no signs of recurrence.
      Conjunctival squamous cell carcinoma (CSCC) is the most common non pigmented malignancy of the ocular surface. It is a rare tumor usually affecting people aged between 50 and 75 years. In Africa the incidence of CSCC is about 9 to 10 times higher than Caucasian population.
      • Röck T.
      • Bartz-Schmidt K.U.
      • Bramkamp M.
      • et al.
      Clinical management of squamous cell carcinoma of the conjunctiva.
      Usually CSCC has a male predilection; however, in Africa males and females are equally affected probably due to the higher risk of HIV and HPV infections. HPV increases the odds of conjunctival tumors by 8.4 compared to other ocular surface pathologies or healthy conjunctival mucosa and HPV16 is the most prevalent genotype, followed by HPV18 and HPV33.
      • Ramberg I.
      • Møller-Hansen M.
      • Toft P.B.
      • et al.
      Human papillomavirus infection plays a role in conjunctival squamous cell carcinoma: a systematic review and meta-analysis of observational studies.
      CSCC is considered a low-grade malignancy and its treatment depends on size and extension. The majority of HPV‐related conjunctival tumor may be prevented by currently available HPV vaccines.
      • Ramberg I.
      • Møller-Hansen M.
      • Toft P.B.
      • et al.
      Human papillomavirus infection plays a role in conjunctival squamous cell carcinoma: a systematic review and meta-analysis of observational studies.
      Early diagnosis and effective therapy will minimize the morbidity and the mortality related to the disease. Imaging techniques are pivotal to evaluate the invasion of orbital structures. Rarely CSCC may metastasize with death in a small percentage of patients. The treatment of choice remains a wide surgical excision: ocular enucleation in cases of intraocular invasion, orbital exenteration in cases of orbital extension.
      • Gökmen Soysal H.
      • Ardiç F.
      Malignant conjunctival tumors invading the orbit.

      Funding statement

      This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

      Declaration of Competing Interest

      The authors have no personal, financial, or institutional interest with regards to the authorship and/or publication of this manuscript.

      References

        • Röck T.
        • Bartz-Schmidt K.U.
        • Bramkamp M.
        • et al.
        Clinical management of squamous cell carcinoma of the conjunctiva.
        Am J Case Rep. 2020; 21e919751
        • Ramberg I.
        • Møller-Hansen M.
        • Toft P.B.
        • et al.
        Human papillomavirus infection plays a role in conjunctival squamous cell carcinoma: a systematic review and meta-analysis of observational studies.
        Acta Ophthalmol. 2021; 99: 478-488https://doi.org/10.1111/aos.14666
        • Gökmen Soysal H.
        • Ardiç F.
        Malignant conjunctival tumors invading the orbit.
        Ophthalmologica. 2008; 222: 338-343