Presumed Adenocarcinoma of the Retinal Pigment Epithelium in a Blind Eye with a Staphyloma
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CLINICOPATHOLOGIC REPORT SECTION EDITOR: W. RICHARD GREEN, MD Presumed Adenocarcinoma of the Retinal Pigment Epithelium in a Blind Eye With a Staphyloma Chaim Edelstein, MD, FRCSC; Carol L. Shields, MD; Jerry A. Shields, MD; Ralph C. Eagle, Jr, MD he retinal pigment epithelium (RPE) can undergo reactive hyperplasia and metaplasia following a variety of ocular insults. However, true neoplasms of the RPE are rare. We report a case of a papillary adenocarcinoma of the RPE arising in the blind staphylo- matous right eye of a 79-year-old woman with a long history of bilateral posterior staphy- Tlomas who was seen with increasing pain and exophthalmos of the right eye. Findings from ul- trasonography and computed tomography demonstrated linear calcification consistent with osseous metaplasia of the RPE. Progression of the exophthalmos and worsening exposure keratitis led to enucleation of the eye. Gross pathology showed a 79-mm-long globe. Histopathologic findings re- vealed a largely amelanotic papillary adenocarcinoma arising from the RPE. Positive immunore- activity for cytokeratin supported the epithelial origin of the tumor. Adenocarcinoma of the RPE is rare but may develop in a blind eye. Arch Ophthalmol. 1998;116:525-528 True malignancies of the retinal pigment Findings on examination revealed epithelium (RPE) are extremely rare.1-3 The that the right eye could not perceive light RPE, however, can be involved in a re- and was 9-mm proptotic by Hertel exoph- markable variety of pathologic processes thalmometry. The cornea was com- including reactive hyperplasia, congeni- pletely opaque, precluding a view of the tal hypertrophy, combined hamartoma, fundus, and the episcleral and conjuncti- and, less commonly, adenoma.1 We re- val vessels were diffusely vasodilated port herein a case of an adenocarcinoma (Figure 1). There was marked limita- of the RPE found in a blind eye with a large tion of extraocular movement in all direc- posterior staphyloma. tions of gaze. The intraocular pressure OD was 55 mm Hg. The visual acuity was light perception in the aphakic left eye with an REPORT OF A CASE intraocular pressure of 15 mm Hg. Find- ings on fundus examination showed a large A 79-year-old white woman was seen with posterior pole staphyloma and extensive increasing pain and exophthalmos of the myopic degeneration. right eye. The vision in the right eye had Orbital computed tomographic find- been poor since childhood following an al- ings revealed anterior displacement of the kali burn. Ten years prior to our seeing her, right eye by a massive staphyloma and lin- the vision in that eye had been noted to be ear calcification along the eye wall. The no light perception. Cataract surgery was solid, well-circumscribed staphyloma was performed on the left eye, but her vision heterogeneous in density and was con- remained hand motions because of exten- tinuous with the posterior pole of the globe sive myopic degeneration and posterior (Figure 2). The left eye showed a pos- staphyloma. Beginning 3 months prior to terior staphyloma. Magnetic resonance im- our seeing her, her right eye had become aging findings showed a heterogeneous sig- increasingly prominent and painful. nal in the right eye slightly higher than that From the Ocular Oncology Service (Drs Edelstein, J. A. Shields, and C. L. Shields), and of the left eye (Figure 3). Linear signal the Department of Pathology (Dr Eagle), Wills Eye Hospital, Thomas Jefferson voids on both spin-echo sequences were University, Philadelphia, Pa. related to intraocular calcification. The ARCH OPHTHALMOL / VOL 116, APR 1998 525 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 Figure 1. External photograph of the right eye showing pannus and complete Figure 2. Axial computed tomographic scan demonstrating exophthalmos of opacification of the cornea (November 1995). the right globe and linear calcification along the eye wall. Massive lesion filling the orbit is contiguous with the posterior aspect of globe. Figure 3. Axial T1-weighted magnetic resonance imaging scan showing orbital mass behind the right globe with a heterogeneous signal that shows Figure 4. Large, thin-walled posterior staphyloma filled with a mixture of high signal intensity. blood and tumor tissue protrudes from the posterior aspect of the eye. Light-colored necrotic tumor fills the vitreous cavity. C indicates the cornea; arrows, abrupt transition to staphyloma. massive staphyloma involving the in their entirety and fixed in form- markedly atrophic remnants of the right eye showed a heterogeneous aldehyde. iris and ciliary body. A thick layer signal with hyperintense foci on T1- of dense collagen incorporating weighted images and hypointense/ large quantities of metaplastic bone PATHOLOGIC FINDINGS hyperintense foci on T2-weighted covered the inner surface of the images suggestive of blood prod- choroid. Retina was not identified. ucts (oxyhemoglobin/methemoglo- Macroscopically, a dark brown mass A papillary neoplasm composed of bin). The staphylomatous left eye with a smooth multinodular sur- polarized, cuboidal, and columnar demonstrated a normal low signal face protruded from the posterior epithelial cells with moderately intensity on T1-weighted images and surface of the firm right globe. The pleomorphic round to oval nuclei high signal intensity on T2-weighted anteroposterior length of the eye and and nucleoli filled the remaining images. posterior mass was 79 mm. The cor- interior of the globe and posterior The patient was followed up nea was scarred and opacified. The staphyloma. The tumor cells closely for 9 months and was found globe contained bone, necessitat- formed tubules, cords, and papillae to have increasing exophthalmos of ing decalcification prior to section- and rested on prominent connec- the right eye (13 mm) and worsen- ing. After sectioning, tan to brown tive tissue septa (Figure 5). ing exposure keratitis with corneal tumor tissue mixed with blood Although the tumor was largely epithelial breakdown. Because the was found to fill the interior of the amelanotic, a few strands of cells eye was blind and painful, with globe and posterior staphyloma contained melanin, suggesting ori- chronic corneal exposure and im- (Figure 4). gin from the RPE. Twenty-eight minent corneal ulceration, enucle- Microscopically, a thick layer mitotic figures were counted in 40 ation was recommended. Using a of dense fibrous tissue adhered to high-power fields. Focal necrosis modified enucleation approach with the posterior surface of the scarred and extensive intralesional hemor- lateral canthotomy, the globe and and ectatic cornea, obliterating the rhage were present. Parts of the massive staphyloma were removed anterior chamber and enveloping clotted blood were markedly ARCH OPHTHALMOL / VOL 116, APR 1998 526 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 Figure 5. Top, Amelanotic epithelial cells composed of papillary Figure 6. Neoplastic epithelium composed of papillary tumor exhibits adenocarcinoma rest on prominent connective tissue septae. Cells show mild intense positive immunoreactivity for cytokeratin cell adhesion molecule nuclear pleomorphism (hematoxylin-eosin, original magnification 3100). marker CAM 5.2 (immunoperoxidase, original magnification 350). Bottom, Arrows denote septa in this higher-magnification photomicrograph (hematoxylin-eosin, original magnification 3250). degenerated and contained choles- reported by Garner,3 none of the me- In our case, the soft tissue defi- terol clefts and large amounts of tastases were confirmed histologi- nition of computed tomographic hematoidin and hemosiderin pig- cally. Loeffler et al7 reported a case studies was insufficient to differen- ment consistent with chronicity. of RPE malignancy with suspected tiate a solid neoplasm from a hem- The tumor cells showed intense extraocular extension. Some au- orrhagic process. The excellent soft (+4)positiveimmunoreactivityforcy- thors believe that an adenocarci- tissue contrast of magnetic reso- tokeratin cell adhesion molecule noma of the RPE with extraocular nance imaging studies was also un- marker CAM 5.2 (Figure 6) and extension is capable of metastasis to able to differentiate the hemorrhagic moderatelyintense(+2to+3)focalim- distant sites.8 from the neoplastic component munoreactivity for low-molecular Tumors of the RPE usually oc- within the lesion. Furthermore, the cytokeratin AE-1 and vimentin. cur in the peripheral retina but may relatively poor histopathological Staining for melanoma-specific occur close to the optic disc. Vitre- specificity of magnetic resonance im- antigen HMB-45, carcinoembry- ous seeding may occur if the tumor aging does not readily allow differ- onic antigen, S100 protein, and breaks through sensory retina. His- entiating primary from secondary high-molecular weight AE-3 was topathologically, tumors of the cili- uveal or retinal tumors.10 negative. ary pigment epithelium and RPE are This papillary malignancy was classified into vacuolated, tubular, and found coincidentally on histo- COMMENT mixed types. The more anterior tu- pathological sectioning of the mors tend to be vacuolated whereas globe. Following histopathological Although the RPE rarely gives rise the more posterior ones tend to be tu- diagnosis, it was decided not to to true neoplasms,1-3 it readily bular.1,2 Immunohistochemistry find- treat the patient with radiation undergoes reactive hyperplasia and ings