Hrev_master [Eye Reports 2011; 1:e7] [page 17] Oncocytoma of the upper conjunctival fornix Zaina Al-Mohtaseb, Seongmu Lee, Michael T. Yen Cullen Eye Institute, Department of Ophthalmology, Baylor College of Medicine, Houston, Texas, USA Abstract Oncocytomas are tumors characterized by large, eosinophilic epithelial cells with abun- dant mitochondria that form ductular or glandu- lar spaces. While these tumors have been described in other organs, those of the ocular adnexa occur infrequently, with the caruncle being the most common site of involvement. Conjunctival oncocytomas are extremely rare and are believed to arise from the ductal ele- ments of the lacrimal gland proper and the accessory lacrimal glands of the conjunctiva. We describe the clinical and histological features of a case of an oncocytoma presenting as an atyp- ically located superior fornix mass in a 78-year- old female, with a review of the literature. Case Report Oncocytomas are tumors characterized by large, eosinophilic epithelial cells with abun- dant mitochondria that form ductular or glan- dular spaces. While these tumors have been described in many organs including the sali- vary, thyroid, adrenal glands, and kidneys, those of the ocular adnexa are uncommon and are believed to arise from metaplasia of the ducts of the lacrimal gland epithelium. The caruncle is the most common site of involve- ment, representing approximately 3% of tumors in this region.1-3 Oncocytomas of the conjunctiva, however, are extremely rare.1-6 We report a case of an atypically located conjuncti- val oncocytoma and review the literature. A 78-year-old female was referred for evalu- ation of fornix lesion. Examination revealed a round, well-circumscribed, mobile, elevated red lesion measuring 3.0¥3.0¥3.0 mm in the bulbar conjunctiva of the superior fornix near the right medial canthus, concealed under the eyelid (Figure 1). Histological examination showed a well-demarcated mass composed of tubular epithelial structures with a central lumen (Figure 2). The cells showed uniform round/oval nuclei surrounding central lumina and prominent basement membrane. The cyto- plasm of the epithelial cells contained promi- nent eosinophilic granules that stained with Masson trichrome. The luminal border of the tubules also stained with PAS and colloidal iron. A diagnosis of oncocytoma was made. Discussion Oncocytomas of the ocular adnexa are uncommon and have been described in the Eye Reports 2011; volume 1:e7 Correspondence: Seongmu Lee, Cullen Eye Institute, Department of Ophthalmology, Baylor College of Medicine, 6565 Fannin NC-205, Houston, Texas 77030, USA. Tel. +1.713.798-3231 - Fax: +1.713.798.8739. E-mail: SeongL@bcm.edu Key words: oncocytoma, oxyphil adenoma, con- junctival, lacrimal, caruncle. Contributions: the authors contributed equally. Conflict of interest: the authors report no con- flicts of interest. Funding: supported in part by an unrestricted educational grant from Research to Prevent Blindness, Inc. (New York, NY). Received for publication: 22 June 2011. Accepted for publication: 22 August 2011. This work is licensed under a Creative Commons Attribution NonCommercial 3.0 License (CC BY- NC 3.0). ©Copyright Z. Al-Mohtaseb et al., 2011 Licensee PAGEPress, Italy Eye Reports 2011; 1:e doi:10.4081/eye.2011.e7 Figure 1. Anterior segment image revealing a round, well-circumscribed, mobile, ele- vated red lesion measuring 3.0¥3.0¥3.0 mm in the bulbar conjunctiva of the supe- rior fornix concealed under the eyelid. Figure 2. A) Histological examination showed a well-demarcated mass composed of tubu- lar epithelial structures with a central lumen consistent with oncocytoma. B) The cyto- plasm of the epithelial cells contained prominent eosinophilic granules that stained with Masson trichrome. The luminal border of the tubules also stained with C) PAS and D) colloidal iron. [page 18] [Eye Reports 2011; 1:e7] caruncle, lacrimal sac, lacrimal gland, and accessory glands of Krause.3,4 Conjunctival oncocytomas are extremely rare.1-5 The tumor location in the conjunctival fornix in this case is particularly atypical. Of the reported cases in the published English literature, the average age was 68-years, and 60% were female. The duration of symptoms ranged from two months to five years, with no evidence of recurrence reported after excision (Table 1).1-5,7-8 Clinically, oncocytomas have been described as red-brown or yellow-tan lesions that are ele- vated and at times lobulated or cystic. Histopathologically, these tumors are charac- terized by a proliferation of large, uniform, polygonal epithelial cells that exhibit abun- dant, fine granular eosinophilic cytoplasm and contain dark round to ovoid paracentral nuclei. The cytoplasm contains dark granules that are positive for modified phosphotungstic acid- haematoxylin and immunostain with mono- clonal antimitochondrial antibodies due to the high density of mitochondria.1 The oncocytes form solid cords and tubular structures that occasionally display papillary infoldings and have a characteristic absence of inflammatory cells. In some areas, the tubules form large cystic spaces, which contain a seromucinous material that is periodic acid schiff and alcian- blue positive.3,6 Many authors believe that oncocytomas arise from age-related oncocytic metaplasia of ducts and acinar cells of the accessory lacrimal glands of the ocular adnexa, while others sug- gest oncocytic transformation of the caruncu- lar surface and conjunctival epithelium as the site of origin.1,3,6 In a more recent study, Ostergaard et al. examined the cytokeratin profile of oncocytic lesions of the ophthalmic region to investigate the origin of these tumors. The authors reported that oncocy- tomas of the ocular adnexa demonstrated a profile similar to that of the lacrimal gland proper and the accessory lacrimal gland duct elements, and that all lesions showed a similar expression pattern irrespective of histological differentiation, suggesting that oncocytic metaplasia, hyperplasia, and oncocytoma may represent the same type of lesion in different stages of development.1 In summary, oncocytomas of the conjunctiva are benign epithelial tumors most likely aris- ing from ductal elements of the lacrimal gland proper and accessory lacrimal glands of the conjunctiva and may present in the conjuncti- val fornix. References 1. Ostergaard J, Prause JU, Heegaard S. Oncocytic lesions of the ophthalmic region: a clinicopathological study with ArticleCase Report Ta bl e 1. S um m ar y of r ep or te d ca se s of c on ju nc tiv al o nc oc yt om a: c lin ic al fe at ur es a nd o ut co m es Ca se St ud y Ag e Ge nd er D ur at io n D ia gn os is Cl in ic al Lo ca tio n Si ze ( m m ) Tr ea tm en t Re cu rr en ce Ye ar o f s tu dy of le si on de sc rip tio n 1 Bi gg s 65 F 3 m on th s On co cy to m a c om bi ne d wi th Pe du nc ul at ed m as s Bu lb ar co nj un ct iva , n ea r p lic a s em ilu na ris * Ex cis io n No 19 77 sq ua m ou s ce ll p ap ill om a 2 Bi gg s 72 F * On co cy to m a * Bu lb ar co nj un ct iva , 1 m m fr om p lic a s em ilu na ris 1 Ex cis io n No 19 77 3 Bi gg s 55 M 2 m on th s On co cy to m a * Co nj un ct iva at lo we r f or ni x * Ex cis io n No 19 77 4 Bi gg s 80 M * On co cy to m a Lo bu lat ed ch er ry -re d m as s Co nj un ct iva at lo we r f or ni x 15 X 12 Ex cis io n No 19 77 5 Re id el * * * On co cy to m a * Co nj un ct iva * Ex cis io n No 19 83 6 Gr os sn ikl au s 87 M * On co cy to m a * Bu lb ar co nj un ct iva * Ex cis io n No 19 87 7 Sp ra ul 72 F 2 y ea rs On co cy to m a Ch er ry re d le sio n Bu lb ar co nj un ct iva n ea r p lic a s em ilu na ris * Ex cis io n No 19 96 8 Pe rc or el la 55 M 2 m on th s On co cy to m a Sw el lin g Co nj un ct iva at lo we r f or ni x * Ex cis io n No 19 97 9 Pe rc or el la 68 F * On co cy to m a Pa pi llo m a Co nj un ct iva at m ed ial ca nt hu s * Ex cis io n No 19 97 10 Ku rli 49 F 5 y ea rs On co cy to m a El ev at ed re d- or an ge tu m or Co nj un ct iva ad jac en t t o ca ru nc le * Ex cis io n No 20 06 11 Os te rg aa rd * * * On co cy to m a * Co nj un ct iva * Ex cis io n No 20 09 12 Al -M oh ta se b 78 F un kn ow n On co cy to m a M ob ile , e le va te d re d tu m or Co nj un ct iva at su pe rio r f or ni x 3 X 3 X 3 Ex cis io n No 20 10 *I nf or m at io n un av ail ab le [Eye Reports 2011; 1:e7] [page 19] emphasis on cytokeratin expression. Acta Ophthalmol 2011;89:263-7. 2. Kurli M, Finger PT, Garcia JP Jr, Schneider S. Peribulbar oncocytoma: high-frequency ultrasound with histopathologic correla- tion. Ophthalmic Surg Lasers Imaging 2006;37:154-6. 3. Biggs SL, Font RL. Oncocytic lesions of the caruncle and other ocular adnexa. Arch Ophthalmol 1977;95:474-8. 4. Pecorella I, Garner A. Ostensible oncocy- toma of accessory lacrimal glands. Histopathology 1997;30:264-70. 5. Grossniklaus HE, Green WR, Luckenbach M, Chan CC. Conjunctival lesions in adults: A clinical and histopathologic review. Cornea 1987;6:78-116. 6. Rennie IG. Oncocytomas (oxyphil adeno- mas) of the lacrimal caruncle. Br J Ophthalmol 1980;64:935-9. 7. Reidel K, Stefani FH, Kampik A. Oncocytoma of the ocular adnexa. Klin Monatsbl Augenheilkd 1983;182:544-8. 8. Spraul CW, Lang GK. Oncocytoma of the conjunctiva. Klin Monbl Augenheilkd 1996;209:176-7. Case Report