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Adenoma of the Pigment Epithelium of the Ciliary Body Simulating a Malignant Melanoma
ADENOMA O F T H E P I G M E N T E P I T H E L I U M O F T H E CILIARY BODY SIMULATING A MALIGNANT MELANOMA MEIMEI CHANG,
M.D.
New York, New York J E R R Y A. S H I E L D S , Philadelphia,
M.D.
Pennsylvania
AND D A N I E L L. W A C H T E L , Boundbrook,
Acquired tumors of the pigment epithe lium of the ciliary body are rare. 1 - 3 The correct diagnosis is rarely made clinically and most eyes containing pigment epithe lial tumors have been enucleated because of the diagnosis of malignant melano ma.4""9 We report herein a case of a benign adenoma of the pigment epithelium of the ciliary body in a woman in whom the history and clinical findings suggested a malignant melanoma of the ciliary body with invasion of the anterior chamber angle, subluxated lens, cataract, and vitre ous seeding. The correct diagnosis was not made clinically despite the use of ultrasonography and the radioactive phos phorus (32P) uptake test, procedures which have been reliable in differentiat ing between melanoma and pseudomelanoma. 1 0 - 1 3 We emphasize the limita tions of these diagnostic techniques in differentiating uveal melanomas from pigment epithelial tumors.
M.D.
New Jersey No history of previous ocular trauma or inflamma tion was noted. Best corrected visual acuities were R.E.: 6/6 (20/20) and L.E.: 6/12 (20/40). Applanation tensions were R.E.: 10 mm Hg and L.E.: 16 mm Hg. The pupils were round and symmetrically reactive. In the left eye a prominent episcleral blood vessel inferonasally just posterior to the corneoscleral limbus was noted. Slit-lamp biomicroscopy revealed a pigmented lesion involving the peripheral iris infer onasally (Fig. 1). A sector cortical cataract infero nasally adjacent to a pigmented tumor was noted, which was producing a slight subluxation of the lens. Irregular clumps of pigment were present in the retrolental space. Indirect ophthalmoscopy revealed a somewhat irregular, pigmented ciliary body mass approximate ly 7 x 7 x 6 mm in size extending from the 6 to 9 o'clock meridians. Transillumination revealed a shadow measuring 7 mm in diameter corresponding to this area inferonasally. The posterior fundus was unremarkable and the optic disks were physiologi cally cupped. The right eye was normal. Fluorescein studies could not be performed be cause the peripheral location and cataractous chang es precluded adequate visualization and photo graphic documentation. Both immersion (Fig. 2)
CASE REPORT A 27-year-old woman had a two-month history of painless, progressive blurred vision in her left eye. From the Oncology Unit, Retina Service, Wills Eye Hospital (Dr. Shields), Philadelphia, Pennsyl vania; and the Department of Ophthalmology, Lenox Hill Hospital (Dr. Chang), New York, New York. Dr. Wachtel is in private practice. This study was supported in part by the Retina Research and Development Foundation, and the Pennsylvania Lions Sight Conservation and Eye Research Foun dation, Inc., Philadelphia, Pennsylvania. Reprint requests to Jerry A. Shields, M.D., Oncol ogy Unit, Retina Service, Wills Eye Hospital, 1601 Spring Garden St., Philadelphia, PA 19130. 40
Fig. 1 (Chang, Shields, and Wachtel). Left eye, external photograph. Note area of pigmentation in the inferonasal portion of the iris (arrow).
AMERICAN JOURNAL O F OPHTHALMOLOGY 88:40-44, 1979
VOL. 88, NO. 1
PIGMENT EPITHELIAL ADENOMA
Fig. 2 (Chang, Shields, and Wachtel). Immersion B-scan ultrasonogram of ciliary body mass (arrow). and contact (Fig. 3) B-scan ultrasonography re vealed a ciliary body mass with an irregular but well-defined border and acoustic solidity. A-scan ultrasonography showed the mass to have moderate internal reflectivity and a sharp spike between the dome of the tumor and the vitreous cavity (Fig. 4). A transscleral 3 2 P uptake test was positive with an uptake of 150% over the lesion as compared to the control quadrant. 1 0 , 1 1 Results of a thorough physical examination and chest x-rays were normal. Test results of liver en-
Fig. 4 (Chang, Shields, and Wachtel). A-scan ultra sonogram showing moderate internal reflectivity within the tumor (between arrows). Left arrow indi cates tumor base in contact with the sclera. Right arrow indicates interface between tumor and vitre ous cavity.
41
Fig. 3 (Chang, Shields, and Wachtel). Contact B-scan ultrasonogram showing the ciliary body mass (arrow).
zymes (lactic dehydrogenase, serum glutamic pyruvic transaminase, gammaglutamyl transpeptidase) and carcinoembryonic antigen levels were also within normal limits. The final clinical diagnosis was malignant mela noma of the ciliary body with invasion of the anterior chamber angle, subluxated lens, and secon dary cataract. The eye was enucleated and submitted for pathologic study. Gross examination revealed a normal sized eye. Transillumination showed a shadow measuring 7 x 7 mm extending posterior from the corneoscleral limbus in the inferonasal quadrant. The sectioned globe showed slight anterior displacement of the iris and minimal invasion of the anterior chamber angle by a deeply pigmented, slightly irregular but smooth surfaced tumor of the ciliary body. The lens had a focal cortical opacity inferonasally and was slightly subluxated superotemporally by the tumor. Clumps of pigment were present adjacent to the tumor in the retrolental space. Results of the remainder of the examination were normal. Microscopic examination revealed a deeply pig mented mass of the ciliary body (Fig. 5). The tumor infiltrated the ciliary muscle and anterior chamber angle and largely replaced the ciliary epithelium in the anterior portion of the pars plana. Posteriorly at the base of the tumor the pigment epithelium of the ciliary body seemed to end abruptly and disappear into the tumor itself (Fig. 6). Numerous large blood vessels were present in the basal portion of the tumor. The equatorial portion of the lens, in apposi tion to the tumor, showed cataractous changes char-
42
AMERICAN JOURNAL OF OPHTHALMOLOGY
JULY, 1979
Fig. 6 (Chang, Shields, and Wachtel). Base of the tumor. Note abrupt termination of the pigmented ciliary epithelium (arrow). The sclera is in lower portion of the photograph (hematoxylin and eosin, xlO).
Fig. 5 (Chang, Shields, and Wachtel). Microscopicsection of enucleated left globe. Ciliary body mass (arrow) has subluxed the lens and displaced the iris anteriorly. Retina is artifactitiously detached.
acterized by the presence of Morgagnian globules (Fig. 7). Numerous clear vacuoles were present throughout the tumor (Figs. 6 and 7). Higher power microscopy revealed round to oval pigmented tumor cells with granular cytoplasm and large clear intracellular vacuoles (Fig. 8). The pig ment granules bleached with potassium permanga nate. The bleached preparations more clearly showed the large polyhedral cells characterized by abundant cytoplasm, ovoid nuclei, and prominent nucleoli. The nuclei were often compressed by the clear vacuoles against the cell membrane, forming a signet ring appearance (Fig. 9). No mitotic figures were seen. Periodic acid Schiff (PAS) reaction indi cated mild granular cytoplasmic staining. There was no staining of the vacuolated areas with either mucicarmine or Alcian blue. The histopathologic diagnosis was benign adeno ma of the pigment epithelium of the ciliary body. Despite the benign cytologic features, the tumor
Fig. 7 (Chang, Shields, and Wachtel). Morgagnian globules in lens (arrow) adjacent to tumor. The iris is displaced anteriorly. Artifactitious separation has occurred (hematoxylin and eosin, x40).
Fig. 8 (Chang, Shields, and Wachtel). Photomicro graph of tumor. Note characteristic large vacuolated cells and abundant pigment (hematoxylin and eosin, x 100).
VOL. 88, NO. 1
PIGMENT EPITHELIAL ADENOMA
Fig. 9 (Chang, Shields, and Wachtel). Bleached preparation showing tumor cell morphology. Note the abundant cytoplasm, ovoid nuclei, large vacuoles, and signet ring configuration (potassium per manganate, hematoxylin and eosin, x200).
showed malignant behavior as indicated by the invasion of the anterior chamber, increased intraoc ular pressure, subluxated lens, cataract, and vitreous seeding. DISCUSSION
Tumors originating from the pigmented epithelium of the ciliary body and retina are rare and may clinically resem ble malignant melanoma. 1 - 9 As in previ ously reported cases, the lesion in our patient was virtually impossible to differ entiate clinically from a ciliary body mel anoma. The correct diagnosis was not made in this case despite the use of ancillary studies such as ultrasonography 1 2 , 1 3 and the 3 2 P uptake test, 10 ' 11 which have been thought to be reliable methods of differ entiating melanoma from pseudomelanoma. Results of A- and B-scan ultrasono graphy revealed a pattern commonly ob served in melanomas of the ciliary body, particularly if there are areas of necrosis within the tumor. Additionally, the 3 2 P uptake test pro duced a false-positive result in our case. Although statistical reliability of this test in the diagnosis of adenoma of the ciliary pigment epithelium is unknown, a recent study of 500 cases suggested that this test had greater than 96% reliability in differ
43
entiating between benign and malignant tumors. 1 1 However, there were no cases of pigment epithelial tumors in that series. In an earlier report of 41 eyes enucleated after a mistaken diagnosis of malignant melanoma, only two eyes contained tu mors of the ciliary epithelium. However, there was no recorded information as to whether 3 2 P studies were performed in the two cases. 14 Any highly cellular tumor indicating increased metabolic activity would seem to concentrate the isotope more readily. 10 Therefore the 3 2 P uptake test would not be helpful in differentiat ing melanoma from a proliferating tumor of the pigment epithelium. Although there has been a recent trend toward a more conservative approach to the management of uveal tumors, 1 5 most conservative techniques would have been objectionable in our case, because the tumor showed clinical evidence of prolif eration with visual symptoms and dam age to adjacent ocular structures. An iridocyclectomy could have been attempted, but a good visual result would not have been obtained because of the size and extent of the tumor. In selected cases of tumors involving the ciliary body, a biop sy has been helpful in deciding upon definitive therapy. 1 6 - 1 8 However, if ma lignancy is suspected in large and grow ing tumors, enucleation remains the treat ment of choice. SUMMARY
A benign adenoma of the pigment epi thelium of the ciliary body in a 27-yearold woman simulated a malignant mela noma clinically and resulted in enuclea tion. The correct diagnosis was not made clinically despite the use of A- and Bscan ultrasonography and the radioactive phosphorus (32P) uptake test, procedures which are usually reliable in differentiat ing between melanoma and pseudomelanoma. We emphasize the shortcomings of these ancillary tests in differentiating
44
AMERICAN JOURNAL OF OPHTHALMOLOGY
pigment epithelial tumors from nant melanoma.
malig
REFERENCES 1. Reese, A. B.: Hyperplasia and neoplasia of the pigment epithelium. In Boniuk, M.(ed.): Ocular and Adnexal Tumors. St. Louis, C. V. Mosby Co., 1964, pp. 303-307. 2. Greer, C. H.: Ocular Pathology, 2nd ed. Ox ford, Blackwell Scientific Publications, 1972, pp. 189-192. 3. Reese, A. B.: Tumors of the Eye, 3rd ed. Hagerstown, Harper and Row, 1976, pp. 64-88. 4. Fair, J. R.: Tumors of the retinal pigment epi thelium. Am. J. Ophthalmol. 45:495, 1958. 5. Streeten, B. W., and McGraw, J. L.: Tumor of the ciliary epithelium. Am. J. Ophthalmol. 74:420, 1972. 6. Greer, C. H.: Epithelial tumors of the retinal pigment epithelium. Trans. Ophthalmol. Soc. U. K. 72:265, 1952. 7. Font, R. L., Zimmerman, L. E., and Fine, B. S.: Adenoma of the retinal pigment epithelium. Am. J. Ophthalmol. 73:544, 1972. 8. Minckler, D., and Allen, A. W.: Adenocarcinoma of the retinal pigment epithelium. Arch. Oph thalmol. 96:2252, 1978. 9. Wilensky, J. T., and Holland, M. C : A pigmented tumor of the ciliary body. Arch. Ophthal mol. 92:219, 1974. 10. Hagler, W. S., Jarrett, W. H., II, and Hum
JULY, 1979
phrey, W. T.: The radioactive phosphorus uptake test in the diagnosis of uveal melanomas. Arch. Ophthalmol. 83:548, 1970. 11. Shields, J. A.: Accuracy and limitations of the 32 P test in the diagnosis of ocular tumors. An analy sis of 500 cases. Ophthalmology 85:950, 1978. 12. Coleman, D. J.: Reliability of ocular tumor diagnosis with ulatrasound. Trans. Am. Acad. Oph thalmol. Otolaryngol. 77:677, 1973. 13. Ossoinig, K. C , and Blodi, F. C : Diagnosis of intraocular tumors. 3. In Blodi, F. C. (ed.): Current Concepts in Ophthalmology. St. Louis, C. V. Mosby Co., vol. 4, 1974, p p . 264-343. 14. Shields, J. A., and Zimmerman, L. E.: Le sions simulating malignant melanoma of the poster ior uvea. Arch. Ophthalmol. 89:466, 1973. 15. Shields, J. A.: Current approaches to the diag nosis of choroidal melanomas. Surv. Ophthalmol. 21:443, 1977. 16. Shields, J. A., Green, W. R., and McDonald, P. R.: Uveal pseudomelanoma due to post-traumatic pigmentary migration. Arch. Ophthalmol. 89:519, 1973. 17. Shields, J. A., Leonard, B. C., and Sarin, L. K.: Multinodular uveal melanoma masquerading as a postoperative choroidal detachment. Br. J. Oph thalmol. 60:386, 1976. 18. Jakobiec, F. A., and Chattock, A.: The role of cytology and needle biopsies in the diagnosis of ophthalmic tumors and simulating conditions. In Jakobiec, F. A. (ed.): Ocular and Adnexal Tumors. Birmingham, Aesculapius Publishing Co., 1978, p p . 341-358.
M.D.
New York, New York J E R R Y A. S H I E L D S , Philadelphia,
M.D.
Pennsylvania
AND D A N I E L L. W A C H T E L , Boundbrook,
Acquired tumors of the pigment epithe lium of the ciliary body are rare. 1 - 3 The correct diagnosis is rarely made clinically and most eyes containing pigment epithe lial tumors have been enucleated because of the diagnosis of malignant melano ma.4""9 We report herein a case of a benign adenoma of the pigment epithelium of the ciliary body in a woman in whom the history and clinical findings suggested a malignant melanoma of the ciliary body with invasion of the anterior chamber angle, subluxated lens, cataract, and vitre ous seeding. The correct diagnosis was not made clinically despite the use of ultrasonography and the radioactive phos phorus (32P) uptake test, procedures which have been reliable in differentiat ing between melanoma and pseudomelanoma. 1 0 - 1 3 We emphasize the limita tions of these diagnostic techniques in differentiating uveal melanomas from pigment epithelial tumors.
M.D.
New Jersey No history of previous ocular trauma or inflamma tion was noted. Best corrected visual acuities were R.E.: 6/6 (20/20) and L.E.: 6/12 (20/40). Applanation tensions were R.E.: 10 mm Hg and L.E.: 16 mm Hg. The pupils were round and symmetrically reactive. In the left eye a prominent episcleral blood vessel inferonasally just posterior to the corneoscleral limbus was noted. Slit-lamp biomicroscopy revealed a pigmented lesion involving the peripheral iris infer onasally (Fig. 1). A sector cortical cataract infero nasally adjacent to a pigmented tumor was noted, which was producing a slight subluxation of the lens. Irregular clumps of pigment were present in the retrolental space. Indirect ophthalmoscopy revealed a somewhat irregular, pigmented ciliary body mass approximate ly 7 x 7 x 6 mm in size extending from the 6 to 9 o'clock meridians. Transillumination revealed a shadow measuring 7 mm in diameter corresponding to this area inferonasally. The posterior fundus was unremarkable and the optic disks were physiologi cally cupped. The right eye was normal. Fluorescein studies could not be performed be cause the peripheral location and cataractous chang es precluded adequate visualization and photo graphic documentation. Both immersion (Fig. 2)
CASE REPORT A 27-year-old woman had a two-month history of painless, progressive blurred vision in her left eye. From the Oncology Unit, Retina Service, Wills Eye Hospital (Dr. Shields), Philadelphia, Pennsyl vania; and the Department of Ophthalmology, Lenox Hill Hospital (Dr. Chang), New York, New York. Dr. Wachtel is in private practice. This study was supported in part by the Retina Research and Development Foundation, and the Pennsylvania Lions Sight Conservation and Eye Research Foun dation, Inc., Philadelphia, Pennsylvania. Reprint requests to Jerry A. Shields, M.D., Oncol ogy Unit, Retina Service, Wills Eye Hospital, 1601 Spring Garden St., Philadelphia, PA 19130. 40
Fig. 1 (Chang, Shields, and Wachtel). Left eye, external photograph. Note area of pigmentation in the inferonasal portion of the iris (arrow).
AMERICAN JOURNAL O F OPHTHALMOLOGY 88:40-44, 1979
VOL. 88, NO. 1
PIGMENT EPITHELIAL ADENOMA
Fig. 2 (Chang, Shields, and Wachtel). Immersion B-scan ultrasonogram of ciliary body mass (arrow). and contact (Fig. 3) B-scan ultrasonography re vealed a ciliary body mass with an irregular but well-defined border and acoustic solidity. A-scan ultrasonography showed the mass to have moderate internal reflectivity and a sharp spike between the dome of the tumor and the vitreous cavity (Fig. 4). A transscleral 3 2 P uptake test was positive with an uptake of 150% over the lesion as compared to the control quadrant. 1 0 , 1 1 Results of a thorough physical examination and chest x-rays were normal. Test results of liver en-
Fig. 4 (Chang, Shields, and Wachtel). A-scan ultra sonogram showing moderate internal reflectivity within the tumor (between arrows). Left arrow indi cates tumor base in contact with the sclera. Right arrow indicates interface between tumor and vitre ous cavity.
41
Fig. 3 (Chang, Shields, and Wachtel). Contact B-scan ultrasonogram showing the ciliary body mass (arrow).
zymes (lactic dehydrogenase, serum glutamic pyruvic transaminase, gammaglutamyl transpeptidase) and carcinoembryonic antigen levels were also within normal limits. The final clinical diagnosis was malignant mela noma of the ciliary body with invasion of the anterior chamber angle, subluxated lens, and secon dary cataract. The eye was enucleated and submitted for pathologic study. Gross examination revealed a normal sized eye. Transillumination showed a shadow measuring 7 x 7 mm extending posterior from the corneoscleral limbus in the inferonasal quadrant. The sectioned globe showed slight anterior displacement of the iris and minimal invasion of the anterior chamber angle by a deeply pigmented, slightly irregular but smooth surfaced tumor of the ciliary body. The lens had a focal cortical opacity inferonasally and was slightly subluxated superotemporally by the tumor. Clumps of pigment were present adjacent to the tumor in the retrolental space. Results of the remainder of the examination were normal. Microscopic examination revealed a deeply pig mented mass of the ciliary body (Fig. 5). The tumor infiltrated the ciliary muscle and anterior chamber angle and largely replaced the ciliary epithelium in the anterior portion of the pars plana. Posteriorly at the base of the tumor the pigment epithelium of the ciliary body seemed to end abruptly and disappear into the tumor itself (Fig. 6). Numerous large blood vessels were present in the basal portion of the tumor. The equatorial portion of the lens, in apposi tion to the tumor, showed cataractous changes char-
42
AMERICAN JOURNAL OF OPHTHALMOLOGY
JULY, 1979
Fig. 6 (Chang, Shields, and Wachtel). Base of the tumor. Note abrupt termination of the pigmented ciliary epithelium (arrow). The sclera is in lower portion of the photograph (hematoxylin and eosin, xlO).
Fig. 5 (Chang, Shields, and Wachtel). Microscopicsection of enucleated left globe. Ciliary body mass (arrow) has subluxed the lens and displaced the iris anteriorly. Retina is artifactitiously detached.
acterized by the presence of Morgagnian globules (Fig. 7). Numerous clear vacuoles were present throughout the tumor (Figs. 6 and 7). Higher power microscopy revealed round to oval pigmented tumor cells with granular cytoplasm and large clear intracellular vacuoles (Fig. 8). The pig ment granules bleached with potassium permanga nate. The bleached preparations more clearly showed the large polyhedral cells characterized by abundant cytoplasm, ovoid nuclei, and prominent nucleoli. The nuclei were often compressed by the clear vacuoles against the cell membrane, forming a signet ring appearance (Fig. 9). No mitotic figures were seen. Periodic acid Schiff (PAS) reaction indi cated mild granular cytoplasmic staining. There was no staining of the vacuolated areas with either mucicarmine or Alcian blue. The histopathologic diagnosis was benign adeno ma of the pigment epithelium of the ciliary body. Despite the benign cytologic features, the tumor
Fig. 7 (Chang, Shields, and Wachtel). Morgagnian globules in lens (arrow) adjacent to tumor. The iris is displaced anteriorly. Artifactitious separation has occurred (hematoxylin and eosin, x40).
Fig. 8 (Chang, Shields, and Wachtel). Photomicro graph of tumor. Note characteristic large vacuolated cells and abundant pigment (hematoxylin and eosin, x 100).
VOL. 88, NO. 1
PIGMENT EPITHELIAL ADENOMA
Fig. 9 (Chang, Shields, and Wachtel). Bleached preparation showing tumor cell morphology. Note the abundant cytoplasm, ovoid nuclei, large vacuoles, and signet ring configuration (potassium per manganate, hematoxylin and eosin, x200).
showed malignant behavior as indicated by the invasion of the anterior chamber, increased intraoc ular pressure, subluxated lens, cataract, and vitreous seeding. DISCUSSION
Tumors originating from the pigmented epithelium of the ciliary body and retina are rare and may clinically resem ble malignant melanoma. 1 - 9 As in previ ously reported cases, the lesion in our patient was virtually impossible to differ entiate clinically from a ciliary body mel anoma. The correct diagnosis was not made in this case despite the use of ancillary studies such as ultrasonography 1 2 , 1 3 and the 3 2 P uptake test, 10 ' 11 which have been thought to be reliable methods of differ entiating melanoma from pseudomelanoma. Results of A- and B-scan ultrasono graphy revealed a pattern commonly ob served in melanomas of the ciliary body, particularly if there are areas of necrosis within the tumor. Additionally, the 3 2 P uptake test pro duced a false-positive result in our case. Although statistical reliability of this test in the diagnosis of adenoma of the ciliary pigment epithelium is unknown, a recent study of 500 cases suggested that this test had greater than 96% reliability in differ
43
entiating between benign and malignant tumors. 1 1 However, there were no cases of pigment epithelial tumors in that series. In an earlier report of 41 eyes enucleated after a mistaken diagnosis of malignant melanoma, only two eyes contained tu mors of the ciliary epithelium. However, there was no recorded information as to whether 3 2 P studies were performed in the two cases. 14 Any highly cellular tumor indicating increased metabolic activity would seem to concentrate the isotope more readily. 10 Therefore the 3 2 P uptake test would not be helpful in differentiat ing melanoma from a proliferating tumor of the pigment epithelium. Although there has been a recent trend toward a more conservative approach to the management of uveal tumors, 1 5 most conservative techniques would have been objectionable in our case, because the tumor showed clinical evidence of prolif eration with visual symptoms and dam age to adjacent ocular structures. An iridocyclectomy could have been attempted, but a good visual result would not have been obtained because of the size and extent of the tumor. In selected cases of tumors involving the ciliary body, a biop sy has been helpful in deciding upon definitive therapy. 1 6 - 1 8 However, if ma lignancy is suspected in large and grow ing tumors, enucleation remains the treat ment of choice. SUMMARY
A benign adenoma of the pigment epi thelium of the ciliary body in a 27-yearold woman simulated a malignant mela noma clinically and resulted in enuclea tion. The correct diagnosis was not made clinically despite the use of A- and Bscan ultrasonography and the radioactive phosphorus (32P) uptake test, procedures which are usually reliable in differentiat ing between melanoma and pseudomelanoma. We emphasize the shortcomings of these ancillary tests in differentiating
44
AMERICAN JOURNAL OF OPHTHALMOLOGY
pigment epithelial tumors from nant melanoma.
malig
REFERENCES 1. Reese, A. B.: Hyperplasia and neoplasia of the pigment epithelium. In Boniuk, M.(ed.): Ocular and Adnexal Tumors. St. Louis, C. V. Mosby Co., 1964, pp. 303-307. 2. Greer, C. H.: Ocular Pathology, 2nd ed. Ox ford, Blackwell Scientific Publications, 1972, pp. 189-192. 3. Reese, A. B.: Tumors of the Eye, 3rd ed. Hagerstown, Harper and Row, 1976, pp. 64-88. 4. Fair, J. R.: Tumors of the retinal pigment epi thelium. Am. J. Ophthalmol. 45:495, 1958. 5. Streeten, B. W., and McGraw, J. L.: Tumor of the ciliary epithelium. Am. J. Ophthalmol. 74:420, 1972. 6. Greer, C. H.: Epithelial tumors of the retinal pigment epithelium. Trans. Ophthalmol. Soc. U. K. 72:265, 1952. 7. Font, R. L., Zimmerman, L. E., and Fine, B. S.: Adenoma of the retinal pigment epithelium. Am. J. Ophthalmol. 73:544, 1972. 8. Minckler, D., and Allen, A. W.: Adenocarcinoma of the retinal pigment epithelium. Arch. Oph thalmol. 96:2252, 1978. 9. Wilensky, J. T., and Holland, M. C : A pigmented tumor of the ciliary body. Arch. Ophthal mol. 92:219, 1974. 10. Hagler, W. S., Jarrett, W. H., II, and Hum
JULY, 1979
phrey, W. T.: The radioactive phosphorus uptake test in the diagnosis of uveal melanomas. Arch. Ophthalmol. 83:548, 1970. 11. Shields, J. A.: Accuracy and limitations of the 32 P test in the diagnosis of ocular tumors. An analy sis of 500 cases. Ophthalmology 85:950, 1978. 12. Coleman, D. J.: Reliability of ocular tumor diagnosis with ulatrasound. Trans. Am. Acad. Oph thalmol. Otolaryngol. 77:677, 1973. 13. Ossoinig, K. C , and Blodi, F. C : Diagnosis of intraocular tumors. 3. In Blodi, F. C. (ed.): Current Concepts in Ophthalmology. St. Louis, C. V. Mosby Co., vol. 4, 1974, p p . 264-343. 14. Shields, J. A., and Zimmerman, L. E.: Le sions simulating malignant melanoma of the poster ior uvea. Arch. Ophthalmol. 89:466, 1973. 15. Shields, J. A.: Current approaches to the diag nosis of choroidal melanomas. Surv. Ophthalmol. 21:443, 1977. 16. Shields, J. A., Green, W. R., and McDonald, P. R.: Uveal pseudomelanoma due to post-traumatic pigmentary migration. Arch. Ophthalmol. 89:519, 1973. 17. Shields, J. A., Leonard, B. C., and Sarin, L. K.: Multinodular uveal melanoma masquerading as a postoperative choroidal detachment. Br. J. Oph thalmol. 60:386, 1976. 18. Jakobiec, F. A., and Chattock, A.: The role of cytology and needle biopsies in the diagnosis of ophthalmic tumors and simulating conditions. In Jakobiec, F. A. (ed.): Ocular and Adnexal Tumors. Birmingham, Aesculapius Publishing Co., 1978, p p . 341-358.