Benign Melanomas of the Retinal Pigment Epithelium*

Benign Melanomas of the Retinal Pigment Epithelium*

207 LEIOMYOMA OF THE CILIARY BODY benign leiomyoma from the malignant melanoma, which occurs with greater fre­ quency and in the same location, is c...

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benign leiomyoma from the malignant melanoma, which occurs with greater fre­ quency and in the same location, is clinically

important because of the obvious implica­ tions for the patient's life expectancy. Eye and Ear Hospital (13).


Bettman, J. W., and Fellows, V.: Radioactive phosphorus as a diagnostic aid in ophthalmology. Arch. Ophth., 51:171,1954. Blodi, F. C.: Leiomyoma of the ciliary body. Am. J. Ophth., 33:939, 1950. Davis, W. T., Sheppard, E., and Romejko, W. J.: Leiomyoma of the iris. Am. J. Ophth., 27 :467, 1944. Dunphy, E. B., and Selverstone, B.: Uptake of radioactive phosphorus by normal and neoplastic ocular tissues. Am. J. Ophth., 36:774, 1953. Dunphy, E. B., Dreisler, K. K., Cadigan, J. B., and Sweet, W.: Uptake of radioactive phosphorus by neoplasms. Am. J. Ophth., 37 :45, 1954. Eisenberg, I. J., Leopold, I. H., and Sklaroff, D.: Use of radioactive phosphorus in detection of intra­ ocular neoplasms. Arch. Ophth., 51:633, 1954. Erdbrink, W. L., and Harbert, F.: Leiomyoma of the iris. Arch. Ophth., 53:643, 1955. Ewing, J.: Neoplastic Diseases. Philadelphia, Saunders, 1942, ed. 4, p. 224. Fleming, N.: A case of pigmented leiomyoma of the iris. Brit. J. Ophth., 32 :885, 1938. Moulton, E. C, and Moulton, E. C, Jr.: Leiomyomas of the iris. Am. J. Ophth., 31:214, 1948. O'Day, K.: Leiomyomata of the Iris. Brit. J. Ophth., 33 :283, 1949. Reese, A. B.: Tumors of the Eye. New Y'ork, Hoeber, 1951. Rinhard, E. H , Moore, C. V., Bierbaum, O. S., and Moore, S.: Selective uptake of radioactive phos­ phorus by tissues and cells. J. Lab. & Clin. Med., 31:107, 1946. Snodgrass, M. B., Lenihan, J., and Primrose, D. A.: Radioactive phosphorus as an aid in the diagnosis of malignant melanomata of the eye. Brit. J. Ophth., 38 :553, 1954. Terner, I. S., Leopold, I. H., and Eisenberg, I. J.: The radioactive phosphorus uptake test in ophthal­ mology. Arch. Ophth., 55:52, 1956. Thomas, C. I., Krohmer, J. S., and Storaasli, J. P.: Detection of intraocular tumors with radioactive phosphorus. Arch. Ophth., 47 :276, 1952. Thomas, C. I., Krohmer, J. S., Storaasli, J. P., and Friedall, N. L.: Detection of intraocular tumors by the use of radioactive phosphorus. Am. J. Ophth., 38 :93, 1954.





Out of a total of 151 eyes in which a fundus lesion raised the question of malig­ nant melanoma of the choroid, nine eyes showed a singular finding which is our pur­ pose to describe in this communication. It is our belief that these cases represent be­ nign melanomas of the retinal pigment epithelium. It is commonly accepted as a true paradox that the retinal pigment epithelium prolif­ erates readily in response to noxious stimuli of an inflammatory nature but rarely, if * From the Institute of Ophthalmology of the Presbyterian Hospital.

ever, undergoes true neoplastic prolifera­ tion. Contrariwise, the choroidal, pigmentbearing cells do not reproduce their kind in response to stimuli, but they are a common source of benign or malignant melanomas. Consequently, any circumscribed pigmented area of the fundus which does not appear consistent with the usual sequelae of chorioretinitis is regarded by exclusion as a possible benign or malignant melanoma of the cho­ roid. We are confronted, therefore, with the problem of identifying and separating from choroidal tumors as many simulating lesions as possible. Our nine cases, of which seven are here described, were all viewed as suffi-



ciently suspicious of malignant melanoma to warrant its serious consideration. How­ ever, their characteristics seem distinctive, and these we shall attempt to portray in this paper. CLINICAL APPEARANCE

The lesion is unilateral, isolated, black, sharply demarcated, and flat. The shape is irregularly round or oval. The size is one to three disc diameters. The normal retinal vascular pattern courses undisturbed over the lesion. The pigmentation is uniformly black to gray except for occasional small unpigmented areas with sharp borders. These changes are placed in the pigment epithelium because of the black color, the sharp outline, and the clarity of the granular tissue mark­ ings. The pigment areas show no progres­ sive changes. Field defects corresponding to the lesions cannot be elicited. The eyes har­

boring these pigment foci seem otherwise normal and comparable to the fellow eye. The involved eye shows no indication of an acquired pathologic process or foci else­ where in the fundus, suggesting choroiditis. As far as we can say, there are no familial factors. There seems to be no correlation with general body pigment or pigmented lesions. CASES

Case 1. (A. H.) A white woman, aged 42 years. A pigment area was discovered at routine eye examination and a question of a malignant melanoma of the choroid arose. The area was located in the midzone of the lower nasal quadrant of the left fundus. It was circular, two disc diameters in size, black, well circumscribed, slightly pale in the center, and with a granular appearance. The retinal vessels crossed the lesion un­ disturbed.

Fig. 1 (Reese and Jones). Case 1 (A. H.).


Fig. 2 (Reese and Jones). Case 2 (R. A.).

Case 2. (R. A.) A white girl, aged 17 years. A pigmented lesion was discovered in the fundus of the right eye at routine eye examination and the question of a malignant melanoma of the choroid was raised. It was located at the equator in the lower temporal quadrant, was oval, dark gray, and about two disc diameters in the long axis. There were several nonpigmented areas present, one breaking through the border. The ret­ inal vessels crossed undisturbed. Case 3. (S. T.) A white man, aged 54

(Reese and Jones). Case 3 (S. T.).


Fig. 4 (Reese and Jones). Case 4 (C. D.).

years. An area thought to be a melanoma was discovered during an examination for detached retina of the opposite eye. The le­ sion was located below the right macular area, was irregularly round, two disc di­ ameters in size, and densely black with a granular-appearing surface. Case 4. ( C D . ) A white woman, aged 61 years. An irregularly square, dark-gray pig­ mented spot, three disc diameters in size, was discovered incidentally during a fundus examination. The borders were sharp and two nonpigmented lacunae broke through the border. Retinal vessels crossed undis­ turbed. Case 5. (W. K.) A white man, aged 29 years, had a lesion in the left fundus thought suspicious of malignant melanoma. It was located adjacent to the temporal bor­ der of the disc, was rounded with a black granular appearance, and sharply demar­ cated. Several large, pinkish, nonpigmented islands were present in the lesion. Case 6. (M. K.) A white man, aged 47 years, had a routine eye examination after bumping his head. A dark, sharply demar­ cated lesion of three disc diameters was found above in the right fundus. It con­ tained a nonpigmented area along the in-



Fig. 5 (Reese and Jones). Case 5 (W. K.). ferior border. No scotoma was found. Vi­ sion was normal. Case 7. (D. C.) An adult female was found to have a two and one-half disc di­ ameter, round, granular, dark area in the right eye along the inferior temporal vessels. It was not elevated, and the retinal vessels crossed it undisturbed. The patient sav^ many ophthalmologists whose opinions and interpretations varied. Because the possif bility of a malignant melanoma still existed; the patient's apprehension mounted to the point where she requested an enucleation. PATHOLOGY

Fig. 6 (Reese and Jones). Case 6 (M. K.).

Fortuitously, we have been furnished a drawing and a hematoxylin and eosin stained section from the patient described as Case 7. The section shows the pigmenta­ tion to be due essentially to excessive pig­ ment epithelium. In the choroid under the site of the reduplicated epithelium, the melanoblasts are also more pigmented. They



Fig. 7 (Reese and Jones). Case 7 (D. C ) .

do not appear to be abnormal or excessive in number. The increased pigment content of these stromal melanoblasts does not seem to be from phagocytosis of pigment from the epithelium. There is no evidence at all of present or past inflammation or its se­ quelae. Our feeling is that this represents a localized, congenital overgrowth of pigment epithelium.

from Case 7 (D. C ) .

The isolated, sharply outlined black pig­ ment area in the fundus described in this paper sometimes presents itself for differ­ entiation from a malignant melanoma of the choroid. The black color, sharp demarcation, lack of elevation, and absence of a corre­ sponding scotoma all bespeak against a mela­ noma of the choroid. We believe that serious consideration should be given the possibility that such pigment is a proliferative reaction from previous choroiditis. Our opinion is that such is not the case. Postinflammatory proliferation is not uniform but tends to be around the periphery or mottled. It shows progression following the active stage and most important is that in our cases there were no other foci in the fundus of the same or fellow eye. Furdiermore, the section of the enucleated eye revealed no evidence or sequelae of inflammation. It is true that postinflammatory proliferation of the pig­ ment epithelium can produce a fundus lesion which simulates closely the one which we are describing and no doubt could produce one rarely that might be indistinguishable. This would be possible when the noxious factor in the choroid was short of being lethal to the overlying epithelium, and, therefore, only stimulating, thus provoking the proliferation of a sheath of pigment epithelium. Under the usual conditions the noxious agent is lethal to the overlying epithelium and stimulating to the epithelium around the periphery of the inflammatory focus thus producing the usual postinflam­ matory fundus picture of a light center sur­ rounded by a collar of pigment. Depending on the degree of toxicity, we may see all degrees of pigmentation from this usual pic­ ture to that of a lesion resembling the one we described here. Whether or not this isolated congenital pigment area is related to the retinal pig­ ment spots described as "bear tracks" or congenital group pigmentation of the retina is not clear. If so, we would expect some



cases to show transitions. In none of our cases did we see any accompanying lesions similar to "bear tracks." However, in Hoeg's extensive description of congenital grouped pigmentation of the retina, he described soli­ tary lesions, in addition to the grouped spots, which answer the description of ours. Pigment areas which appear to be similar to the ones we discussed have been described under various names and with various in­ terpretations by Jaeger, Stephenson, Batten and Spicer, Dodd, Roll, Deutschmann, Segalowitz, and Crespi. These are the re­ ports of isolated cases and in no instance has there been a concerted effort to assign the

lesion a definite interpretation. We feel that the lesion is more common, definite, and consistent than the literature indicates and deserves the recognition of being an entity. CONCLUSION

There is a type of benign melanoma of the pigment epithelium which is sometimes confused with a malignant melanoma of the choroid. The lesion is congenital and so far as we know it never becomes malignant. 73 East 71st Street (21). We wish to express our appreciation to Dr. W. M. Boles and Dr. Stirling S. McNair for permission to use one case.


Batten, R. D., and Spicer, W. T. H.: Congenital pigmentary plaques of retina. Tr. Ophth. Soc. U. Kingdom, 14:127-129,1894. Crespi Jaume, G.: Melanosis ocular congenita. Arch. Soc. oftal. Hisp.-Amer., 8:437-447, 1948. Deutschmann, F.: Ueber ein atypisches Netzhautgliom. Ztschr. f. Augenh., 27:22S-235, 1912. Dodd, H. W.: Congenital pigmentation of retina. Tr. Ophth. Soc. U. Kingdom, 15:194, 1895. Hoeg, N.: Die gruppierte Pigmentation des Augengrundes. Klin. Monatsbl. f. Augenh., 49:49-77, 1911. Jaeger, E.: Die gruppierte Pigmentierung der Netzhaut. Kurzes Handb. d. Ophthal, Vol. S, Berlin, Springer, 1930, Chap. O, p. 588. Roll, G. W.: Congenital pigmentation patch in the retina. Tr. Ophth. Soc. U. Kingdom, 22 :193, 1902. Segalowitz: Pigmentnavus der Chorioridea. Ophth. Klin., 10:225, 1906. Stephenson, S.: A peculiar form of retinal pigmentation. Tr. Ophth. Soc. U. Kingdom, 11:77-82, 1890-91.




Yonagi City, Japan Contrary to our expectation only a few cases of atom-bomb catract were experienced until 1952. There are, however, many pa­ tients suffering from radiation cataract among atom-bomb survivors to date. The cataract itself is so mild that the patients do not complain of any visual disturbance, even if they have characteristic yellow-golden glistening, round or oval or doughnutlike opacities at the posterior pole region of the lens. The atomic raid occurred in August, We should like to express our heartiest thanks to Ronald Atmore Cox, M.D., Professor of Ophthal­ mology, George Washington University, and Sidney John Glueck, M.D., Department of Ophthalmology, District of Columbia General Hospital, who as­ sisted in preparation of this paper.

1945, more than 10 years ago. We should like to present the statistical observations of Dr. Yoshiya Masuda, head of the Depart­ ment of Ophthalmology, Red Cross Hos­ pital, Hiroshima, Japan. STATISTICAL OBSERVATIONS

1. Incidence. This consists of 435 patients examined from June 1, 1953, to October 31, 1954. Among them, 354 came directly to the eye clinic for examination and 81 were trans­ ferred from the other clinics, such as derma­ tology and surgery clinics. Of these, 116 pa­ tients (26.6 percent) were suffering from atom-bomb cataract, and 37 other cases were possibly of the same etiology.