SECTION EDITOR: W. RICHARD GREEN, MD Intraocular Neoplastic Cyst From Mucoepidermoid Carcinoma of the Conjunctiva Kaan Ginditz, MD; Carol L. Shields, MD; Jerry A. Shields, MD; Gary Mercado, MD; Ralph C. Eagle, Jr, MD 180-year-old woman with recurrent conjunctival mucoepidermoid carcinoma devel- oped intraocular inflammation and an elevated fundus lesion that simulated choroi- dal detachment in her affected right eye, Intraocular invasion of squamous cell car- cinoma was suspected and the eye was enucleated. Pathologic examination of the enucleated eye showed intraocular invasion by conjunctival mucoepidermoid carcinoma that formed a suprauveal cyst lined with malignant epithelial cells. The patient developed an orbital recur- rence 1 year later and underwent orbital exenteration. She died 2 years later from an unrelated cause. Conjunctival mucoepidermoid carcinoma can exhibit intraocular invasion and produce an intraocular neoplastic cyst Mucoepidermoid carcinoma arises mainly tm the salivary glands, but has also been noted in the upper respiratory tract and rarely in other sites.' In the ocular re- gion, itcan affect the eyelid, conjunctiva caruncle, lacrimal gland, lacrimal sac, and the paranasal sinuses." Conjunctival mu- coepidermoid carcinoma has a tendency to recur alter excision and invade the eye and the orbit."? We deseribe a patient with recurrent conjunctival mucoepidermoid carcinoma that demonstrated intraocular invasion and formed a large intraocular cyst lined by neoplastic cells. REPORT OF A CASE An 89-year-old woman developed an exo- phytic papillary conjunctival lesion in her pseudophakic right eye, affecting the na- sal bulbar and limbal conjunctiva be- toween the 2-o'elock and 4-o'clock posi- ons. The lesion was treated with wide excision and cryotherapy. Histopatho- logic examination revealed conjunctival squamous cell carcinoma with focal ru cin production, consistent with mucoepi- dermoid carcinoma (Figure 1). She had a recurrence 3 months later (Figure 2), From the Oncology Service (Drs Gindiz,C.L. Shiels]. A. shields, and Mercado) «and Pathology Department (Dr Eagle), Wills Eye Hospital, Thomas eferson University, Philadelphia, Pa Downloaded From: on 01/21/2018 Arch Ophthalmol, 1998;116:1521-1523 and was again treated with wide excision and cryotherapy. A wound leak through scleral dehiscence was discovered during, the second surgery but was successfully treated with a patch graft of Tenon fascia and conjunctiva The patient had ocular paits2 months later. Ophthalmic examination disclosed aared epibulbar mass atthe prior site. There was no wound leak or hypotony, but marked intraocular inflammation was found. An elevated fundus lesion reser- bling choroidal detachment was ob- served ophthalmoseopically through hazy nflammed media. The mass was located inferiorly between the 5-o'elock and ‘o'clock positions and measured approxi- mately 1010 mm in basal diameter. B- scan ultrasonography showed a 9.5-mm- thick, acoustically hollow cystic lesion in the suprauveal space (Figure 3). A neo- plastic cyst caused by intraocular inva- Sion of the conjunctival malignant neo- plasm was suspected based on these findings. The patient underwent modi- fied enucleation with wide tenonectomy and conjunctivectomy to include the re- current bulbar tumor. Results ofa histopathologic examina tion showed that the conjunctival earcl- roma had invaded the interior of the through a scleral wound, The tumor had grown posteriorly through a cyclodialysis (©1908 American Medical Association, All sights reserved.Figure 1. Canunctal biopsy specimen shows conunctva squamous ce carcinoma with fc mucin figure 2. Aner segment view hows the eurent macopie fanjuncta and mb ar) figure 3.B-scanuasonogram shows 2 9S:marstick acoustalybolow inacul eta). Downloaded From: on 01/21/2018 cleft into the supraciliary and su- prachoroidal space, forming 4 neo- plastic eyst (Figure 4). ‘The cyst was lined by tumor cells growing on the inner surface of the sclera and the outer surface of the detached uves. No mucin production was identi- fied intraocilatly or inthe tumor on the outer surface of the globe The patient developed mas- sive orbital recurrence 1 year alter enucleation. An eyelid-splituing or bital exenteration was performed. Histopathologically, the tumor ex- hibited squamous cell carcinoma but rho mucts-producing cells we dent. The patient had no further r currence and died 2 years later of cardise disease COMMENT Squamous cell carcinoma ofthe con- junctiva generally occurs in older individuals with a history of ex- cessive sun expostire, The iimor typically is located in the interpal- pebral region near the corneo- scleral limbus, Squamous cell eat cinoma of the conjunctiva tends to be superficially invasive. Deep in traociilar invasion is uncommon, Mucoepidermoid carcinoma, ant of squamous cell carcinoma, is more locally aggressive. Ina review of 12 cases of conjunctival muco- idermoid carcinoma from the Et plish-language literature, 6 cases were found to have intraocular in- vasion and 4 cases had orbital in- volvement. No intraocular oF of bital involvement was found in the remaining 2 patients.’ Intraocular in- vasion in conjunctival squamous cell ia may simulate intraoci- lar inflammation.” Similarly, the in- traocular invasion was heralded by marked intraocular inflammation in our patient The recommended te conjunctival squamous ce ind mucoepidermoid carci- noma includes alcohol corneal epi- theliectomy, wide surgical excision with uimor-free margins, and eryo- therapy of the remaining unin- volved margins.” Radiation treat- ment, either with B-irradiation oF plaque radiotherapy, has been ad- vocated to reduce the risk of recur- ment of rence in conjunctival mucoepider- oid carcinoma,”conjunctival mucoepidermotd car ‘cinoma should raise suspicion of in- traocular invasion by the tumor. Accepted for publication July 8, 1998. This study was supported by the Pennsylvania Lion's Sight Conserva- tion and Eye Research Foundation, Philadelphia (Drs Gundiz, C. L. Shields, and J. A. Shields), the Paul Kayser International Award of Merit in Retina Research, Houston, Tex (Dr J.-A. Shields), the Eye Tumor Re- search Foundation (Dr C. L. Shields), Philadelphia, and the Macula Foun. dation (Drs Gundiz, C. L. Shields, J-A Shields), New York, NY. Reprint requests: Jerry A. Shields, MD, Oncology Service, Wills Eye Hos” pital, 900 Walnut St, Philadelphia, PA 19107. Pag, Vev tense tuner ("sane spe ld sas el ccna (pe tint unc iy cna i ap tig ga or (create Asta Babe ro aca oui Sgmcat scan 5 ee — uns oie mgnian 1 ced mp 0) 1 oR ou stra had 2 unusual fea- Recurrent conjunctival muco- Tecoma: clmcapaolge stat fe our pe (aes, anc 07638600170. tures. In the reported cases ofinira- epidermoid carcinoma generally ex- Cclarinvasionby conjunctival mur bits fewer mucus-ecreting cells Soman btm means cepidermeidearcinoma, the tmor than the original amor! The recur. ony eae ee gencally involved theanterioruvea rent conjunctival and orbital tu- Sata Kegan Mba DG 8. SSasolidmasand there asnocvi. —morswerecomposedemticlyefsqta.——-krmapaennres ntl a dence of a fundus lesion." The oc- mous carcinoma cells in our patient. ‘noma wth ntaocuar invasion and diphasic mor- fol. Ach Ophhalne 198210010811 imal Erman A, Car. Maco ld aceama ofthe cane, Art Oph hot 1084912790. 781 ‘currence of a neoplastic eyst in the A prior report! noted that the suprauveal space that simulated a scleral defect from prior surgery ps choroidal detachment,asin our pa- disposed the eye to the develop- 5. Cara, Sknan Gi RE Mini tient, is exceptionally rare. In addi- ment of intraocular invasion through, Iidercrama tthe conus, Ota Past tion the intraocular invasion from recurrent conjunctival squamouscell Puan Su 1H 1 conjunctival mucoepidermotd car carcinoma, The scleral defect ob- © SH JA Shs. Conti sara ‘inomais generally characterized by served during our patient's second ‘fuss Sand Gretna Tans Pa the presence of mucus-secreting conjunctivalexcision probably also, ila PELpneat awe bears nes. cells? Ithasbeen suggested that ine played.arole in the development ot 7 MA Maio Gren WR. ase Su traocular enviromental factors may intrsocularinvaston, Webellevethat aol esas ioe 1 LWW ot TH Zea nec lvaion plovarolcinthiweype oltsucdil, thedelect wastelatd toscleral nes © Lith cnn only squamous epithelial cells with- ated. Intraocular inflammatory signs saeco mos ade Alook at the past... Pigmentons and congenital eatarac, and ls frequently with albinism, total and prt rderemin micropbthal- ts jeri cataract, colaboma of the macula ites, cep ents, extreme myopia, and bps. Retinitis Plgmeniosn cit almost always Be traced back to consanguinity ofthe parents a gresly cxngerated satement-Herman Knapp. Congenial cataracts mostly due to direct transmission and in albinism and congenial uphthalmms consanguin- iy pis the chit ole Congenital malformations ofthe iar ransmitd from generation to generation, A ns hereditary eause for diseases oF malformations ofthe eyes was found by Laqueur most frequently with retinitis Reference: Arch Ophthalmol. 1898;27:576-577, (©1908 American Med Association, All rights reserved. Downloaded From: on 01/21/2018