|Year : 2014 | Volume
| Issue : 3 | Page : 185-188
Ocular surface squamous neoplasia
Penumala Viswamithra1, Neethipudi Bhaskara2
1 Deparment of Ophthalmology, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India
2 Deparment of Anaesthesiology, Rajiv Gandhi Institute of Medical Sciences, Srikakulam, Andhra Pradesh, India
|Date of Web Publication||17-Sep-2014|
Department of Ophthalmology, Andhra Medical College, Visakhapatnam - 530 003, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Ocular surface squamous neoplasia (OSSN) represents a rare spectrum of disease ranging from mild dysplasia to carcinoma in situ to invasive squamous cell carcinoma of the ocular surface. OSSN is more common in elderly males, in countries closer to equator and countries where exposure to excessive sunlight is more common. Diagnosis of OSSN is mainly based on clinical suspicion. Definitive diagnosis needs histopathological evaluation. In this article, we report a case of OSSN in a 28 year old male presented with limbal nodule of right eye. Excisional biopsy was done. Histopathological examination revealed a well differentiated squamous cell carcinoma.
Keywords: Cornea, Ocular Surface Squamous Neoplasia, OSSN, squamous cell carcinoma of conjunctiva
|How to cite this article:|
Viswamithra P, Bhaskara N. Ocular surface squamous neoplasia. J NTR Univ Health Sci 2014;3:185-8
| Introduction|| |
Ocular surface squamous neoplasia (OSSN) represents a rare spectrum of disease ranging from mild dysplasia to carcinoma in situ to invasive squamous cell carcinoma of the ocular surface involving the conjunctiva and the cornea, occurring usually in the interpalpebral area, mostly at the limbus.  Prevalence of OSSN varies from 0.03-1.9 per million population depending on the geographic location.  In a National Institutes of Health study, the incidence of OSSN in the United States was 0.03 cases per 100 000 persons. In a study performed in Uganda, the incidence of OSSN was 0.13 cases per 100 000 persons. Though OSSN is seen, but rarely, in Indian population. OSSN is more common in countries that are closer to the equator and countries where excessive exposure to sunlight is more common. Risk factors for OSSN include ultraviolet light exposure, fair skin, human papilloma virus (HPV) infection, human immune deficiency virus (HIV) infection, and cigarette smoking.  OSSN has been reported to masquerade as chronic blepharo conjunctivitis, chronic conjunctivitis, corneal ulcer, pterygium, necrotizing scleritis and sclerokeratitis. Clinically, the diagnosis is suspected by the appearance of epithelial changes of the ocular surface. Slit-lamp examination shows gelatinous, leukoplakic, papilliform or nodular lesions. Histopathological evaluation is essential for the definitive diagnosis and also to differentiate the three lesions in the spectrum of OSSN i.e., epithelial dysplasia, carcinoma in situ and invasive squamous cell carcinoma. Recently, immuno histochemically detectable p53 protein, bcl-2 protein, MIB-1 are being used as markers of proliferative potency having a possible prognostic value. MIB-1 is a monoclonal antibody that recognizes Ki-67 antigen which is a marker of cell proliferation.Bcl-2 is a proto oncogene which is thought to have a role in oncogenesis by inhibiting programmed cell death and preserving cells from p53 induced apoptosis. p53 gene is a common cellular target in human carcinogenesis and thought to have an important role in cellular proliferation. The interplay between the effects of the mutant p53, absence of bcl-2 expression and enhanced expression of MIB-1 are believed to contribute to the progression of OSSN.
We report a case of OSSN in a 28-year-old male who has been misdiagnosed with chronic conjunctivitis and subsequently been treated for chronic conjunctivitis for the past 2 years.
| Case report|| |
A 28-year old male patient presented with the chief complaint of mass in his right eye for 2 years, which was progressively growing in size, and associated with redness, watering and mild pain. The patient had no history of trauma or surgery to that eye in the past. He was on topical antibiotics and steroids for the past 2 years for his right eye condition. General examination and systemic examination were normal. Best corrected visual acuity in his right eye was 6/9. On local examination of the right eye, a greyish white nodular lesion of size 6 mm × 8 mm was present at the limbus between 7°-8° clock position, extending 2 mm into the cornea [Figure 1] Engorged conjunctival vessels were seen at the base of the lesion. The surface of the lesion was irregular. Blood investigations were normal. Serology test for HIV was negative. Right eye ultrasonography was normal. Surgical excision is the predominant modality of management for OSSN.  The possible diagnosis of his condition, further steps of treatment basing on the pathology report and chances of recurrence of the lesion were discussed with the patient. After taking informed written consent from the patient, surgical excision biopsy following ''Shield's no touch technique'' was done to the limbal lesion of the right eye of the patient and the specimen was sent for histopathological evaluation. Postoperatively patient was given topical antibiotic with steroid drops in a tapering dose. Histopathology report showed sheets of malignant squamous cells with moderate nuclear pleomorphism and with foci of epithelial keratin pearl formation, confirming the diagnosis of a well differentiated squamous cell carcinoma [Figure 2]. The stroma in between the tumor cells was infiltrated with diffuse sheets of inflammatory cells. Sections from adjacent squamous epithelium showed moderate dysplastic changes [Figure 3]. Sections from all the surgical margins were free from tumour [Figure 4] Patient was reviewed regularly. Post-operative period was uneventful [Figure 5].
|Figure 2: Histopathology section showing malignant squamous cells and foci of epithelial keratin pearls; 40x magnifi cation, H&E stain|
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|Figure 3: Histopathology section showing moderate dysplasia of adjacent squamous epithelium. 10x magnifi cation, H&E stain|
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|Figure 4: Histopathology section showing surgical margins free of|
tumor; 10x magnifi cation, H&E stain
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| Discussion|| |
Lee and Hirst (1995) first proposed the term ''Ocular surface squamous neoplasia (OSSN)'' to encompass the spectrum of dysplasia, carcinoma in situ and squamous cell carcinoma of ocular surface i.e., conjunctiva, limbus and cornea. OSSN occurs in all races, more commonly in countries that are closer to the equator and in those where exposure to sunlight is more frequent. Reported risk factors for OSSN include ultraviolet light exposure, HPV infection, HIV infection, increased p53 expression, chronic inflammatory diseases of the ocular surface such as mucous membrane pemphigoid, chronic blepharoconjunctivitis, and atopic eczema.  Commonest clinical presentation of OSSN is an elevated, vascularised lesion in the interpalpebral area, mostly at the limbus. It can also appear as a papilliform, gelatinous or leucoplakic lesion on the ocular surface. Symptoms range from none to severe pain and visual loss. Clinically, it is difficult to distinguish between epithelial dysplasia, carcinoma in situ, and invasive squamous cell carcinoma. Definitive diagnosis is made by histopathological examination of the excision biopsy specimen.
Though OSSN is considered as a localized, slow growing lesion with low metastatic potential, intraocular, intra orbital and distant metastasis do occur rarely. OSSN has intraocular and orbital extension rate of 4%, regional and distant metastasis rate of 1.2%, and a mortality rate of 0.8%. Sites of metastasis include the preauricular, submandibular, and cervical lymph nodes, the parotid gland, lungs, and bones. Regional lymph node involvement precedes the development of distant metastases.  The main cause for metastasis is delay in diagnosis and treatment. 
Surgical excision with ''wide margin, no touch'' technique is currently the best established form of treatment. Nevertheless, recurrences of these lesions are common after surgical excision, depending on the involvement of the surgical margins. Recurrence rates following excision of OSSN alone range from 15 to 52%, with an average of 30%. Recurrence rate is 5% when the surgical margins are free and 53% when the surgical margins are involved.  Adjunctive therapy to reduce recurrence includes intra operative cryotherapy and brachytherapy, postoperative topical chemotherapy using mitomycin C,5-FU and interferon alfa-2b.
As the histopathological evaluation of our case showed tumour free surgical margins, patient was put on observation without any adjunctive therapy. At 3 months post-surgical review, the patient was free from any clinical recurrence.
| Conclusion|| |
Slow growth of lesions of OSSN and the ever present malignant potential makes regular follow-up of these patients for the remainder of their life mandatory.
| Acknowledgement|| |
We would like to express our sincere gratitude toward Dr. Bhagyalakshmi, M.D. Pathology, Professor and HOD, Department of Pathology, Andhra Medical College for her kind co-operation in preparation of this case report. We would like to sincerely thank Dr. V. Rajeswara Rao, M.S. Opthalmology, Professor, Department of Ophthalmology, Andhra Medical College for his support and encouragement.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]