RECURRENT ORBITAL MALIGNANT MELANOMA AFTER THEEVISCERATION OF AN UNSUSPECTED CHOROIDAL MELANOMA

ROBERT A. LEVINE, M.D., ALLEN M. PUTTERMAN, M.D.,AND MICHAEL S. KOREY, M.D.

Chicago, Illinois

The extension of a choroidal melanomainto the orbit is usually associated with arapidly fatal outcome. We describe a man,currently well, who underwent an exen­teration for an orbital melanoma 15 yearsafter an evisceration for an unsuspectedmelanoma of the choroid.

CASE REPORT

In 1964, a 47-year-old man complained of pain ofthree days' duration in his blind left eye. Examina­tion of this eye showed a nonreactive pupil, posteri­or synechiae, cataract, and intraocular pressure of81 mm Hg. The left fundus could not be visualized.The right eye was normal. The ocular history andsystem review were unremarkable. Because of painand the failure of the glaucoma to respond to medi­cal therapy, the patient was hospitalized and the eyewas eviscerated. The pathologist reported that theeviscerated specimen consisted of "inflammationand hemorrhage."

The patient wore a prosthetic ocular shell withoutdifficulty until 1976, at which time he noted swell­ing of his left eyelids, enlargement of the left orbitaltissues, and frequent, spontaneous extrusion of theocular prosthesis. In February 1978, the swollen anderythematous left eyelids were displaced anteriorlyby a black, firm, and immovable orbital mass(Figs. 1 and 2).

Radiographically, the left orbit appeared slightlydeformed and expanded in its transverse diameter(Fig. 3). The enlargement of the orbital cavity in theabsence of invasion of the orbital bones suggestedthe presence of a slow-growing neoplasm. By com­puted axial tomography, the left orbital tumorshowed definite enhancement with contrast media(Fig. 4). No intracranial extension of the tumor wasnoted.

From the Goldenberg Eye Pathology Laboratory(Dr. Levine) and the Department of Ophthalmology,Michael Reese Hospital and Medical Center (Drs.Levine, Putterman, and Korey); and the Universityof Illinois Eye and Ear Infirmary (Drs. Levine andPutterman), Chicago, Illinois. This study was sup­ported in part by a grant from the National Institutesof Health, Public Health Service Grant EY 1792.

Reprint requests to Allen M. Putterman, M.D., IIIN. Wabash, Chicago, IL 60602.

Fig. 1 (Levine, Putterman, and Korey). Patient'sswollen left eyelids displaced anteriorly by orbitalmass.

A-scan ultrasonography showed a left orbitaltumor of extremely low acoustic reflectivity withill-defined borders (Fig. 5). These acoustic findingswere most consistent with the lymphoma-sarcomagroup of orbital tumors.

In February 1978, the left orbit was exploredsurgically. The black-brown orbital tumor was re­moved at a biopsy and frozen sections showed thepresence of a malignant melanoma. During exenter­ation of the left orbit, the floor, medial wall, and roofof the orbit were found to be paper thin. In dissect­ing periosteum, two small penetrating areas werecreated in the floor and medial walls. Upon severingthe apex of the exenterated specimen, we found that

Fig. 2 (Levine, Putterman, and Korey). Pigmentedorbital tumor, exposed at time of surgery.

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572 AMERICAN JOURNAL OF OPHTHALMOLOGY APRIL, 1980

Fig. 3 (Levine, Putterman, and Korey). Transverseexpansion of left orbit without bony invasion inconventional orbital x-ray film.

tumor tissue had been transsected. The remainder ofthe tumor in the apex of the orbit was removed pieceby piece until all visible residual tumor had beeneliminated from the orbit.

Postoperatively, the left orbit healed in a normalfashion (Fig. 6). An examination by the oncologyservice, including liver-spleen/gallium and bonescans, showed no evidence of metastatic disease.Because of the possibility of residual tumor in theorbit, the left orbit was irradiated; 2,000 neutronrads were delivered in eight divided treatments. Thepatient also received bacillus Calmette-Guerinimmunotherapy. When last seen, 14 months after the

Fig. 5 (Levine, Putterman, and Korey). A-Scanultrasonography, showing low acoustic reflectivity,compatible with lymphoma-sarcoma group of tu­mors.

Fig. 4 (Levine, Putterman, and Korey). Computedaxial tomogram showing tumor involving and ex­panding entire left orbit.

exenteration, the patient had no evidence of localrecurrence or of metastatic disease.

Sections obtained from the eviscerated specimenof 1964 were examined. These sections showed thepresence of a spindle-cell melanoma (Fig. 7), pre­sumably of choroidal origin. The orbital recurrencewas also of the spindle-cell variety (Fig. 8).

DISCUSSION

The presence of an intraocular melano­ma should be suspected in any white

Fig. 6 (Levine, Putterman, and Korey). Patientseven months after left orbital exenteration andradiation treatment.

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Fig. 7 (Levine, Putterman, and Korey). Review of1964 evisceration specimen revealed presence of aspindle-cell melanoma, presumably originatingwithin the choroid. Elongated cells with fusiformnuclei are present.

person over age 40 years whose visualloss is associated with unilateral glauco­ma and cloudy media.t-f Four percent ofblind eyes with opaque media containunsuspected choroidal melanomas.' In­tractable glaucoma invariably promptsthe enucleation of these eyes. Microscop­ic examination of enucleated specimenshas shown that as high as 10% of cho­roidal melanomas are clinically unsus­pected."

In our case, the classic findings thatshould have aroused the suspicion con­cerning intraocular melanoma were either

Fig. 8 (Levine, Putterman, and Korey). Exentera­tion specimen shows densely pigmented melanomacells of the spindle-cell variety.

discounted or ignored. Additionally, mak­ing recurrence or metastasis more likely,an evisceration was done. An early deathfor this patient should have been antici­pated for at least two reasons. First, if theoperative manipulation of a melanoma­containing eye fosters early dissemina­tion, certainly evisceration of such an eyewould further enhance the likelihood ofmetastasis.v f Instances of death frommetastatic melanoma after the eviscera­tion of an unsuspected intraocular mela­noma have occurred.t-" Second, the pres­ence of orbital recurrence or extrascleralextension is associated with an extremelyhigh mortality. In the Armed Force Insti­tute of Pathology series," the five-yearmortality in their cases with extraocularextension was nearly 70%, emphasizingthe rapidly lethal clinical course thatcharacterizes recurrent orbital melanoma.

What then accounts for our patient'ssurvival 15 years after the evisceration ofan unsuspected melanoma and develop­ment of a bulky orbital recurrence thatnecessitated orbital exenteration? Report­ed instances of recurrent orbital melano­ma occuring ten,10 13,11 24,12 and 28 13

years after enucleation indicate that thepresence of an orbital melanoma does notinvariably cause a rapidly fatal outcome.Immunologic factors, such as the pres­ence of tumor-associated antibodies, mayprevent the spread and growth of metasta­sis. A more tangible explanation, in ourcase, is the absence of epithelioid cellswithin the tumor. A favorable biologicbehavior of spindle-cell melanoma hasbeen emphasized by Gass.14Our case alsosupports Cass's contention that spindlecells and epithelioid cells are distinctclones of cells, and that spindle cellsseldom dedifferentiate into epithelioidcells.

One might avoid evisceration of an eyecontaining an unsuspected melanoma.We believe that the evisceration proce­dure probably did permit the develop-

574 AMERICAN JOURNAL OF OPHTHALMOLOGY APRIL, 1980

ment of an orbital recurrence. Yet, despitethe surgical manipulation of the tumorand the orbital recurrence, the patient isstill alive, largely because of the apparentcytologic benignity of this melanoma.

SUMMARY

Fifteen years ago a 47-year-old manunderwent an evisceration for an un­suspected choroidal melanoma. A bulkyrecurrence required exenteration. The pa­tient is currently alive and has no evi­dence of metastatic disease.

REFERENCES

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3. Kirk, H. Q., and Petty, R. W.: Malignant mela­noma of the choroid. A correlation of clinical andhistopathologic findings. Arch. Ophthalmol. 56:843,1956.

4. Zimmerman, L. E., McLean, 1. W., and Foster,W. D.: Does enucleation of the eye containing amalignant melanoma prevent or accelerate the dis­semination of tumor cells? Br. J. Ophthalmol.62:420, 1978.

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7. Starr, H. J., and Zimmerman, L. E.: Extra­scleral extension and orbital recurrence of malig­nant melanomas of the choroid and ciliary body. Int.Ophthalmol, Clin. 2:369, 1962.

8. MacDonald, R., Jr., and Edwards, W. D.: Mela­noma of the orbit. An interesting case followingevisceration. Surv. Ophthalmol. 12:253, 1967.

9. McLean, I. W., Foster, W. D., and Zimmer­man, L. E.: Prognostic factors in small malignantmelanomas of choroid and ciliary body. Arch.Ophthalmol, 95:48, 1977.

10. Reese, A. B.: Tumors of the Eye, 3rd ed. NewYork, Harper and Row, 1976, p. 210.

11. Newton, F. H.: Local recurrence of melanomaof the choroid 13 years after enucleation. Am. J.Ophthalmol. 21:668, 1938.

12. Saunders, D. H., Rodriques, M. M., andShannon, G. M.: Orbital recurrence of malignantmelanoma of the choroid 24 years after enucleation.Ophthalmic Surg. 8:31, 1977.

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14. Gass, J. D. M.: Problems in the differentialdiagnosis of choroidal nevi and malignant melano­mas. The XXXIII Edward Jackson Memorial Lec­ture. Am. J. Ophthalmol. 83:299, 1977.