The Management of Choroidal Melanomas

AMERICAN JOURNAL OF OPHTHALMOLOGY™ FRANK

W. NEWELL, Editor-in-Chief

Tribune Tower, Suite 1415, 435 North Michigan Ave., Chicago, Illinois 60611

EDITORIAL BOARD Mathea R. Allansmith, Boston Douglas R. Anderson, Miami Crowell Beard, San Jose Bernard Becker, St. Louis Benjamin F. Boyd, Panama Charles J. Campbell, New York Ronald E. Carr, New York Thomas Chalkley, Chicago Claes H. Dohlman, Boston Fred Ederer, Bethesda

DuPont Guerry III, Richmond Robert W. Hollenhorst, Rochester Herbert E. Kaufman, New Orleans Arthur H. Keeney, Louisville Carl Kupfer, Bethesda Irving H. Leopold, Irvine A. Edward Maumenee, Baltimore Irene H. Maumenee, Baltimore Edward W. D. Norton, Miami G. Richard O'Connor, San Francisco

Arnall Patz, Baltimore Steven M. Podos, New York Albert M. Potts, Louisville Algernon B. Reese, New York Robert D. Reinecke, Albany Marvin L. Sears, New Haven David Shoch, Chicago Bruce E. Spivey, San Francisco Bradley R. Straatsma, Los Angeles Gunter K. von Noorden, Houston

Published monthly by the Ophthalmic Publishing Company Tribune Tower, Suite 1415, 435 North Michigan Avenue, Chicago, Illinois 60611 Directors: A. EDWARD MAUMENEE, President; DAVID SHOCH, Vice President; FRANK W. NEWELL, Secretary and Treasurer; EDWARD W. D. NORTON, BRUCE E. SPIVEY, BRADLEY R. STRAATSMA

THE MANAGEMENT OF CHOROIDAL MELANOMAS The management of malignant melanoma of the choroid has recently become a topic of considerable controversy. Several years ago most ophthalmologists believed that enucleation was warranted without delay for any eye containing a mass suspected of being a choroidal melanoma.' A number of more recent observations have led to a reassessment of the advisability of enucleation as a treatment for choroidal melanomas. One observation that prompted reassessment of early enucleation was that many eyes removed because of a suspected choroidal melanoma were shown on subsequent pathologic studies to contain a lesion that simulated a melonoma (pseudomelanoma) rather than a true melanoma.F' Another development that has led to reassessment of early enucleation was a recent reappraisal of Callender's classification of choroidal melanomas in which many small spindle-cell melanocytic tumors were downgraded from a malignant category to a benign

one." As a result, an ophthalmologist who several years ago enucleated an eye for a small choroidal melanoma diagnosed histopathologically as malignant may now be advised retrospectively that he actually removed a useful eye containing a benign tumor. A third development that has brought about reassessment of the advisability of enucleation for small melanomas is the finding on follow-up studies of untreated small melanocytic choroidal tumors that most lesions of this type are biologically dormant, have little tendency to grow, and virtually no tendency to metastasize.S" A fourth observation that has prompted reassessment of early enucleation is that removal of an eye containing a malignant melanoma may not improve the patient's prognosis for life and may actually hasten his demise from disseminated metastases. 7,8 A recent article by Manschot and van Peperzeel" disregarded the observations set forth above and asserted the advisability of early enucleation of any eye containing a presumed choroidal melano-

266

VOL. 90, NO. 2

EDITORIAL

267

rna. Their article has further clouded the enucleation, rarely if ever, develop mechoroidal melanoma management contro- tastases even when no treatment is used. versy and requires critical comment. If enucleation were not performed, these Manschot and van Peperzeel used data patients would continue to lead healthy that are largely theoretical, based on lives as binocular rather than as monocustudies of highly malignant nonocular lar individuals. Our clinical observations tumors, to draw conclusions regarding suggest that one should not assume that a the clinical behavior of small choroidal pigmented lesion will grow or produce melanomas. They assumed that all mela- metastasis just because it is there. Most nocytic choroidal lesions greater than 5 of these lesions probably develop or mm in diameter are steadily growing acquire pigmentation at puberty and tumors which, if not treated, will lead most remain permanently stable. eventually to metastasis and death. Our On the basis of their theoretical matericlinical data suggest that this assumption al Manschot and van Peperzeel concluded is incorrect. that the only acceptable method of Between 1974 and 1979 almost 500 management is "early enucleation of all patients with a uveal melanoma were choroidal melanomas, but especially of examined and managed by the Oncology small melanomas, in order to prevent the Service of Wills Eye Hospital. Approxi- initial or later dissemination of tumor mately 90% of the 175 patients with a cells" and that "all other treatments, such small melanoma (between 5 x 5 x 2 mm as photocoagulation, radiation and exciand 10 x 10 x 3 mm) and about 30% of sion of choroidal melanoma, and certainly the 125 patients with a medium-sized also the attitude of observation, will be melanoma (between 10 x 10 x 3 mm and harmful to the patient because dissemina15 x 15 x 5 mrn) have been followed up tion of tumor cells is promoted, either with serial fundus photographs and other actively or passively." Our clinical data ancillary studies without enucleation or also suggest that this assumption is incorother forms of therapy. Preliminary data rect. from these patients suggest that only 10 In recent years we have routinely used to 15% of small melanomas and approxi- methods other than enucleation to manmately 50% of medium-sized melanomas age almost all patients with small and can be documented to grow. The remain- medium-sized choroidal melanomas that ing patients with small or medium-sized show evidence of growth." Since 1974, melanomas appear to have biologically we have managed 35 patients with photodormant lesions that grow slowly if at all coagulation of their melanoma. There and pose no serious threat to the patient's have been no tumor-related deaths in this general health. group and none of these patients currentThese observations suggest that early ly have evidence of metastatic disease. enucleation of eyes containing a small Since 1975, 90 patients have been manmelanocytic choroidal tumor, as advocat- aged with episcleral cobalt plaque radioed by Manschot and van Peperzeel, will therapy. Two of these patients have died result in unnecessary enucleation in at of disseminated metastasis; one of them least 80% of cases. This seems irrational had clinical and laboratory evidence of for patients who often have excellent hepatic metastasis before placement of visual acuity in the involved eye. Our the plaque, and both of them had a large preliminary data also suggest that pa- melanoma (greater than 15 x 15 x 5 mm) tients with small melanomas, for whom at the time of treatment. Since 1974, ten Manschot and van Peperzeel recommend patients have been treated with eye wall

268

AMERICAN JOURNAL OF OPHTHALMOLOGY

AUGUST, 1980

resection for choroidal melanoma. None 3. Shields,]. A., and Zimmerman, L. E.: Lesions of them have developed metastasis de- simulating malignant melanoma of the posterior uvea. Arch. Ophthalmol. 89:466, 1973. spite a significant number of epithelioid 4. McLean, I. W., Zimmerman, L. E., and cells having been demonstrated patholog- Evans, R. M.: Reappraisal of Callender's spindle A type of malignant melanoma of the choroid and ically in some of the resected tumors. ciliary body. Am. J. Ophthalmol. 86:557, 1978. To date, there have been no tumor5. Cass, J. D. M.: Problems in the diagnosis of related deaths in our series of patients choroidal nevi and malignant melanomas. Am. J. with small and medium-sized melanocy- Ophthalmol. 83:299, 1977. 6. Shields, J. A.: Changing trends in the managetic choroidal tumors observed without ment of choroidal melanomas. Trans. Pac. Coast treatment or treated with methods other Oto-Ophthalmol, Soc. 60:215, 1979. 7. Zimmerman, L. E., McLean, I. W., and Fosthan enucleation. This compares favorater, W. D.: Does enucleation of the eye containing a bly with reported mortality among simi- malignant melanoma prevent or accelerate the dislar patients managed by enucleation. 10 semination of tumor cells? Br. J. Ophthlamol. 62:420, Data from the Oncology Research Labo- 1978. 8. Zimmerman, L. E., and McLean, I. W.: An ratory at Wills Eye Hospital suggest that evaluation of enucleation in the management of uveal the immunologic antimelanoma defense melanomas. Am. ]. Ophthalmol. 87:741, 1979. 9. Manschot, W. A., and van Peperzeel, H. A.: mechanisms in patients with a choroidal Choroidal melanoma. Enucleation or observation? A melanoma may be enhanced by treat- new approach. Arch. Ophthalmol. 98:71, 1980. ment such as photocoagulation or irradia10. McLean, I. W., Foster, W. D., Zimmerman, tion but not by enucleation. 12 We current- L. E.: Prognostic factors in small malignant melanomas of the choroid and ciliary body. Arch. Ophthally believe and recommend that most mol. 95:45, 1977. patients with small choroidal melanomas II. Shields, J. A.: Current approaches to the should be managed by observation and diagnosis and management of choroidal melanomas. Surv, Ohthalmol. 21:443, 1977. serial photographs only and that most 12. Federman, J., Felherg, N. T., and Shields, patients with medium-sized choroidal ]. A.: The effect oflocal treatment on antibody levels melanomas should be managed by obser- in malignant melanoma of the choroid. Trans. Ophthalmol. Soc. U.K. 97:436, 1978. vation or methods other than enucleation, particularly if the patient is older, or if the involved eye is the only eye that has useful vision. OBITUARY Our duty as ophthalmic clinicians is also to salvage vision, particularly if this PETER CLEMONS KRONFELD can be achieved without endangering the 1899-1980 patients' systemic health. The value of Peter C. Kronfeld, professor emeritus conservative treatment rather than early of ophthalmology at the University of enucleation in such cases is supported by Illinois Medical School, died January 1, our preliminary clinical data. 1980, at the age of 80. It was characterisJERRY A. SHIELDS tic that his quiet reserved manner, couAND pled with his enthusiasm and productiviJAMESJ. AUGSBURGER ty, concealed the seriousness of his longstanding illness from most of his countREFERENCES less friends and admirers. 1. Dunphy, E. B., Forest, A. W.. and Leopold, It is difficult to put into perspective I. H: The diagnosis and management of intraocular melanomas. Trans. Am. Acad. Ophthalmol. Otolarthose characteristics that most distinyngol. 62:517, 1958. guished this remarkably talented person. 2. Ferry, A. P.: Lesions mistaken for malignant Born in Vienna, Austria, he retained his melanoma of the posterior uvea. Arch. Ophthalmol. 72:463, 1964. continental charm and mannerisms, as