N O T E S , CASES, I N S T R U M E N T S 629

8. Graham, T. N. : Idiopathic multiple hemorrhagic sarcoma (Kaposi) . Arch. Ophth., 27:1188-1192, 1942.

9. Duke-Elder, W. S.: Textbook of Ophthalmology. St. Louis, Mosby, 1941, v. 2, pp. 1740-1742. 10. Kaposi, M.: Arch. Dermat. & Syph., 4:265, 1872. 11. Pepler, W. J.: The origin of Kaposi's hemangiosarcoma : A histochemical study. J. Path. Bact.,

78:553-557, 1959.

M A L I G N A N T M E L A N O M A O F T H E I R I S

M O D E O F E X T E N S I O N A N D D I S S E M I N A T I O N

S. L A W R E N C E S A M U E L S , M . D .

Plainfield, New Jersey A N D

B R I T T A I N F . P A Y N E , M . D .

New York

Malignant melanoma is the most common intraocular tumor. It may involve any part of the uveal tract. The choroid is by far the most frequently involved, the iris being af­fected rather rarely in comparison. The cho-roidal tumors are considered most malig­nant, the iris growth least malignant.

The iris tumor usually is present in the stroma and anterior border layer but may easily break through the surface or extend out from the crypts and grow on the sur­face. If the growth extends into the angle, the corneal-scleral trabeculae and Schlemm's canal are involved. The trabecular spaces may be blocked and, if a sufficient area is obstructed, secondary glaucoma may ensue and may be the immediate cause of the pa­tient seeking ophthalmologic advice. If the diagnosis is missed, the patient may be treated for glaucoma with medication and surgery before the underlying cause is rec­ognized. Sometimes, too, these patients are not seen until intractable absolute glaucoma necessitates enucleation, and the diagnosis is made in the pathology laboratory.

This report is of two cases of melanoma of the iris which had already spread to the angle. They illustrate the importance of finding the cause of unilateral elevated ocu­lar tension. In one case the diagnosis was made preoperatively by slitlamp and gonios-copy; in the other, the patient was seen at the time of enucleation when the glaucoma

Fig. 1 (Samuels and Payne) . Case 1. Malignant melanoma of the iris. (C) Cornea. (S) Scierai spur. ( T ) Tumor.

was absolute and the patient was in great pain. He had been under medical care for some time elsewhere.

CASE REPORTS

CASE 1

A woman, aged 55 years, first noted blurring of vision, R.E., three weeks ago. Vision was 20/50; tension ( Schlitz ) 50 mm. H g ; anterior segment clear except at the angle where a pigmented mass was seen. The globe was normal in size. The an­terior chamber was well formed. A tumor of the temporal iris had invaded the angle. There was mild glaucomatous cupping. On the temporal side the cor­neal-scleral trabeculae, the root of the iris and the peripheral portion of the anterior surface of the iris were infiltrated by spindle-type cells. The tumor was mildly pigmented and pigment was also seen in the corneal-scleral trabeculae of the op­posite angle. Schlemm's canal was obscured by tumor cells and some cells were in the confines of the canal itself. There was no apparent extension beyond the canal.

CASE 2

A man, aged 78 years, said that hi« right eye "went bad" about seven months ago. He was seen by an ophthalmologist and drops were prescribed. When seen two months later the eye was stony hard but not painful. The eye transilluminated well in all directions. He refused operation but returned

630 N O T E S , CASES,

Fig. 2 (Samuels and Payne). Case 1. Enlargement of Figure 1.

five months later because the pain was unbearable and there was no vision.

The eye was normal in size, with a shallow an­terior chamber. The pupil was eccentric. Thicken­ing of the nasal iris and marked atrophy of the temporal iris were present. There was deep pig­mentation of the nasal iris and ciliary body and blockage of the angle. There was glaucomatous excavation with a fine layer of cells in the cup and over the retina for a short distance on either side.

The nasal iris was thickened and filled with cells of malignant melanoma of the mixed type with an outgrowth completely covering the surface and extending into the angle. The corneal-scleral trabec-ulae were invaded and Schlemm's canal was filled with spindle cells which extended into the aqueous veins leading from the canal. There was extension of the spindle cells into the emisseria of the ante­rior ciliary vessels.

The ciliary body on the nasal side was almost

Fig. 3 (Samuels and Payne). Case 2. Malignant melanoma of the iris. (C) Cornea. (D) Descemet's membrane. (L) Lens. ( T ) Tumor.

I N S T R U M E N T S

Fig. 4 (Samuels and Payne) . Case 2. Enlarge­ment of Figure 3, showing arrangement of tumor cells.

completely infiltrated by tumor cells and broken-down pigment. The processes were only slightly involved but the valleys between were filled with cells typical of malignant melanoma which ex­tended over the ciliary processes and along the zonules. On the nasal side the tumor extended for a short distance into the choroid and then stopped abruptly.

There was atrophy of the inner layers of the retina and marked glaucomatous cupping. The ex­cavation was filled with spindle-B cells in an or­derly ribbonlike manner. The cells extended onto the surface of the retina on either side, gradually

Fig. 5 (Samuels and Payne) . Case 2. Implanta­tion growth in the papilla and on the surface of the retina.

NOTES, CASES, INSTRUMENTS 631

Fig. 6 (Samuels and Payne). Case 2. Enlarge­ment of Figure S, showing arrangement of tumor cells.

thinning out about five mm. from the edges of the glaucomatous cup. The new-formed membrane in the papillary excavation and over the surface of the retina apparently was subvitreal.

C O M M E N T

What makes Case 2 of particular interest is that there was an implantation growth on the surface of the papilla and the inner sur­face of the adjacent retina and no demon­strable tumor of the choroid.

There was a small amount of choroidal tissue in the lamina cribrosa and the tumor could have arisen from this source. How­ever, none of the sections studied showed any such origin and we assume that the

layer of tumor cells on the papilla and ret­ina was a distant implantation growth from the primary tumor of the anterior uvea. W e can conjecture that some tumor cells broke away and dropped into the postlental space and through Cloquet's canal to the papilla where they took root and spread.

Multiple origins of retinoblastoma are well known and seedings are characteristic of this tumor. However, seedings are very rare in malignant uveal melanoma. No simi­lar implantation growth has been previously reported to our knowledge, although Reese (fig. 120, p. 242) has seen one instance of an implantation growth on the iris from a malignant melanoma of the choroid.

S U M M A R Y

Two cases of malignant melanoma of the iris are presented to illustrate the mode of extension of these tumors. In both cases, the tumor invaded the ciliary body and ex­tended into the filtration angle, involving the corneal-scleral trabecular spaces and Schlemm's canal, the anterior ciliary veins and the posterior cornea. Failing vision due to glaucoma was a prominent feature in both cases. The correct diagnosis was made pre-operatively in one case. In the other case, the markedly diffuse malignant melanoma of the iris and ciliary body, already show­ing surface growth and seeding and implan­tation on the papilla and adjacent retina, was diagnosed at enucleation.

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REFERENCES

Anderson, B., and O'Neill, J.: Malignant melanoma of the uvea. AMA Arch. Ophth., 58:337-347 (Sept.) 1957.

Cleasby, G. W.: Malignant melanoma of the iris. AMA Arch. Ophth. 60:403-417 (Sept.) 1958. Duke, J. R., and Dunn, S. N.: Primary tumors of the iris. AMA Arch. Ophth., 59:204-214 (Feb.)

1958. Dunphy, E. A., et al.: Symposium: The diagnosis and management of intraocular melanomas. Tr. Am.

Acad. Ophth., 6:2:517-555 (July-Aug.) 1958. Makley, T. A., Jr., and Teed, R. W. : Unsuspected intraocular malignant melanomas. AMA Arch.

Ophth., 60:475-478 (Sept.) 1958. Reese, A. B.: Tumors of the Eye. New York, Hoeber, 1951. Rones, B., and Zimmerman, L. E. : The prognosis of primary tumors of the iris. AMA Arch. Ophth.,

60:193-205 (Aug.) 1958. Scheie, H. G.: Gonioscopy in the diagnosis of tumors of the iris. Tr. Am. Ophth. Soc., 51:313-331,

1953.