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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 4  |  Issue : 2  |  Page : 246-248

Conjunctival-corneal intraepithelial neoplasia: Presenting as a pterygium


1 Department of Pathology, Dr. D.Y. Patil Medical College and Research Center, Pimpri, Pune, India
2 Department of Ophthalmology, Dr. D.Y. Patil Medical College and Research Center, Pimpri, Pune, India
3 Department of Pathology, Mahatma Gandhi Medical College, Mumbai, Maharashtra, India

Date of Web Publication9-Mar-2015

Correspondence Address:
Mohammad Banyameen Iqbal
Department of Pathology, Dr. D.Y. Patil Medical College and Hospital, Pimpri, Pune
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0513.148918

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  Abstract 

Ocular surface squamous neoplasia (OSSN) comprises a wide spectrum of dysplastic alterations of the squamous epithelium of the surface of the eye, for example, the cornea and the conjunctiva ranging from "precancerous" lesions to bona fide invasive carcinoma. The epithelial infiltration can range from mild to severe dysplasia (i.e. mild, moderate, or severe corneal intraepithelial neoplasia) to full thickness epithelial dysplasia. We are presenting a case of a 55-year-old male patient who presented with a sudden increase in the size of her pterygium for the last 3 months, which were finally diagnosed to be carcinoma in situ.

Keywords: Carcinoma in situ, dysplasia, ocular surface squamous lesions


How to cite this article:
Iqbal MB, Mushtaq I, Jain A, Arun S. Conjunctival-corneal intraepithelial neoplasia: Presenting as a pterygium. Clin Cancer Investig J 2015;4:246-8

How to cite this URL:
Iqbal MB, Mushtaq I, Jain A, Arun S. Conjunctival-corneal intraepithelial neoplasia: Presenting as a pterygium. Clin Cancer Investig J [serial online] 2015 [cited 2020 Jun 1];4:246-8. Available from: http://www.ccij-online.org/text.asp?2015/4/2/246/148918


  Introduction Top


Ocular surface squamous neoplasia (OSSN) comprises a wide spectrum of dysplastic alterations of the squamous epithelium of the surface of the eye, for example, the cornea and the conjunctiva ranging from "precancerous" lesions to bona fide invasive carcinoma. In the former case they are classified as carcinoma in situ (CIS) lesions or conjunctival-corneal intraepithelial neoplasia (CCIN) and in the latter case invasive squamous cell carcinoma (SCC).

Ocular surface squamous neoplasia is a disease of the elderly [l] having predilection for the interpalpebral area mostly the corneoscleral limbus. [1] OSSN can involve the conjunctiva or the cornea individually but more commonly start in the conjunctiva and extend across the limbus to involve the adjacent cornea. It has a predilection for corneo-scleral limbus reinforcing the theory that transition zone is at increased susceptibility for dysplastic changes. [2] Lack of awareness, misinterpretation of OSSN as benign conditions such as keratoconjunctivitis, pterygium, papilloma, limbal dermoid, or foreign body granuloma and slow growth of lesion in relatively asymptomatic patient, may mislead the clinician into false sense of security with resultant recurrence and metastasis.


  Case report Top


A 55-year-old male presented in the Ophthalmology Department, with the complaints of a sudden increase in the size of his pterygium in the right eye from the last 3 months. The pterygium was present in his eye from the last >10 years. It was associated with redness, watering, and foreign body sensation. Best corrected visual acuity in right and left eye was 6/36 and 6/12 respectively. The anterior chamber was clear. Intraocular pressure was 16 mm of Hg in both eyes. On slit lamp biomicroscopy, a whitish pink, raised, fixed nodular mass of 5 mm diameter was noted at the nasal as well as temporal periphery of the cornea of right eye. The lesion involved the superficial layer of the cornea without any visible extension to adjacent ocular tissue. Conjunctival congestion was noted adjacent to the lesion. Left eye was normal. There was no lymphadenopathy, and systemic examination was within the normal limits. HIV and HBsAg status were negative. The patient was operated for pterygium, and the specimen was sent for histopathological examination.

Histopathological examination

Sections studied revealed conjunctival epithelial thickening with full thickness dysplasia. Scattered cells with large, pleomorphic nuclei and scattered mitotic figures are present. However, the cells do not cross the basement membrane at any point. The submucosa showed focal areas of perivascular chronic inflammatory cell infiltrate and dilated congested blood vessels. Features are suggestive of conjunctival CIS (CCIN) [Figure 1] and [Figure 2].
Figure 1: Low power view of the conjunctival squamous epithelium with full thickness dysplasia of the epidermal layers and normal dermis (H and E, ×20)

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Immunohistochemical examination was performed in this case to confirm the diagnosis that came out to be positive for panKeratin and human papilloma virus (HPV) [Figure 3].
Figure 2: High power view of the conjunctival squamous epithelium showing dysplastic arrangement of cells with a few mitotic figure (note that the dysplastic cells do not cross the basement membrane at any point) (H and E, ×40)

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Figure 3: Positivity for human papilloma virus in the nuclei of squamous cells (×40)

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  Discussion Top


Ocular surface tumors are relatively rare with an incidence of 0.13-1.9/100,000. [3],[4] The highest incidence of OSSN occurs in men between the ages of 50 and 75 years. [3],[4] OSSN is an umbrella term that encompasses dysplastic lesions involving the squamous epithelium of the conjunctiva or cornea, which includes squamous papilloma, CCIN, CIS, and invasive SCC. The epithelial infiltration can range from mild to severe dysplasia (i.e. mild, moderate, or severe CIN) to full thickness epithelial dysplasia CIS to invasive SCC, when tumor cells invade through the epithelial basement membrane. [3],[4],[5] Fortunately, of these conditions, invasive SCC is the least common. Fair skin, pale irises, high propensity to sunburn, and a history of skin cancer have all proven to be related risk factors. [5],[6] Other risk factors for OSSN include chronic infection by HPV, HIV, or trachoma, vitamin A deficiency, xeroderma pigmentosum, chronic irritants, and chronic epitheliopathies. [1],[4],[5],[6] Immunosuppression whether due to organ transplantation or secondary to AIDS, is a major risk factor, especially in conjunction with one of the above risk factors. It is estimated that the risk of conjunctival malignancies increases 13 fold in patients with HIV. [7] However, our case was immunocompetant and negative for HIV. OSSN lesions can frequently be distinguished from other conjunctival lesions, such as pterygia and conjunctival lymphoma. However, studies have shown it is very difficult to distinguish between the different types of OSSN; experienced physicians were only able to accurately diagnose OSSN stages 40% of the time. [4] Thus, a tissue specimen is needed for histologic diagnosis to distinguish CIN from invasive SCC. It is not unusual for invasive SCC to invade locally into the sclera, intraocularly, or into the orbit, with one study estimating incidence rates to be 37%, 13%, and 11% respectively. [8] In cases where extensive spread is suspected, it is important to assess the extent of the lesion with ultrasound biomicroscopy (to assess sclera or intraocular invasion), gonioscopy or gadolinium-enhanced magnetic resonance imaging scans (to assess orbital extension). Fortunately, even the most aggressive form of OSSN, invasive SCC, usually, is not associated with regional or distant metastases, and only a few cases have been reported. [9],[10] All forms of OSSN tumors are treated aggressively but with markedly different anticipated therapeutic endpoints; the goal of less invasive disease is complete eradication, while the goal of invasive disease is to minimize the spread of the disease. Depending on the lesion and histopathologic findings, treatment can range from topical chemotherapy or excision alone for smaller lesions versus a combination of surgical excision, cryotherapy, and chemotherapy for larger or invasive. Rarely, radiotherapy, and in extreme cases, enucleation and even exenteration, may be necessary. [6],[11],[12]

The patient was discharged after surgery and presently on close follow-up in the Department of Surgical Oncology.


  Conclusions Top


Ocular surface squamous neoplasia (CCIN) should be kept in mind while dealing with ocular biopsies, especially pterygium, not only in the elderly, but also in middle-aged as well as young individuals even in the absence of HIV and other immunosuppressive diseases.

 
  References Top

1.
Chen C, Louis D, Dodd T, Muecke J. Mitomycin C as an adjunct in the treatment of localised ocular surface squamous neoplasia. Br J Ophthalmol 2004;88:17-8.  Back to cited text no. 1
    
2.
Erie JC, Campbell RJ, Liesegang TJ. Conjunctival and corneal intraepithelial and invasive neoplasia. Ophthalmology 1986;93:176-83.  Back to cited text no. 2
[PUBMED]    
3.
Coroi MC, Rosca E, Mutiu G, Coroi T. Squamous carcinoma of the conjunctiva. Rom J Morphol Embryol 2011;52:513-5.  Back to cited text no. 3
    
4.
Lee GA, Girst LW. Occular surface sqamous neoplasia. Surv Ophthalmol 1995;39:429-50.  Back to cited text no. 4
    
5.
Newton R. A review of the aetiology of squamous cell carcinoma of the conjunctiva. Br J Cancer 1996;74:1511-3.  Back to cited text no. 5
    
6.
Birkholz ES, Goins KM, Sutphin JE, Kitzmann AS, Wagoner MD. Treatment of ocular surface squamous cell intraepithelial neoplasia with and without mitomycin C. Cornea 2011;30:37-41.  Back to cited text no. 6
    
7.
Margo CE, Mack W, Guffey JM. Squamous cell carcinoma of the conjunctiva and human immunodeficiency virus infection. Arch Ophthalmol 1996;114:349.  Back to cited text no. 7
    
8.
Tunc M, Char DH, Crawford B, Miller T. Intraepithelial and invasive squamous cell carcinoma of the conjunctiva: Analysis of 60 cases. Br J Ophthalmol 1999;83:98-103.  Back to cited text no. 8
    
9.
Zimmerman L. The cancerous, precancerous, and pseudocancerous lesion of the cornea and conjunctiva: Corneoplastic surgery. Proceedings of the Second Annual International Corenoplastic Conference. London: Pergamon Press; 1969.  Back to cited text no. 9
    
10.
Tabbara KF, Kersten R, Daouk N, Blodi FC. Metastatic squamous cell carcinoma of the conjunctiva. Ophthalmology 1988;95:318-21.  Back to cited text no. 10
    
11.
Papaioannou IT, Melachrinou MP, Drimtzias EG, Gartaganis SP. Corneal-conjunctival squamous cell carcinoma. Cornea 2008;27:957-8.  Back to cited text no. 11
    
12.
Zaki AA, Farid SF. Management of intraepithelial and invasive neoplasia of the cornea and conjunctiva: A long-term follow up. Cornea 2009;28:986-8.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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