Malignant Melanoma of the Choroid*

Malignant Melanoma of the Choroid*

MALIGNANT TREATED WITH MELANOMA BONIUK, M.D., RADON AND Houston, The following case is reported because of some unusual pathologic complication...

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MALIGNANT TREATED

WITH

MELANOMA

BONIUK,

M.D.,

RADON AND

Houston,

The following case is reported because of some unusual pathologic complications that were observed following treatment of a choroidal melanoma with photocoagulation, transscleral diathermy, and implanted radon seeds. CASE

CHOROID*

PHOTOCOAGULATION, TRANSSCLERAL IMPLANTED

MILTON

OF THE

REPORT

E. P. R., a white man, 64 years of age, was first seen in consultation on November 25, 1960, through the courtesy of Dr. Maxwell Thomas of Dallas and Dr. Robert E. Nystrom of Abilene, Texas. There was a large, lightly pigmented tumor located below the nervehead and an incipient corti­ cal cataract in the left eye. The vision in this eye was 20/70. The vision in the right eye was light perception and faulty projection. He had had a childhood injury resulting in an adherent leukoma, a membranous cataract and secondary glaucoma. A capsulectomy and iridocorneosclerectomy were performed in the right eye but shortly following this operation light perception was lost. Ρ"" studies in the left eye were strongly positive. Forty-eight hours after injection, there was a 243% increase of radioactive phosphorus uptake over the site of the tumor, in comparison with readings in the superior quadrant. The first photocoagulation was performed Decem­ ber 1, 1960. Because of surrounding retinal detach­ ment and the proximity of the nervehead, the base of the tumor could not be photocoagulated. The entire elevated portion was photocoagulated, using settings on the Zeiss photocoagulator of normal load 3 and overload 1. The retina overlying the tumor became completely white following the coagulations. Postoperatively, the eye was moderately inflamed. There was corneal edema centrally with some flare and cells in the anterior chamber. The reaction and corneal edema subsided within a month, but some atrophy of the iris was noted. Subsequent photo­ coagulations were performed on January 5 and February 16, 1961. On both occasions it was noted that no visible photocoagulation reaction could be obtained. On March 16, 1961, the cornea showed some edema and bullae formation, the iris showed marked atrophy, but there was no change in the size of the tumor. On that day, after a personal communication with Prof. Gerde Meyer-Schwickerath, the tumor * From the Department of Ophthalmology, Baylor University College of Medicine.

DIATHERMY

AND

SEEDS

LOUIS

J.

GIRARD,

M.D.

Texas base was outlined with indirect ophthalmoscopy and the entire sclera over the base was treated with nonpenetrating electrodiathermy. One month later the retina surrounding the tumor was flat, but the tumor had extended somewhat over the optic nerve. Additional photocoagulations were performed on April 20, June 8 and August 17, 1961, without visible evidence of change in size of the tumor. After a personal communication with Prof. H. B. Stallard, on November 16, 1961, a plastic shell containing five radon seeds of 1.0-mc strength was sutured to the sclera overlying the tumor, deliver­ ing a calculated dose of 3,500 r to the summit of the tumor at a distance of 6.0 mm.t This was left in place for three days and then removed. Preoperatively the vision was 20/400 but, postopera­ tively, the reaction was moderately severe and the patient lost all light perception in this eye. S i x weeks later the postoperative reaction had subsided, the cornea was still edematous mferiorly, the anterior chamber was shallow, the iris was atrophic and the lens was markedly cataractous. The tumor, however, could still be seen by indirect ophthal­ moscopy. There was no change in the size of the tumor, and the entire retina was detached. Enuclea­ tion was advised and performed on January 20, 1962. HISTOPATHOLOGIC

FINDINGS

The enucleated specimen was firm and measured 32 by 24.5 by 24.5 mm. with 6.0 mm of optic nerve attached. The corneal epithelium was partly abraded; the extra­ ocular muscles were thickened; and a large amount of fat and connective tissue were attached to the posterior surface of the globe. No tumor was visible grossly. Transillumi­ nation revealed a large defect posteriorly. The eye was opened in a vertical plane. The anterior chamber was shallow, and posterior synechiae were present. The iris was atrophic and the pupil was dilated. The retina was t The shell was designed from a mold of a human eye by Joseph W . Soper and the radiation dosage and radioactive material were supplied by the Department of Radiology, Baylor University College of Medicine. The isodose distribution in this case is illustrated in a recent article by Fingerhut and Collins* (fig. 3 ) . 212

MALIGNANT MELANOMA

OF THE

CHOROID

213

rounding the base of the tumor. Microscopic examination revealed early peripheral anterior synechiae on both sides. The trabecular fibers were thickened and infiltrated with melanin-laden macrophages. The iris stroma was severely atrophic and there was extensive postnecrotic scarring of the pupillary and midzonal portions (fig. 2 ) of the iris. Posterior synchiae were present on both sides. The lens was in place, but there was severe degeneration of the entire lens epithelium. The lens was swollen and there were early degenerative changes in the anterior, posterior and equatorial cortex.

Fig. 1 (Boniuk and Girard). ( A ) Mushroomshaped malignant melanoma with retinal break (arrow) in adjacent detached retina. (B and C ) Outer aspect of horseshoe-shaped retinal break (arrows).

almost totally detached and there were mul­ tiple retinal folds inferiorly. A horseshoeshaped retinal tear with operculum was pre­ sent nasally (fig. 1 ) . A band-shaped retinal membrane was located in the vicinity of the retinal tear. A brownish-white tumor mass measuring 10 by 10 by 8.0 mm occupied the choroid inferiorly and extended to cover much of the optic nervehead. Several areas of chorioretinal scarring were noted sur-

Arising from the choroid posteriorly near the optic disc was a large mushroom-shaped neoplasm (fig. 3 ) composed mainly of oval and spindle-shaped cells with oval nucleolated nuclei. The cells were arranged in whorls and fascicles and the neoplasm was lightly pigmented. Many engorged blood vessels were located in the summit of the neoplasm and there was severe degeneration of the retina overlying the tumor, but no necrosis of the summit or base of the neo­ plasm. The surrounding retina was detached and there was moderate degeneration of the outer retinal layers. The superior retina was thrown into folds. There were several firm vitreoretinal adhesions and some folding and thickening of the internal limiting membrane. In one area a band of proliferated pigment epithelium and glial tissue was adherent to the outer layers of the retina. The anterior portion of the optic nerve was severely atro­ phic and replaced by dense scar tissue (fig. 4 ) . The sclera was thickened posterior­ ly and a mass of fibro-fatty tissue and skeletal muscle was adherent to the outer surface of the sclera. In some sections there were focal areas of infiltration by foreignbody giant cells that surrounded small birefringent particles. There was mild chronic cellular infiltration within the fibro-fatty connective tissue. Several large arteries in the orbital tissue revealed severe subintimal hyperplasia with thickening and calcification of the internal elastic lamina (fig. 5 ) .

214

MILTON BONIUK AND LOUIS J . G I R A R D

Kig. 2 (Boniuk and Girard). (A and B) Severe postnecrotic scarring of the midzonal and pupillary portions of the iris on both sides. (Hematoxylin-eosin, χ 3 8 . )

The pathologic diagnoses were: malignant melanoma of the choroid, subtype spindle B, treated with photocoagulation, transscleral diathermy, and radon seed; retinal detach­ ment ; complicating cataract; severe post-

Fig. 3 (Boniuk and Girard). The mushroomshaped malignant melanoma shows no evidence of necrosis in either the superficial or the deeper portion of the neoplasm. (Hematoxylin-eosin, re­ duced from X l l . )

Fig. 4 (Boniuk and Girard). Dense scar tissue (area bounded by arrows) has replaced the anterior part of the optic nerve. (Hematoxylin-eosin, re­ duced from χ9.ϊ.)

MALIGNANT MELANOMA

OF THE

CHOROID

215

Fig. 5 (Boniuk and Girard). ( A and B) Large arteries in orbital tissue showing marked subintimal hyperplasia and calcification (arrows) of the internal elastic lamina. (Hematoxylin-eosin, ( A ) reduced from X80, ( B ) reduced from X215.)

necrotic scarring of the iris; optic atrophy secondary to radiation vasculitis. DISCUSSION

and vitreous changes that were secondary to photocoagulation therapy. The association of choroidal melanomas and retinal breaks, re­ ported previously, was merely coincidental and differs from the present case wherein the retinal break presumably developed following photocoagulation therapy. 3. The necrosis involving the pupillary and midzonal portions of the iris was also apparently related to photocoagulation treat­ ment. The iris necrosis may have been due to scattering of light rays at the time of treatment. However, it is also possible that cataractous changes initially present became more advanced as a result of photocoagula­ tion and, with increasing lens changes, light from the photocoagulator was partially ab­ sorbed by the lens. This light may have been converted to heat and some of it may have been dissipated into the adjacent pupillary and midzonal portions of the iris. 1

A number of conservative techniques have been used in recent years in the treatment of small choroidal melanomas in one-eyed in­ dividuals. Several of these techniques were used in treatment of the case under consid­ eration, and some of the pathologic findings in the eye were probably related to treatment with photocoagulation, transscleral diathermy and radon seeds. 1. Pathologic examination of the choroidal melanoma itself revealed no changes that might be related to the various treatments employed. Although the neoplasm was lightly pigmented, one would have expected to see necrosis of the superficial portion of the tumor as a result of the multiple photoco­ agulation treatments. However, the tumor was considerably elevated, and others have noted difficulty in destroying highly elevated tumors with photocoagulation. The absence of changes in the deeper portion of the tumor, in spite of transscleral diathermy and radon-seed implantation, is also difficult to explain. 3

2. The horseshoe-shaped retinal tear that was present nasally was not seen clinically and may have developed just prior to enu­ cleation. This retinal break was apparently iatrogenic and probably resulted from retinal

4. The optic nerve changes were extremely interesting. At the time of radon-seed im­ plantation visual acuity was 20/400. S i x weeks later at the time of enucleation the eye was completely blind. Pathologic examina­ tion revealed an area of postnecrotic scarring involving the anterior portion of the optic nerve. This part of the optic nerve is supplied by the arterial circle of Zinn-Haller with branches derived from the short posterior ciliary arteries. Presumably, the postradiation fibrosis and vasculitis were responsible

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MILTON BONIUK A N D L O U I S J . G I R A R D

for the optic-nerve changes. Evidence of radiation vasculitis was found in the orbital tissue overlying the tumor and this finding lends weight to the hypothesis of postradiation damage. SUMMARY

A malignant melanoma of the choroid was unsuccessfully treated with photocoagulation, transscleral diathermy and radon seeds. Path­ ologic examination revealed little or no effect

of treatment on the malignant melanoma. Photocoagulation was presumably respon­ sible for an iatrogenic retinal break, pro­ gressive cataractous changes and necrosis of the pupillary and midzonal portions of the iris. Radiation fibrosis and vasculitis were apparently responsible for the postnecrotic scarring involving the anterior portion of the optic nerve. Baylor University College of Medicine.

REFERENCES

1. Boniuk, M , and Zimmerman, L. E.: Problems in differentiating idiopathic serous detachments from solid retinal detachments. Int. Ophth. Clin, 2:411-430, 1962. 2. Fingerhut, A. G , and Collins, V. P.: Local treatment of retinal tumors with radon. Radiology, 81:1003-07, 1963. 3. Curtin, V. T , and Norton, W . D.: Pathological changes in malignant melanomas after photocoagu­ lation. Arch. Ophth, 70:150-157, 1963.

STATISTICAL OBSERVATIONS

OF

SURVIVORS OF THE CLINICAL GEORGE

AND J.

STATISTICAL INADA,*

OBSERVATIONS

M.P.H.

New INTRODUCTION

An atomic bomb was dropped on Hiro­ shima, Japan, on August 6, 1945, exposing many persons living in the city to manmade ionizing radiation. Among the possible effects of radiation exposure to man was to cause radiation cataracts. Studies made of survivors of the atomic bomb by various investigators: Flick, Cogan, et a l , Kimura, Fillmore, Sinskey, ' Hi rose, and Kandori have shown that heavily exposed survivors of either the Hiroshima or Nagasaki A-bombs have radi­ ation cataracts. Work done by Sinskey has indicated some doubt that the effect of ioniz­ ing radiation was to produce a radiation 1

2 , 3

8

7

8

4

LENTICULAR

HIROSHIMA

5

9

7

AND

CARL

CHANGES

OF

DELAYED

W.

HALL,

T

EFFECTS M.D.

York

cataract of a specific nature which could differentially be diagnosed on the basis of its characteristics and the history of the pa­ tient. This raised the question: Could one of the effects of exposures to ionizing radi­ ation be to increase the incidence of certain types of lenticular opacities? Several studies have been reported relat­ ing the percentage of cases of examined patients showing cataract to the exposure distance or a combination of distance and other parameters. Dr. Hirose, at the Na­ gasaki Medical School, reported in 1953 that 182 cases of 436 survivors (41.8 percent) showed cataract. Dr. Sinskey showed that of 165 survivors who experienced epilation, 135 cases (81.8 percent) had demonstrable cata­ ractous changes. Dr. Kandori in 1956 re­ ported that: ( 1 ) in 159 survivors, exposed > two kilometers from the hypocenter, 87 cases (54.7 percent) showed cataractous changes, and of 277 survivors < two kilo8

7

9

* Formerly, chief of operations, Biostatistics De­ partment, A B C C ; presently, statistical advisor, USAID. t Formerly, ophthalmologist A B C C ; currently, president A F Physical Evaluation Board, U S A F Hospital.

IX

A-BOMB