neoplasms of the iris pigment epithelium

VOL. 66, NO. 1 T R A N S S C L E R A L L A S E R R A D I A T I O N 31

by a second method, using ruby and neo-dymium lasers as the radiation source.

REFERENCES

1. Verhoeff, F. H., Bell, L. and Walker, C. B.: The pathological effects of radiant energy on the eye. Proc. Am. Acad. Arts and Sei 51:627,1916.

2. Luckiesh, M. : Infra-red radiant energy and the eye. Am. J. Physiol. Optics 2:3,1921.

3. Duke-Elder, W . S. : Text-book of Ophthalmol-ogy, S t Louis, 1940, Mosby, v. 1, p. 819.

4. Kutscher, C. F. : Ocular effects of radiant en-ergy. Tr. Am. Acad. Ophth. Otolaryng. 50:230,1946.

5. Kinsey, V. E. : Spectral transmission of die eye to ultraviolet radiations. Arch. Ophth. 39:508,1948.

6. Deutschmann, R.: Ueber die Blendung der Netzhaut durch directes Sonnenlicht Arch. f. Ophth. 28:241.1882.

y". Rathkey, A. S. : Accidental laser burn of the macula. Arch. Ophth. 74:346,196S.

8. Fine, B. S. and Geeraets, W . J. : Observations on early pathologic effects of photic injury to the rabbit retina. Acta Ophth. 43:684,1965.

9. Geeraets, W . J., Williams, R. C . Chan, G , Ham, W . T., Jr., Guerry, D , III and Schmidt, F. H. : The relative absorption of thermal energy in retina and choroid Invest Ophth l.:340, 1962.

10. Smith, R. S. and Stein, M . N. : Unpublished observations.

11. Dorsey, N.E. : Properties of ordinary water substance. New Yotjk, Reinhold, 1940, p. 341

12. Geeraets, W . J., Williams, R. C , Chan, G., Ham, W . T., Jr., Guerry, D., I l l and Schmidt, F. H. : The loss of light energy in retina and choroid. Arch. Ophth. 64:606,1960.

13. Zaret, M. M. : In Proceedings of the First Conference on Laser Safety, Orlando, Fla., May, 1966, p. 24.

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

DONALD A . MORRIS, M . D . AND PAUL HENKIND, M . D .

New York

Pigmented intraocular masses involving the anterior segment are diagnostic and ther-apeutic problems. We present two cases of pigment epithelial neoplasms of the iris; both were mistaken for malignant melano-mas of the ciliary body and treated by enu-cleation. The first case also had large excrescences of the pigment epithelium at the pupillary margin and drusen of the optic disc. The literature on iris pigment epithelial tumors is reviewed.

CASE 1

In January 1967, a 32-year old white me-chanic was found to have a pigmented mass of the anterior segment of the right eye. Past medical and family histories were non-contributory. Vision was 20/20 in both eyes and applanation tension was 1 1 mm Hg in

From the Department of Ophthalmology, New York University School of Medicine, and Manhattan Veterans Administration Hospital. This work was supported by U S P H S Grant NB-05059 of the Na-tional Institute of Neurological Diseases and Blind-ness, National Institutes of Health, Bethesda, Mary-land, and the Seeing Eye, Inc., Morristown, New Jersey.

the right eye and 1 6 mm Hg in the left. Both eyes contained floccules (excrescences of the pigment epithelium at the pupillary margin) of moderate size. The left eye was otherwise unremarkable. Slitlamp examination of the right eye revealed a large, nontransillu-minating, globular, dark-gray tumor which appeared to occupy the iris stroma from the 6 - to 9-o'clock positions from periphery to pupillary margin, and which projected into both the anterior and posterior cham-bers (fig. 1 ) . The lesion compressed the iris stroma against the corneal periphery and caused stromal atrophy but did not fully de-stroy the surface pattern. Large irregular blood vessels coursed over the iris surface above the mass. The pupil was straightened and rigid in the involved sector of the iris. • Through the dilated pupil could be seen a dark mass covered by furrowed pigment epi-thelium and indenting the lens, which showed mild anterior cortical opacification subjacent to the tumor; an incipient poste-rior axial subcapsular cataract was present. There were several flecks of pigment on the anterior surface of the lens. The aqueous

32 A M E R I C A N JOURNAL OF O P H T H A L M O L O G Y JULY, 1968

opaque, due to fixation. The sclera overlying the choroid transilluminated normally, but the pupil was irregular in the inferotemporal quadrant. On opening the globe, an 8-mm black mass was seen inferotemporally, in-volving the iris and possibly the anterior cil-iary body. The anterior surface of the lens was indented by the tumor (fig. 2 ) .

MICROSCOPIC EXAMINATION

Paraffin-embedded sections stained with hematoxylin and eosin, PAS, and Masson's

Fig. 1 (Morris and Henkind). Retouched clinical photograph of right eye showing pigmented mass distorting iris and occupying the inferotemporal portion of the pupil (arrows).

showed mild flare with occasional cells. Gon-ioscopy revealed a closed angle in the area of the neoplasm, but the angle opened widely at the tumor borders; small clumps of coarse, dark pigment were present in the angle in-feriorly. The ciliary body and zonules were visible at the inferior border of the tumor where the iris was lifted away from the lens. The fundus appeared normal but the area behind the tumor could not be visu-alized.

A general physical examination was uh-revealing. Laboratory studies, including CBC, urinalysis, ESR, liver function stud-ies, FBS, BUN, VDRL, EKG and a chest X-ray were within normal limits. An orbital series failed to reveal an opaque foreign body or osseous destructive lesion.

Several consultants, suspecting a mela-noma of the ciliary body, advised enucle-ation. The enucleated eye was placed in Zenker's solution, and later transferred to 10% formalin.

MACROSCOPIC EXAMINATION

The specimen was an intact right eye measuring 25 X 25.5 X 27 mm with 3 mm of attached optic nerve. The cornea was

Fig. 2 (Morris and Henkind). Photograph of gross specimen. The pigmented tumor is seen to occupy the iris interiorly. The anterior chamber is narrowed and the lens is indented (arrows). Pigment flecks are present in the region of the indentation.

VOL. 66, NO. 1 N E O P L A S M S OF IRIS P I G M E N T E P I T H E L I U M 33

Fig. 3 (Morris and Henkind). Low-power view of pigmented mass arising from posterior surface of iris. Note the posterior displacement of the anterior ciliary process (arrow). (Hematoxylin-eosin, x24.)

trichrome, as well as bleached sections, were examined. A well-circumscribed but nonen-capsulated, vascularized, pigmented mass arose from the iris pigment epithelium (fig. 3 ) . The tumor cells were polygonal and con-tained abundant cytoplasm. The pigment consisted of coarse, brown, intracellular granules, varying from scant to heavy in dif-ferent areas. The nuclei stained darkly, were small-to-moderate in size, round or oval, and contained prominent nucleoli. Mitotic fig-ures were rare. The cells were arranged in sheets and cords, interspersed with pseudo-acinar groupings containing nests of deeply pigmented cells within their lumina (fig. 4 ) . Several larger cystlike spaces also contained amorphous eosinophilic material. Numerous medium-to-large-sized thin-walled vessels were present. Pigmented cells of varying size were scattered around vessels. The stroma was scant and Masson's stain re-vealed no myofibrils.

The tumor compressed the iris stroma an-

terior to it, especially toward the periphery, and extended backward toward the posterior chamber. It projected slightly beyond the pu-pillary margin, and peripherally compressed the engorged ciliary processes, which were elongated and conformed to the shape of the tumor. The nonpigmented ciliary epithelium of the anterior ciliary processes had under-gone pressure atrophy. Numerous pigmented cells were present within the trabecular meshwork adjacent to the tumor, but not within Schlemm's canal. The iris stroma an-terior to the tumor, especially toward the en-gorged periphery, contained numerous pig-mented cells of varying size. In contrast, the uninvolved iris was sparsely pigmented.

There were large excrescences of hyper-plastic, darkly pigmented epithelial cells at the pupillary margin on the same side as the tumor, but no connection between these ex-crescences and the tumor could be demon-strated (fig. 5 ) . Similar smaller floccules were seen elsewhere at the pupillary margin.

34 A M E R I C A N JOURNAL OF O P H T H A L M O L O G Y JULY, 1968

Fig. 4 (Morris and Henkind). Higher-power view showing details of tumor. The cells are polygonal with relatively small nuclei, and are arranged in sheets. Multiple cystlike spaces containing pigmented cells ( ? macrophages) are present ( Hematoxylin-eosin, X320.)

Several drusen were present within the tis-sue of the nerve head, appearing as large, ir-regular, basophilic masses anterior to the lamina cribrosa. Scattered throughout the ocular tissues were precipitated biréfringent crystals of mercuric chloride, resulting from primary fixation in Zenker's solution.

The diagnosis was pigment epithelial neo-plasm of the iris, floccules, and drusen of the optic disc.

Fig. S (Morris and Henkind). Prominent ex-crescence of pigment epithelium at pupillary mar-gin on the side of the tumor (Hematoxylin-eosin, XSO.)

CASE 2*

The patient was a 24-year-old white woman. Examination of the involved eye re-vealed a cataract and a pigmented tumor of the anterior segment. A diagnosis of malig-nant melanoma of the ciliary body was made and enucleation was performed.

MICROSCOPIC EXAMINATION

A celloidin section stained with hematoxy-lin and eosin showed a well-circumscribed, nonencapsulated, vascularized and pigmented mass, honeycombed with cystic spaces, arising from the pigment epithelium of the iris (fig. 6 ) . The heavily pigmented tu-mor cells were polygonal in shape, and the lumina of the cysts which they formed often contained amorphous eosinophilic material.

* Courtesy of Professor Norman Ashton, Depart-ment of Pathology, Institute of Ophthalmology, Judd Street, London, England.

VOL. 66, NO. 1 N E O P L A S M S OF IRIS P I G M E N T E P I T H E L I U M 3 5

Fig. 6 (Morris and Henkind). Case 2. Low-power view of cystic pigmented mass arising from iris pigment epithelium. A large cyst is present in the pupillary area. Tumor cells are present within the anterior portion of the ciliary body. A small nest of tumor cells is present in the area of the base of the vitreous. (Hematoxylin-eosin, x20.)

Blood vessels were moderate in size and number. A small, round, pigmented cyst pro-jected from the posterior surface of the tumor. A cyst lined by nonpigmented cuboi-dal cells similar to nonpigmented ciliary epi-thelium was also present at the posterior sur-face of the tumor. The tumor extended from near the periphery of the iris to the pupillary margin where it gave rise, via a bridge of pigmented cells, to a large cyst filled with amorphous eosinophilic material, red blood cells, pigmented cells, and free pigment granules. The tumor bulged into the anterior and posterior chambers ; it did not invade the iris stroma, but compressed it against the posterior corneal surface. The stroma over-lying the tumor contained several scattered darkly pigmented cells. The pars ciliaris at the base of the ciliary processes contained a large pocket of darkly pigmented tumor cells which had no connection with the main tumor mass in the section examined. Anterior to the pars plana and lying free in the area

of the base of the vitreous was a small round plug of tumor cells. We could not determine if this was an artifact of processing, but its position behind the zonules seemed unusual.

The diagnosis was pigment epithelial neo-plasm of the iris.

DISCUSSION

The pigment epithelium of the iris is a continuation of the nonpigmented layer of the ciliary epithelium. It is reflected at the pupillary margin to become continuous with the iris sphincter and dilator muscles.1 The inner layer of the optic vesicle gives rise to the highly differentiated retinal layers as well as to the less complex ciliary and iris epithe-lium. The latter has the potential for giving rise to a wide variety of tumors.

Classification of such tumors can be ex-ceedingly difficult because of the transitional forms which may occur, and because they may be confused with tumors of other deri-vation. Disagreements also arise as to

3 6 A M E R I C A N J O U R N A L O F O P H T H A L M O L O G Y J U L Y , 1 9 6 8

TABLE 1

TUMORS ARISING FROM THE FIGMENT EPITHELIUM OF THE IRIS

Hyperplasia 1. Primary

Congenital a. At pupillary margin b. At margins of colobomata

Acquired a. Region of sphincter; migrating epithelial

cells appear in stroma as clump cells (equivocal origin')

b. Cells can reach anterior surface of iris and proliferate (velvety black in appearance)*

2. Secondary Intraocular inflammation a. Pigmented cells proliferate around the

pupillary margin onto anterior iris surface. Long-standing glaucoma

a. Same as above b. Migration through stroma to anterior

surface at collarette. Trauma (including surgery)

a. Proliferation of pigment epithelium on anterior surface of iris, across pupil, or on posterior surface of cornea.

Strong Cholinesterase« a. Hyperplasia often associated with cystic

formation. Neoplasia

1. Benign Well-differentiated epithelial cells, usually pigmented, often with pseudoacinar arrange-ment and cysts. May have limited locally invasive properties.

2. Malignant Carcinoma* Papillary cystadenoma4

Local invasion, cellular atypia, intraocular metastases'

Medulloepithelioma, embryonal type (dik-tyoma)*'*'7

whether the original site of a particular tumor is the iris or ciliary body. The pig-ment epithelium of the iris can proliferate in response to a variety of insults, and primary and secondary hyperplasias can, on occasion, be confused with neoplasms; transitional forms between hyperplasia and neoplasia also occur. Table 1 classifies tumors of the iris pigment epithelium.

There are few well-documented cases of primary neoplasm of the iris pigment epithe-lium to compare with those presented here. A partial review of the subject appears in the Graefe-Samisch Handbuch.30 Table 2 contains a compilation of the histologically verified cases in the literature. We have not included the case report by Alt 2 1 in 1911 of

an eye which harbored a "choroidal sar-coma" and showed evidence of previous in-flammation. The tumor reported by Funk,2 2

and cited by Laval,17 was omitted because the description and accompanying photo-graphs did not provide sufficient evidence to determine its derivation. Litricin's18 sec-ond case was not included since the tumor consisted of cysts of the epithelium of the iris and ciliary body in an eye with retinal detachment and gliosis. Reese felt that the tumor reported by Hirschberg and Birnbacker* was a diktyoma; one reported by Seefelder2' may also belong to this group (these were not included in the table). Nev-ertheless the table covers a wide spectrum from the minute benign lesion reported by Anagyros1 0 to the large, invasive tumor re-ported by Vrabec and Soukup,5 which per-haps could be classified as a malignant epithe-lioma. We have reservations about some of the tumors listed in the table. The older re-ports, when illustrated, had camera lucida drawings, and more recent reports contained photographs which were often of poor qual-ity. Some of the previously reported tumors resemble cases of melanocytomas of the iris. This diagnosis was not recognized at the time the earlier reports were written.

No statistical analysis is possible because of insufficient data, but the following infor-mation can be recorded : Both sexes are af-fected from the third to the eighth decades, there is no antecedent inflammation, and glaucoma is not a feature. Visual acuity is not usually affected by the tumor. It can ap-pear in any sector of the iris, often toward the periphery. The size varies from 1 to 8 mm. In a few cases, pressure on the lens resulted in local opacification. Histologically, the po-lygonal tumor cells are well-differentiated and contain varying amounts of pigment. They are arranged in sheets and cords, and may have a pseudoacinar arrangement. The stroma is scant, and cysts are common. Vas-cularity is slight to marked. Malignant mela-noma is the most common clinical diagnosis, and the tumors have been treated most com-

VOL. 66, NO. 1 N E O P L A S M S O F IRIS P I G M E N T E P I T H E L I U M 37

monly by iridectomy or enucleation. No deaths have been ascribed to the tumor.

In Case 1 the tumor arose in an eye which contained hyperplastic excrescences of the pigment epithelium at the pupillary mar-gin. The tumor could not be demonstrated arising directly from these excrescences,

but they appeared more florid in the sec-tor containing the tumor than in the unin-völved portion of the iris. The possibility ttyat the cells of the excrescences prolifer-ated secondary to an irritative effect of the tumor appears unlikely.

Drusen of the optic disc were present in

TABLE 2

NEOPLASMS OF THE IRIS PIGMENT EPITHELIUM

P A » T 1 Anagyros» 1903

Stock* 1905

Gilbert" 1910

Gaben» 1921

Age and sex 62/Male 46/Female 67/Female 76/Female

Symptoms and signa

Poor visual acuity 1 yr Decreased vision

Visual acuity Hand motion Normal

Tension Normal See remarks

Laterality O.S. O.D. O.D. O.D.

Location Inferior Nasally behind iris Lower nasal periphery Temporal periphery

Cnnical characteristics

Black-brown tumor of cylindrical shape. AC deep; pupil duaied well

Iris elevated peripher-ally by black tumor. Pupil round and reac-tive

Pigmented spot

Size 1 X2.S mm 3X1 XO.S mm 2 X 2 . 5 X 3 mm 1.5X2.5 mm

Clinical inflammation

Clinical vascularity

Additional clini-cal findings

Several other benign iris nevi. senile cata-ract

Surgical aphakia, sec-ondary glaucoma (see remarks)

Herpetic involvement of cornea

Clinical diagnosis

Benign melanoma Autopsy specimen Retroiridk cyst (?) Autopsy specimen

Treatment Cataract extraction and removal of tumor: enucleation several days later due to post-operative Infection

Observation, eventual enucleation. (See re-marks)

Deeply pigmented Variably pigmented Heavily pigmented cy- Heavily pigmented cells, somewhat smaller large epithelial cells ir- lindrical cells with round or polygonal than pigment epithe- regularly arranged, large nuclei, promi- cells, occasional mi-l i u m . I r r e g u l a r l y palisading at stromal nent nucleoli. Some toses. Closely packed clumped together border cells with hydropic de- in some areas, more

generation. Central loosely arranged in necrosis others

Stroma None, tumor partially encapsulated

None Sparse and delicate mesh work

Present

Microscopic vascularity

Numerous vessels Sparse vessels Present

Cysts Present, multUoculated

Invasive properties

None None None

Follow-up

Remarks Ft. died of tuberculosis Cataract extraction and superior irldec-t o m y p e r f o r m e d shortly after tumor noted. Lesion grew slightly over 1 r-year period; eye enucleated because of secondary glaucoma, blindness, and pain

Author felt congenital defect of pigment epi-thelium probable ate of origin

(Continued on following page)

Type, arrange-ment and pigmen-tation of cefls

38 A M E R I C A N JOURNAL O F O P H T H A L M O L O G Y JULY, 1968

T A B L E 2 (Continued)

P A S T 2 Morar"

1925 Schmidt"

1934 Asbury (Vail)"

1944 Laval»

1952

Age and sex 60/Fern ale 60/Male 45/Female 40/Female

Symptoms and signs

Visual trouble Cataract Black spot seen on iris Slowly enlarging spot on iris—4 months' dura-tion

Visual acuity

1/50 (see remarks) Good 20/20 6/6

Tension Normal Normal — 20 mm

Laterality O.S. — O.D. O.D.

Location Central iris, nearer periphery

Upper quadrant, peri-phery

Upper periphery to 1 mm of pupillary mar-gin

Lower nasal quadrant central iris, nearer peri-phery

Clinical characteristics

Velvet mat Small gray-brown pig-mented spot. Iris pushed forward

Solid nodule shallow-ing AC. Pupil slightly irregular

Black solid mass, ir-r e g u l a r l y r o u n d , s l ight ly e levated , clearly demarcated

Size — Pinhead 3 X 2 mm 2.5 mm

Clinical Inflammation

None — None —


None Two blood vessels lay over mass. Moderately injected superficial con-junctival vessels at limbus directly oppo-site tumor

Additional clinical findings

Circinate retinitis. Lo-cal lens opacity. Sub-jacent to tumor

Cataract Opacification of outer layers of cortex due to pressure of tumor

—

Clinical diagnosis

Melanotic tumor of iris

(?) Malignancy Malignant melanoma Malignant melanoma of iris, well localized

Treatment Enucleation Iridectomy Enucleation Iridectomy

Type arrang-ment and pig-mentation of cells

Heavily pigmented polygonal cells; some arranged in cords; oc-casionally glandular arrangement

Variably pigmented epithelial cells ar-ranged in bands, rods, or sheets in a loose structure

Heavily pigmented cords and groups of well-differentiated cells. Pseudoacinar arrange-ment

Deeply pigmented, closely packed cells resembling pigment epithelium

Stroma Present, small amount Present, with areas of Moderate amount hyaline degeneration


None Plentiful. Hyaline de-generation present in walls of vessels

— —

Cysts Between internal bor-der of tumor and pig-ment epithelial layer

Small cysts Present on free margin and containing fluid and melanin-bearing macrophages

Invasive properties

Invaded ciliary body None Slender cords and tubes seen in spaces of Fontana, Schlemm's canal and adjacent ven-ous channels; also within anterior ciliary processes

Follow-up — 11 mo without recur-rence

Alive and well after 6 years

—

Remarks Decreased vision due to circinate retinop-athy

Equivocal origin of le-sions, situated between iris and ciliary body. Cf. Zentmayer," Keyes and Moore1*


N E O P L A S M S OF IRIS P I G M E N T E P I T H E L I U M 39


P A R T 3 Litricin» 1961

V ra bee and Soukup* 1963

Reese» 1963

Reese" 1963

Age and sex 46/Male 42/Male 55/Female 43/Female

Symptons and signs

Itching O.D. Blurred vision 1 week Routine examination Enlarging iris lesion—3 months' duration

Visual acuity 1.0 6/6 — — Tension 20 mm. — — Laterality O.S. O.S. O.S. — Location Upper nasal quadrant Scattered over surface,

superiorly 1-1.S mm from pupillary margin

Inferior iris

Pigmented tumor with Yellow-gray tumor Velvety black iris le- Mulberry appearance uneven surface, grow- growing into AC. push- sion, pigment dust on ing into pupillary area. ing iris posteriorly. anterior surface Few other nevi près- Four lobes. Slightly de-sent. Brisk pupillary formed pupil reaction

Size 1.5 mm 7 X3.5 mm — —


— None None —

Clinical — Episcleral vessels up- Ves Yes valscularity — per i of globe enlarged

and tortuous. Superfi-cial and deep vessels running from angle to tumor


— Minute opacification of superior cortex due to pressure of tumor

— —

Clinical diagnosis Melanoma of iris Malignant neoplasm — — Treatment Iridectomy Iridectomy Iridectomy Iridectomy

Type, arrange-ment and pig-mentation of cells

Deeply pigmented densely packed uniform cells with round nuclei.

Main mass irregular tubular structure; heavily pigmented in areas, but not in cen-tral mass. Superficial layer of tumor contain-ed fusiform cells

Heavily pigmented epi-thelial cells embedded in iris stroma. "Iceberg type."

Pigmented epithelial cells growing in an adenomatous arrange-ment.

Stroma None None — — Microscopic vascularity

Few, small vessels Present Present Present

Cysts — Present in lower part of some lobes

— —

Invasive properties

None Through peripheral 1 of iris stroma, into AC and ciliary body. Some globular particles bud from tumor, float in AC penetrate to iris root inferiorly

Follow-up Alive and well after 3$ years

— — —

Remarks Author considered tu-mor a hyperplasia

Author's pathologic di-agnosis, malignant tu-mor of pigment epithe-lium



40 A M E R I C A N JOURNAL O F O P H T H A L M O L O G Y JULY, 1968


P A R T 4 Ash ton" 1964 Ashton" 1964 Our Case 1 Our Case 2

Age ft sex 36/Female 39/Male 32/Male 24/Female

Symptoms ft signs

Noted from birth Present 15 years Noted on routine ex-amination

Symptomatic for 8 mo

Visual acuity — 20/20 — Tension — 11 mm — Laterality O.S. O.D. O.D. — Location • • Inf ero temporally from

periphery to pupillary margin

—

Large nontransillumin- Pigmented tumor of ating globular dark- anterior segment gray tumor projecting into AC and PC vis-ible beyond pupillary margin. Pupil rigid in tumor area

Size — — 8-mm diameter 4X2.5 mm


— — Mild flare with occa- — slonal cells


Large irregular blood — vessels coursed over iris surface above the tumor

Floccules at pupillary Lens cataractous margin. Pigment on lens. Anterior cortical opacification of lens subjacent to tumor; PSC opacity

Clinical diagnosis

— Malignant melanoma of ciliary body

Malignant melanoma of ciliary body

Treatment Iridectomy Iridectomy Enucleation Enucleation

Type, arrange-ment and pig-mentation of cells

Large heavily pigment-ed epithelial cells

Large heavily pigment-ed epithelial cells

Variably pigmented po-lygonal epithelial cells arranged In sheets and cords with interspersed pseudoacinar groupings

Heavily pigmented po-lygonal cells forming cysts

Stroma — — Scant Scant


— Numerous moderate-to-large thin-walled. vessels

Blood vessels moderate in size and numbers

Cysts Present, containing amorphous eosinophilic material

Numerous, containing amorphous eosinophilic material. One cyst ex-tremely large

Invasive properties

— None Invasion of ciliary body on same side as tumor

Follow-up Alive and well after 11 years

Alive and well after 11 years — —

Remarks — — Drusen of optic disc —



the first case. These bodies, which are unre-lated to corpora amylacea or to the drusen of Bruch's membrane, are laminated, acellular, basophilic deposits of varying sizes and shapes. They are found anterior to the lam-ina cribosa within the substance of the disc, and rarely reach the surface of the nerve head."'*5 We can find no previous report of

drusen of the disc associated with a tumor of the iris pigment epithelium.

The tumor in the second case was inva-sive, as evidenced by the presence of tumor cells in the ciliary body, whereas the neo-plasm in the first case was large and nonin-vasive, causing intraocular damage by pres-sure on adjacent structures. It could not be

VOL. 66, NO. 1 N E O P L A S M S OF IRIS P I G M E N T E P I T H E L I U M 41

differentiated clinically, however, from a ma-lignant melanoma of the ciliary body with invasion of the iris, or melanoma of the iris with invasion of the ciliary body. The fol-lowing features, present in the first case, have also been described in cases of anterior segment melanomas: pigmentation; dilated blood vessels running over or into the tumor; sluggishness of the pupil in the in-volved sector; deposition of pigment in the trabecular meshwork; projection of the tumor into the anterior and posterior cham-bers ; and failure to transilluminate.

Several authors have emphasized that me-lanomas of the iris are relatively be-nign1 7 ' 1 9 ' 2 8 ' 2 7 and that a conservative thera-peutic approach is more warranted than in other uveal melanomas. It becomes clear from the cases presented here, as well as from the report of Ferry,2 8 that the clinical diagnosis of malignancy cannot always be verified his-tologically. This fact lends further sup-port to those who favor a conservative ap-proach.

550 First Avenue (10016)

AKNOWLEDGMENTS

W e would like to thank Dr. Marianne Wolff and Mrs. Cora Assali for assistance with translations, Dr. Robert Bart for critical analysis, Mr. Henrick Malpica for the photographs, and Miss Doris Pagan for secretarial assistance.

REFERENCES

1. Duke-Elder, S. and Cook, C. : System of Oph-thalmology. London, Kimpton, 1963, vol. 3, pt. 1, p. 106.

2. Collins, E. T. : Intra-ocular melanomata. Tr. Ophth. Soc. U.K. 46:86, 1926.

3. Reese, A. B. : Tumors of the Eye. New York, Harper and Row, Hoeber Med. Div., 1963, ed. 2, p. 70.

4. Erôss, S., Orbán, T., Kálló, A. and Hanisch, J. : Simultaneous occurrence of iridal metastasiz-ing papillary cystadenoma and heterolateral pal-pebral recurring tumor of identical structure. Oph-thalmologica 149:27, 1965.

5. Vrabec, Fr. and Soukup, Fr. : Malignant epithe-lioma of the pigment epithelium of the human iris. Am. J. Ophth. 56:403, 1963.

6. Hirschberg, J. and Birnbacker, A. : Schwamm-

Krebs der Iris-Hinterschicht. Zbl. Augenh. 20:289, 1896.

7. Duke-Elder, S. and Perkins, E. S. : System of Ophthalmology. London, Kimpton, 1966, vol. 9, p. 790.

8. Anargyros, E. : Melanom der Iris. Arch. Au-genh. 46:62, 1903.

9. Stock, W . : Ein epitheliarer Tumor der Iris, vom hintern Pigmentepithel ausgehend. Klin. Mb. Augenh. 43:503, 1905.

10. Gilbert, W . : Ueber Zysten und Geschwulst-bildung des Pigmentaepithels der Iris. Klin. Mb. Augenh. 48:149, 1910.

11. Gilbert, W . : Ueber Pigmentanomalien des Auges. Arch. Augenh. 88:143, 1921.

12. Morax, V . : Epithelioma pigmenté et sar-come de l'ris. Bull. Soc. Ophtal. France 1925, p. 102.

13. Schmidt, R. : Ein epithelialer Tumor der Iris. Arch. Augenh. 108:457, 1934.

14. Asbury, M. K. : Epithelial tumors of the iris. Am. J. Ophth. 27:1094,1944.

15. Zentmayer, W . : A case of hyperplasia of the epithelium of the ciliary processes (ciliary adenoma of E. T. Collins). Arch. Ophth. 16:677,1936.

16. Keyes, J. E. L. and Moore, P. G. : Adenoma-tous hyperplasia of the epithelium of the ciliary body. Arch. Ophth. 19:39, 1938.

17. Laval, J. : Benign pigment epithelium tumor of the iris. Arch. Ophth. 48:66,1952.

18. Litricin, O. : Zur Frage der Hyperplasie und der Geschwülste des Irispigmentepithels. Klin. Mb. Augenh. 139:249, 1961.

19. Ashton, N. : Primary tumors of the iris. Brit. J. Ophth. 48:650,1964.

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neoplasms of the iris pigment epithelium

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