Photo Essay Conjunctival intraepithelial neoplasia with corneal furrow degeneration Pukhraj Rishi1,2, Carol L Shields1, Ralph C Eagle3 A 68-year-old man presented with redness of left eye since six months. Examination revealed bilateral corneal furrow degeneration. Left eye lesion was suggestive of conjunctival squamous cell carcinoma, encroaching on to cornea. Anterior segment optical coherence tomography (AS-OCT) confirmed peripheral corneal thinning. Fluorescein angiography confirmed intrinsic vascularity of lesion. Patient was managed with “no touch” surgical excision, dry keratectomy without alcohol, cryotherapy, and primary closure. Pathologic examination of removed tissue confirmed clinical diagnosis. Management of this particular case required modification of standard treatment protocol. Unlike the alcohol-assisted technique of tumor dissection described, ethyl alcohol was not used for risk of corneal perforation due to underlying peripheral corneal thinning. Likewise, topical steroids were withheld in the post-operative period. Three weeks postoperatively, left eye was healing well. Hence, per-operative usage of absolute alcohol and post-operative use of topical steroids may be best avoided in such eyes.
Access this article online Website: www.ijo.in DOI: 10.4103/0301-4738.138625
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Key words: Cornea, conjunctival intraepithelial neoplasia, conjunctival squamous cell carcinoma, optical coherence tomography, Corneal Furrow degeneration
A 68‑year‑old man noted redness in his left eye (operating system, OS) for 6 months. Visual acuity was 20/25 right eye and 20/20 OS. Examination revealed bilateral corneal furrow degeneration and coincidental foreign body “rust ring.”[1,2] [Fig. 1] A sessile papillary squamous cell neoplasm was found OS. This measured 10 mm in diameter and overhung the cornea [Figs. 2 and 3].[3] Anterior segment optical coherence tomography (AS‑OCT; Visante OCT 3.0; Carl Zeiss Meditec, Dublin, CA) confirmed 1-2 mm of peripheral corneal thinning.[4] [Fig. 4] Fluorescein angiography established intrinsic tumor vascularity [Fig. 5]. Tumor was surgically managed with “no touch” excision, dry keratectomy without alcohol application, cryotherapy, and primary double‑layer closure.[3] Histopathology disclosed squamous cell carcinoma in situ (CIS) of conjunctival epithelium. Tumor cells were immunoreactive for squamous cell carcinoma marker p63 [Figs. 6 and 7].
Discussion
Figure 1: Bilateral corneal furrow degeneration with additional “rust ring” (arrow) right eye and vascular mass (arrow-head) left eye
Corneal furrow degeneration is painless, bilateral thinning of peripheral cornea between corneal arcus and limbus, predominantly affecting elderly. Commonly, there is no inflammation, vascularization, or induced corneal astigmatism. Subjects are usually asymptomatic. Depending on severity, management involves observation, lubrication, or punctal occlusion.[1,2] Conjunctival squamous cell carcinoma in situ (CIS) of conjunctival epithelium is a malignant neoplasm that usually Oncology Service, Wills Eye Institute, Philadelphia, PA, USA, Shri Bhagwan Mahavir Vitreoretinal Services, Chennai, Tamil Nadu, India, 3Department of Pathology, Wills Eye Institute, Philadelphia, PA, USA 1 2
Correspondence to: Dr. Carol L Shields, Oncology Service, Wills Eye Institute, 840 Walnut Street, Suite 1440, Philadelphia, PA‑19107, USA. E‑mail: [email protected] Manuscript received: 27.07.12; Revision accepted: 18.02.14
Figure 2: Sessile, vascular conjunctival epithelial tumor left eye
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Indian Journal of Ophthalmology
Figure 3: Corneal furrow degeneration with peripheral corneal thinning (arrow-head) and conjunctival tumor overlying cornea (arrow)
Figure 5: Fluorescein angiography highlights vascular pattern of conjunctival tumor
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Figure 4: Anterior segment optical coherence tomography confirms peripheral corneal thinning consistent with corneal furrow degeneration (arrow)
Figure 6: Histopathology discloses sessile papillomatous proliferatoin of atypical squamous cells consistent with squamous cell carcinoma in situ (CIS). Intralesional vessels and characteristic abrupt margin between normal epithelium and the thick plaque of atypical squamous cells are seen in higher magnification image below. (H and E, top × 25, bottom ×100)
arises from limbal stem cells. Part of the spectrum of conjunctival intraepithelial neoplasia (CIN), it may invade through epithelial basement membrane into the conjunctival or corneal stroma or rarely the interior of eye or orbit. Corneal furrow degeneration is not associated with higher incidence of CIN or related malignancies, despite the similar location. Management of CIN involves alcohol‑assisted de‑epithelialization of CIN, complete tumor removal, cryotherapy of margins, and tissue closure. In this case, alcohol was not used for possible risk of corneal perforation in degenerated corneal guttter.[3,5] Similarly, topical steroids or 5‑fluorouracil were avoided. Post‑operative healing was unremarkable. In such cases, alteration of surgical technique should be considered. Figure 7: Atypical squamous cells comprising conjunctival intraepithelial neoplasia (CIN) show positive nuclear staining (brown color) for p63, an immunohistochemical marker for squamous cell carcinoma. (Immunohistochemistry P63, ×250)
Acknowledgement Dr. Jerry A. Shields (JAS) for his help with preparation of this manuscript.
July 2014
Rishi, et al.: CIN and corneal furrow
References 1. Vogt A. Textbook and atlas of slit lamp microscopy of the living eye. In: Yanoff M, Duker JS, editors. Bonn, Wayenborgh Editions, 1981. Sugar A. Conjunctival and Corneal Degenerations. Ophthalmology. Philadelphia: Mosby; 1999. p. 5.6.1‑8. 2. Cohen EJ, Rapuano CJ, Laibson PR, Raber IM. Cornea: Peripheral corneal thinning. In: Rhee DA, Pyfer MA, editors. The Wills Eye Manual, 3rd ed. Philadelphia: Lippincott, William and Wilkins; 1999. p. 97‑101. 3. Shields CL, Shields JA. Tumors of the conjunctiva and cornea. Surv Ophthalmol 2004;49:3‑24. 4. Radhakrishnan S, Rollins AM, Roth JE, Yazdanfar S, Westphal V, Bardenstein DS, et al. Real‑time optical coherence tomography of the anterior segment at 1310 nm. Arch Ophthalmol 2001;119:1179‑85.
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5. Shields JA, Shields CL, DePotter P. Surgical management of conjunctival tumors. Arch Ophthalmol 1997;115:808‑15. Cite this article as: Rishi P, Shields CL, Eagle RC. Conjunctival intraepithelial neoplasia with corneal furrow degeneration. Indian J Ophthalmol 2014;62:809-11. Source of Support: Support provided by the Eye Tumor Research Foundation, Philadelphia, PA (PR, CLS, JAS) and the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Institute. The funders had no role in the design and conduct of the study, in the collection, analysis, and interpretation of the data, and in the preparation, review or approval of the manuscript. Carol L. Shields, M.D. has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Conflict of Interest: None declared.
Copyright of Indian Journal of Ophthalmology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Access this article online Website: www.ijo.in DOI: 10.4103/0301-4738.138625
Quick Response Code:
Key words: Cornea, conjunctival intraepithelial neoplasia, conjunctival squamous cell carcinoma, optical coherence tomography, Corneal Furrow degeneration
A 68‑year‑old man noted redness in his left eye (operating system, OS) for 6 months. Visual acuity was 20/25 right eye and 20/20 OS. Examination revealed bilateral corneal furrow degeneration and coincidental foreign body “rust ring.”[1,2] [Fig. 1] A sessile papillary squamous cell neoplasm was found OS. This measured 10 mm in diameter and overhung the cornea [Figs. 2 and 3].[3] Anterior segment optical coherence tomography (AS‑OCT; Visante OCT 3.0; Carl Zeiss Meditec, Dublin, CA) confirmed 1-2 mm of peripheral corneal thinning.[4] [Fig. 4] Fluorescein angiography established intrinsic tumor vascularity [Fig. 5]. Tumor was surgically managed with “no touch” excision, dry keratectomy without alcohol application, cryotherapy, and primary double‑layer closure.[3] Histopathology disclosed squamous cell carcinoma in situ (CIS) of conjunctival epithelium. Tumor cells were immunoreactive for squamous cell carcinoma marker p63 [Figs. 6 and 7].
Discussion
Figure 1: Bilateral corneal furrow degeneration with additional “rust ring” (arrow) right eye and vascular mass (arrow-head) left eye
Corneal furrow degeneration is painless, bilateral thinning of peripheral cornea between corneal arcus and limbus, predominantly affecting elderly. Commonly, there is no inflammation, vascularization, or induced corneal astigmatism. Subjects are usually asymptomatic. Depending on severity, management involves observation, lubrication, or punctal occlusion.[1,2] Conjunctival squamous cell carcinoma in situ (CIS) of conjunctival epithelium is a malignant neoplasm that usually Oncology Service, Wills Eye Institute, Philadelphia, PA, USA, Shri Bhagwan Mahavir Vitreoretinal Services, Chennai, Tamil Nadu, India, 3Department of Pathology, Wills Eye Institute, Philadelphia, PA, USA 1 2
Correspondence to: Dr. Carol L Shields, Oncology Service, Wills Eye Institute, 840 Walnut Street, Suite 1440, Philadelphia, PA‑19107, USA. E‑mail: [email protected] Manuscript received: 27.07.12; Revision accepted: 18.02.14
Figure 2: Sessile, vascular conjunctival epithelial tumor left eye
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Indian Journal of Ophthalmology
Figure 3: Corneal furrow degeneration with peripheral corneal thinning (arrow-head) and conjunctival tumor overlying cornea (arrow)
Figure 5: Fluorescein angiography highlights vascular pattern of conjunctival tumor
Vol. 62 No. 7
Figure 4: Anterior segment optical coherence tomography confirms peripheral corneal thinning consistent with corneal furrow degeneration (arrow)
Figure 6: Histopathology discloses sessile papillomatous proliferatoin of atypical squamous cells consistent with squamous cell carcinoma in situ (CIS). Intralesional vessels and characteristic abrupt margin between normal epithelium and the thick plaque of atypical squamous cells are seen in higher magnification image below. (H and E, top × 25, bottom ×100)
arises from limbal stem cells. Part of the spectrum of conjunctival intraepithelial neoplasia (CIN), it may invade through epithelial basement membrane into the conjunctival or corneal stroma or rarely the interior of eye or orbit. Corneal furrow degeneration is not associated with higher incidence of CIN or related malignancies, despite the similar location. Management of CIN involves alcohol‑assisted de‑epithelialization of CIN, complete tumor removal, cryotherapy of margins, and tissue closure. In this case, alcohol was not used for possible risk of corneal perforation in degenerated corneal guttter.[3,5] Similarly, topical steroids or 5‑fluorouracil were avoided. Post‑operative healing was unremarkable. In such cases, alteration of surgical technique should be considered. Figure 7: Atypical squamous cells comprising conjunctival intraepithelial neoplasia (CIN) show positive nuclear staining (brown color) for p63, an immunohistochemical marker for squamous cell carcinoma. (Immunohistochemistry P63, ×250)
Acknowledgement Dr. Jerry A. Shields (JAS) for his help with preparation of this manuscript.
July 2014
Rishi, et al.: CIN and corneal furrow
References 1. Vogt A. Textbook and atlas of slit lamp microscopy of the living eye. In: Yanoff M, Duker JS, editors. Bonn, Wayenborgh Editions, 1981. Sugar A. Conjunctival and Corneal Degenerations. Ophthalmology. Philadelphia: Mosby; 1999. p. 5.6.1‑8. 2. Cohen EJ, Rapuano CJ, Laibson PR, Raber IM. Cornea: Peripheral corneal thinning. In: Rhee DA, Pyfer MA, editors. The Wills Eye Manual, 3rd ed. Philadelphia: Lippincott, William and Wilkins; 1999. p. 97‑101. 3. Shields CL, Shields JA. Tumors of the conjunctiva and cornea. Surv Ophthalmol 2004;49:3‑24. 4. Radhakrishnan S, Rollins AM, Roth JE, Yazdanfar S, Westphal V, Bardenstein DS, et al. Real‑time optical coherence tomography of the anterior segment at 1310 nm. Arch Ophthalmol 2001;119:1179‑85.
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5. Shields JA, Shields CL, DePotter P. Surgical management of conjunctival tumors. Arch Ophthalmol 1997;115:808‑15. Cite this article as: Rishi P, Shields CL, Eagle RC. Conjunctival intraepithelial neoplasia with corneal furrow degeneration. Indian J Ophthalmol 2014;62:809-11. Source of Support: Support provided by the Eye Tumor Research Foundation, Philadelphia, PA (PR, CLS, JAS) and the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Institute. The funders had no role in the design and conduct of the study, in the collection, analysis, and interpretation of the data, and in the preparation, review or approval of the manuscript. Carol L. Shields, M.D. has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Conflict of Interest: None declared.
Copyright of Indian Journal of Ophthalmology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
