Case Report

Ophthalmologica 2007;221:200203 DOI: 10.1159/000099302

Received: July 13, 2006 Accepted: September 22, 2006

Intraocular Extension of Conjunctival Squamous Cell Carcinoma

Zhe Zhang Bin Li Jitong Shi Xiaolin Xu Liaoqing Li Fei Gao
Beijing Institute of Ophthalmolog y, Beijing TongRen Eye Center, Capital Universit y of Medical Science, Beijing , China Fig. 1. External photograph of t he eye showing the conjunctival Fig. 2. A chalk-white f riable mass occupied 3/7 space of the ante-

squamous cell carcinoma. Pink elevated tumor mass extends from t he nasal side of the limbus to the lacrimal caruncle.

Key Words Conjunctival squamous cell carcinoma Pterygium

Conjunctivitis

Abstract Conjunctival squamous carcinoma, which is regarded as a low-grade malignancy, usually originates at the limbus. Most cases remain superf icial to the sclera. Intraocular invasion is rarely reported. We describe a woman misdiagnosed as conjunctivitis and pterygium before a clinical diagnosis of conjunctival squamous cell carcinoma. The diagnosis was made by histopathological examination of the biopsy specimen. Examination revealed an elevated mass on the nasal limbus extending intraocularly. White flaky mass occupied approximately 3/7 space of the anterior chamber. Exenteration was performed for control of local lesion. Histopathologic analysis confirmed that intraocular involvement occurs through the emissary vessels near the area of limbus. The case highlights need for accurate diagnosis and prompt intervenGross appearance of sectioned specimen. White tumor Fig. 3. the tion. occ A brief the (dotted clinical and his topathologic feamass upiesreview interior of fornix arrow) and part of anterior chamber arrow). squamous cell carcinoma is also pretures of (black conjunctival sented. Copyright 2007 S. Karger AG, Basel

Histopathological Findings Case Report The eye was sectioned vertically in a somewhat oblique fashion (fig. 3). A 66-year-old female Histopathologic examination presente disclosed d, 12 months a moderate previou tosly, we with ll-difa small pink elevated mass on t he nasal bulbar conjunctiva wit h ferentiated squamou s cell carcinoma that originally arose from the tearing nasal bulbar of her conjunctiva left eye. Ocular extending examination to the fornix, in October nasal 2004 limbal disconclosed a localiz ed the pink lump without effusion position. and bleeding ju nctiva be tween 12-oclock and ulcer, t he 6-oclock The iris on the surface. This lesion was then misdiagnosed as conjunctiroot and ciliary body were infilt rated. The inferior-nasal anterior

rior chamber from the 5-oclock to the 10-oclock position (black arrow). Extending from the nasal side of the iris, it was abnormal in thickness (white arrow). vitis and pterygium. The patient was given anti-inflammatory drugs without any surgical intervent ion by the local opht halmologist. The lesion was progressively increasing in size, and get ting painful with diminished vision for the last 3 months. A pale mass covering t he area of pupil was not iced for about 10 days. The examination at our hospital showed the visual acuit y of 20/80 OD and 20/40 OS wit h normal intraocular pressure. External and slit-lamp ocular examinations revealed a diffuse highly vascularized conjunctival lesion in her left eye, affecting the nasal bulbar, interior fornix and limbal conjunctiva between the 12-oclock and the 6-oclock p osit ion ( f ig. 1). The area of lacrimal caruncle was also involved. An irregular grey frog-eggs-like mass, measuring 8 ! 6 mm in siz e, occupied approximately 3/7 space of the anterior chamber in the inferior-nasal direction ( fig. 2 ). The iris presented nasal thickness, partly superficial depigmentation, superior posterior synechia, but no neovasculariz ation. The shape of t he pupil was not so round without any reaction to light. The lens was opacif ied with anterior subcapsular pigmentation. Bilateral preauricular nodes and submaxillary lymph nodes were untouchable. The right eye was ent irely normal except for an early nasal pterygium. B scan ultrasonography showed an irregular solid lesion on showing a mass solid extended squamous cell Fig. 4. Microscopic the nasal side of thesection eye wall of the lef t eye,of which into carcinoma filling the anterior chamber. The black arrow indicates the orbit. Ultrasound biomicroscopy (UBM) findings consistent Descemets (hematoxylin eosin, original magniwith B scan membrane ultrasonography was the and thickening of the nasal side fication ! root 100). of the iris with infiltration of the ciliary body. MR imaging also revealed abnormal signals of t he nasal angle and the tendon of the medial rectus. Clinically, the patient was diagnosed with conjunctival neoplasm of was chamber the left occupied eye, extending by a chalk-white intraocularly. friable Amass biopsy, between conductthe ed on October demonst ratedwithout t hat theadhesion white tumor mass 5-oclock and t19, he 2005, 10-oclock position to the coradhered the extraocular muscle and sclera. Histopat hologic cells, exnea (f ig.to 4). The tumor was composed of irregu larly arranged ami nati cellular forming on r evea nests led squa and solid mou cords s cell or c arc papillomatous inoma. The structures. me tas tatic car cinoma was excluded by s the whole-body scan. The cells showed variou degrees of cellu lar pleomorphism. Their nuclei Considering were generally the orbital hyperchromatic, and intraocular and invasion, there were exenteration marked was p erformedand polymorphism on November nuclear abnormalities. 11, 2005, toMitotic control figures the local were lesion. numerous and showed frequent atypia. Individually keratinized

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Int raocular Extension of Conjunctival 2007 S. Karger AG, Basel 00303755/07/22130200$23.50/0 Squamous Cell Carcinoma
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Ophthalmologica 2007;221:200 203 Bin Li

Beijing Institute of Ophthalmology, Beiji ng TongRen Eye Center Capital University of Medical Science Beijing, 100730 (China) Tel. +86 10 8333 1507, Fax +86 10 6512 5617, E-Mail libin43_99@yahoo.com

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Fig. 5. Original epibulbar t umor mass. Typical area of the tumor

Fig. 6. Microsc opic appea ra nce of int raoc ula r squamous cell car-

showing squamous pearls (hematoxylin and eosin, original magnification ! 100) .

cinoma. Intraocular extension of the tumor via emissary vessels (black arrow; hematoxylin and eosin, original magnif ication ! 100).

terior pigment epithelium ( fig. 7 ). However, the bulk of ciliary body stroma was replaced by necrotic t umor, the nonpigmented ciliary epithelium had not been disrupted. The tumor cells appeared, but more anaplastic and hyp erchromatic, and there was a marked increase in t he spindle cell type in the stroma of the iris. The differentiation of the cells in the iris was obviously poorer than that in the conjunctiva. The choroid and ret ina did not appear to be affected. But the secondary cataract was noted. The lesion exhibited immunoreactivity for Ki-67, epithelial membrane antigen and cytokeratin.

Discussion

Fig. 7. Microscopic section shows the bulk of ciliary body stroma

was replaced by t umor cells and the nonpigmented ciliary epithelium had not been disrupted (hematoxylin and eosin, original magnification ! 40).

cells (dyskeratosis), concentric collections of keratinized cells (squ amous pearls) and intracellular keratin formation could be fou nd, which allowed us to identif y tumor histogenesis ( fig. 5 ). The emissary vessel was packed with squamous cell carcinoma. The trabecular meshwork and t he inferior-nasal anterior chamber angle were also invaded by the tumor cells ( f ig. 6). The intraocular tumor extended contiguously on the surface of the iris, and infiltrated st roma wit hout breaking through pos-

Conjunctival squamous cell carcinoma is an uncommon di sease that generally occurs in older individuals, and is typically located in the interpalpebral region near the corneoscleral limbus [1, 2]. In most cases, the sclera as well as Bowmans layer a nd adjacent corneal lamellae act as an impenetrable barrier against further i nvasion of the tumor. Thus, conjunctival squamous cell carcinoma tends to be superficia lly i nvasive, and i ntraocu lar i nvasion has been reported to be rare, with 211% of cases [2, 3]. Intraocular involvement probably occurs in cases through its numerous drai nage and nerve channels or the canal left by trauma and surgery. The scleral defect observed during patients first conjunctiv al excision probably played a role in the development of intraocular invasion.

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Ophthalmologica 2007;221:200203

Zhang/Li/Shi/Xu/Li/Gao

Histopathologic examination usually shows i ngrowth findings are suggestive of invasion into the globe or orbit, of the conjunctival squamous cell ca rcinoma through the including an associated scleritis, broad-based synechiae, limbus, with involvement of Schlemms canal, the trabecanterior chamber cells, a contiguous intraocular mass, or ular meshwork, anterior chamber, iris, ciliary body, suspontaneous perforation of the globe. Schlote et al. [7] prachoroidal space, and choroid, sometimes even behind and Shields et al. [8] stated that the intraocular invasion the equator. In one report, intraocular invasion caused also can be heralded by the onset of low-grade inf lammaretina l detachment a nd scleral thinning, leading to spontion and secondary glaucoma. Excisional biopsy before taneous globe rupture [4]. surgery is necessary to differentiate early intraepithelial Three types of invasive conjunctival squamous cell neoplasms that breach the conjunctival basement memcarcinoma with a greater potential for invasion have been brane and invade the underlying stroma. reported, which are spindle cell variant, mucoepiderMost eyes with intraocular invasion of squamous cell moid ca rcinoma and adenoid squamous carcinoma. It is carcinoma are enucleated [8]; in cases with orbital i nvaof note that a lthough the lesion in ou r patient did not fall sion, exenteration is required. L ately Char et a l. [9] rewithi n these subtypes, it still demonstrated invasive beported a successfu l iridocyclochoroidectomy with adhavior. But the tumor cells in the iris stroma were poorly junctive cr yotherapy, avoiding enucleation. Ra monas et differentiated compared with the cells in the conjunctival al.[10] suggested proton beam therapy as a potential alsurface a nd anterior cha mber, and spindle cells could be ternative to enucleation. observed occasionally. We conclude that with the prolifAlthough conjunctival squamous cell carcinoma is eration of the tumor, the spindle cells increase with poorusua lly characteri zed by a mild clinical course, and the er differentiation, which results in the intraocular invadeep invasion of a conjunctival carcinoma are rare, cases sion. are misdiagnosed as a benign process and a long delay in The delineation of conjunctival squa mous cel l carcidiagnosis may result in invasion. This unusual case shows noma invasiveness is importa nt since it alters clinical a number of features of conjunctival squamous cell carmanagement, but the diagnosis of conjunctival carcinocinoma with the eye being treated as conjunctivitis a nd ma as w el l as de l ine at ion o f de e p invasio n c an b e d if pterygium. fic ul t. When the appropriate diagnosis was conSeveral investigators have reported that the use of high firmed by histopathological examination of the biopsy f requency ultr asound combined with t he clinical data alspecimen, intraocular spread had been noted cli nica lly, lows to predict presence or absence of deep involvement which highlights the need for accurate diagnosis a nd [5, 6]. Besides the adjuvant examination, several clinical prompt intervention.

References
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Int raocular Extension of Conjunctival Squamous Cell Carcinoma

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