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MRIMS Journal of Health Sciences 2016;4(2) http://www.mrimsjournal.com/ pISSN: 2321-7006, eISSN: 2321-7294 Original Article Clinical behaviour of ocular surface squamous neoplasia (OSSN) in HIV positive patients P. Padmavathi 1, D. Padma Prabha 2, Mohd. Ather 3, T. Kavitha 4, M. Padma 5 1, 2 & 5 Assistant professor of Ophthalmology, Sarojini Devi Eye Hospital, Osmania Medical College, Hyderabad 3 Professor I/C of Ophthalmology, Gandhi Medical College, Hyderabad 4 Assistant Professor of Pathology, Osmania Medical College, Hyderabad Corresponding Author: Dr. P. Padmavathi E-mail- [email protected] Abstract: Background: A lot of Ocular Surface Squamous Neoplasia(OSSN) may go unnoticed as they are asymptomatic and usually slowgrowing. The prevalence of OSSN is more in tropical region and varies from 12 cases /million/ year in Uganda to 0.3 cases / million/ year in United States of America, possibly related to sunlight exposure. Objective: To study the clinical behaviour of Ocular Surface squamous neoplasia (OSSN) in HIV positive patients. Methods: This is a retrospective study done at the department of the oculoplastics and orbital diseases, Sarojini Devi Eye Hospital Hyderabad over a period of three years from February 2012 to January 2015. 26 cases of HIV positive patients with OSSN were included in the study and non HIV cases were excluded. Results: In our study of 26 cases 17 were males and 9 were females and average age of presentation was 34 years 10 to 15 years younger than non HIV cases. Histopathologically 18 cases were squamous cell carcinoma, 5 cases were carcinoma in situ and 3 cases were with moderate to severe dysplasia. Recurrence was seen in 8 cases (32.1%). Conclusion: OSSN presents at a younger age in HIV positive patients with aggressive behaviour clinically and histopathologically with more chances of recurrence. Keywords: OSSN; HIV association; Clinical profile; Recurrence INTRODUCTION: The term Ocular Surface Squamous Neoplasia [OSSN] is first described by Lee and Hirst in 1995 and it refers to the entire spectrum of dysplastic, pre-invasive and malignant squamous lesions of the conjunctiva and cornea1. A lot of them may go unnoticed as they are asymptomatic and usually slow-growing. The prevalence of OSSN is more in tropical region and varies from 12 cases /million/ year in Uganda2 to 0.3 cases / million/ year in United States of America3, possibly related to sunlight exposure. The two main risk factors are UV- B light and Human Papilloma Virus4. The other risk factors are exposure to petroleum products, heavy cigarette smoking and Infection with HIV is now emerging as a major risk factor 5, 6. The following are the clinical varieties. Gelatinous- Most common type. Nodular type- Rapidly growing and diffuse type is slow growing and mimics chronic conjunctivitis Leukoplakic- Usually pre-invasive represents secondary hyperkeratosis Papilliform- Typically exophytic, strawberry like, with a stippled red appearance Corneal OSSN is usually an extension of conjunctival squamous neoplasia. SCC is the final stage of this tumour where dysplastic epithelium invades beyond the basement membrane to the conjunctival substantia propia or corneal stroma. An advanced lesion or mass that is immobile and fixed to the globe should be suspected for invasion. First site of extra ocular involvement is regional lymph nodes, but metastases are rare. Management includes surgery by “No touch technique” proposed by Sheilds et al is a widely accepted surgical approach as the conjunctival component MRIMS Journal of Health Sciences, Vol. 4, No. 2, April-June 2016 Page 114 Padmavathi P et al. Clinical behaviour of OSSN in HIV positive patients along with Tenon’s fascia are excised followed by double freeze thaw Cryotherapy at the conjunctival margin and alcohol epitheliectomy for the corneal component followed by MMC 0.04% four times a day for 4 weeks1,7,8,9,10 . differentiated in 7cases and poorly differentiated carcinoma in 2 cases (TABLE 1) MATERIAL AND METHODS This is a retrospective study conducted at the department of the oculoplastics and orbital diseases, Sarojini Devi Eye Hospital Hyderabad over a period of three years from February 2012 to January 2015. 26 cases of HIV positive patients with OSSN were included in the study and non HIV cases were excluded. All cases were evaluated thoroughly by detailed examination under slit lamp, recorded visual acuity and subjected for basic haematological investigations. Anterior segment OCT and Computed Tomography (CT) were ordered wherever required. Histopathological variant Well differentiated SCC Surgical excision of lesion with 4 mm of normal conjunctiva and cryoapplication to scleral bed was done [TABLE/FIGURE 2A &2B] for 25 patients and one patient who presented with massive swelling and ocular myiasis [TABLE/FIGURE 5] was treated conservatively in view of her emaciated status and sent to MNJ cancer hospital for palliative treatment after confirming diagnosis by biopsy and histopathological examination. All surgically excised lesions were sent for HPE. All cases were treated postoperatively with 4-6 cycles of topical Mitomycin C 0.04% drops , which is used 4 times a day for 4days with 3days off in a week for 4-6weeks. Patients were followed for a maximum of 9 months and were lost for follow up after that. The patients with recurrences were investigated by taking OCT and CT to know the involvement of the coats of the eye ball. Two patients with invasion into ocular coats were advised Enucleation. Six cases which presented with recurrence were subjected for resurgery with a similar technique and kept on topical Mitomycin drops for 8weeks. RESULTS A total of 26 HIV positive cases were studied. Among these 17 cases were males and 9 cases were females. Average age of presentation was 34 years 10 to 15 years younger than non HIV cases. Males were presenting at an younger age (mean age for females 37.9 and for males 32.52) Out of 26 patients 21 knew they had HIV infection and 5 patients were found to be HIV positive after they were investigated when presented with OSSN. The lesions were larger in size compared to non HIV cases. (FIGURE 3] All the lesions were having feeder vessels. Two lesions presented with pigmentation. FIGURE 2] Complete surgical excision with 4 mm of normal conjunctiva (no touch technique) and cryo application for scleral bed (double freeze thaw technique) was done for 25 cases and specimens sent for histopathological examination. Post operatively all cases were prescribed topical MMC drops 0.04 percent QID for 4-6 weeks. Recurrences were seen in 8 cases (32%) in one case as early as two weeks. HPE reports have shown 18 cases of SCC, 5 cases of CIS, 3 cases of moderate to severe dysplasia. In 18 cases of SCC well differentiated carcinoma was seen in 9 cases, moderately Table1 Distribution of histopathological variant of OSSN Percentage Number 34.61% 9 Moderately Differentiated SCC Poorly differentiated SCC Carcinoma in situ 26.92% 7 7.69% 2 19.23% 5 Moderate dysplasia – severe 11.53% 3 DISCUSSION: In the present study, we observed that Males were affected predominantly which was observed in various other studies done on OSSN11. In a study done in Sub-Saharan population females were more commonly affected than males 12. Average age of presentation was 34 years. 10 to 15 years younger than non HIV cases. Males were presenting at an younger age (mean age for females 37.9 and for males 32.52 ). A similar trend was observed in other studies where OSSN has become more prevalent and aggressive with young people being affected mostly13,14,15,16,17. About 20% of patients had OSSN as their first manifestation of HIV infection. Histopathological data showed higher grade of malignancy [CIS+all grades of SCC] [FIGURE 5 &6] in around 88% of study subjects, a similar prevalence which was also noted in other studies of HIV infected OSSN population 4, 12, 18. Recurrences in our study were seen in 8 cases constituting 32%, a similar rate of higher recurrence was also observed in studies done in Sub-Saharan Africa by Nkomazana O et.al.19, and Khokhar S et.al.20 CONCLUSION: Any young patient presenting with OSSN should be investigated for HIV as OSSN could be the first manifestation of HIV infection and followed up frequently as recurrences are common in patients with HIV and treated aggressively REFERENCES: 1. Lee GA, Hirst LW. Ocular surface squamous neoplasia. Surv Ophthal. 1995;39:429-50. 2. Templeton AC. Tumours of the eye and adnexa in Africans in Uganda. Cancer. 1967;20:1689-98. 3. Sun EC, Fears TR, Goedert JJ. Epidemiology of Squamous cell conjunctival cancer. Cancer Epidemiol Biomarkers Prev. 1997;6:73-7. 4. Karcioglu ZA, Wagoner MD. Demographics, etiology, and behaviour of conjunctival squamous MRIMS Journal of Health Sciences, Vol. 4, No. 2, April-June 2016 Page 115 Padmavathi P et al. Clinical behaviour of OSSN in HIV positive patients 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. cell carcinoma in the 21 st century. Ophthalmology. 2009;116:2045-46. Basti S, Macsai MS. Ocular surface squamous neoplasia: A review. Cornea. 2003;22: 687-704. Weinstein JE, Karp CL. Ocular surface neoplasias and human immunodeficiency virus infection. Curr Opin Infect Dis 2013;26:58-65. Shields CL, Shields JA. Tumors of the conjunctiva and cornea. Surv Ophthalmol 2004;49:3-24. Shields CL, Demirci H, Karatza E, Shields JA. 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A review of the etiology of squamous cell carcinoma of the conjunctiva . Br J Cancer . 1996;74:1511-13. Ni C, Searl SS, Kriegstein HJ, Wu BF. Epibulbar carcinoma. Int Ophthalmol Clin. 1982;22:1-33. Hertle RW, Durso F, Metzler JP. Epibulbar squamous cell carcinomas in brothers with Xeroderma pigmentosa. J Pediatr Ophthalmol Strabismus. 1991;28:250-53. Iliff WJ, Marback R, Green WR. Invasive squamous cell carcinoma of the conjunctiva. Arch Ophthalmol. 1975;93:119-22. Guech-Ongey M, Engels EA, Goedert JJ, Biggar RJ, Mbulaiteye SM. Elevated risk for squamous cell carcinoma of the conjunctiva among adults with AIDS in the United States. Int J Cancer 2008;122:2590-93. Nkomazana O, Tshitswana D. Ocular complications of HIV infection in sub-Sahara Africa. Curr HIV /AIDS Rep. 2008;5:120-5. Khokhar S, Soni A, Singh Sethi H,et al. Combined surgery, Cryotherapy and Mitomycin C for recurrent ocular surface squamous neoplasia. Cornea. 2002;21:189-91. Figure 1 Figure 1 A. LESION WITH FEEDER VESSELS 1B. FOUR WEEKS AFTER EXCISION OF LESION Figure 2 RAISED PIGMENTED LESION MRIMS Journal of Health Sciences, Vol. 4, No. 2, April-June 2016 Page 116 Padmavathi P et al. Clinical behaviour of OSSN in HIV positive patients Figure 6 Figure 3 WELL DIFFERENTIATED SCC A LARGE LESION ENCROACHING THE CORNEA Figure 5 Figure 4 MASSIVE TUMOUR WITH ORBITAL EXTENSION CARCINOMA IN-SITU Cite this article as: Padmavathi P, Padma Prabha D, Mohd. Ather, Kavitha T, Padma M. Clinical behaviour of ocular surface squamous neoplasia (OSSN) in HIV positive patients. MRIMS J Health Sciences 2016;4(2):114-117. Source of Support: Nil. Conflict of Interest: None. MRIMS Journal of Health Sciences, Vol. 4, No. 2, April-June 2016 Page 117