Intraocular extension of a malignant melanoma of the conjunctiva is a rare entity. A 75-year-old woman underwent repeated surgery after receiving the diagnosis of a multilocular recurrent malignant melanoma arising from a primary acquired melanosis. Treatment included 2 lamellar sclerokeratectomies and percutaneous radiotherapy. Five years after initial surgery, intraocular extension of the melanoma was observed, and enucleation was performed. Findings from histopathological examination revealed a malignant melanoma occupying part of the ciliary body, the trabecular meshwork, and the iris. Eyes with recurrent malignant melanoma of the conjunctiva should be carefully monitored for intraocular extension. Deep excision of conjunctival melanoma, including lamellar sclerokeratectomy, may abolish the natural barrier against intraocular extension of malignant melanomas of the conjunctiva.Primary malignant melanomas of the conjunctiva arise from primary acquired melanosis (75%), melanocytic conjunctival nevi (20%-30%), or de novo.The overall tumor-related mortality rate is about 25%,and multilocular tumors especially tend to produce lymphatic metastasis.However, intraocular extension of a malignant melanoma of the conjunctiva is rare.We report a case of a 75-year-old woman with intraocular extension of a recurrent multilocular malignant melanoma of the conjunctiva.REPORT OF A CASEA 75-year-old woman was seen at the Department of Ophthalmology of the University of Erlangen-Nürnberg, Erlangen, Germany. She had of a recurrent multilocular malignant melanoma of the conjunctiva of the left eye (Figure 1). The tumor originated from primary acquired melanosis and involved the limbal, caruncular, and forniceal conjunctiva. Since June 1989, 10 excisional biopsies (sometimes multiple) of conjunctival tumors (Figure 2) had been performed (8 at our institution; 2, elsewhere), including 2 lamellar sclerokeratectomies with full-thickness corneal transplants (February 1990 and October 1993) (Table 1). The deep histopathological margins appeared to be free of tumor cells in both specimens. Additionally, the patient received adjuvant percutaneous radiotherapy with a total radiation dosage of 175 Gy. In May 1994, the patient returned with a nonpigmented conjunctival tumor of the left eye and reported ocular inflammation for 4 weeks. Visual acuity was 20/200 OS, intraocular pressure measured 9 mm Hg, and there were pronounced dry-eye symptoms. On examination, we observed 2 slightly pigmented and prominent tumors of the conjunctiva next to the limbus at the 4- and 7-o'clock positions. The corneoscleral suture from the 4- to 7-o'clock position was tight, the transplant showed stromal vascularization, and the recipient cornea was clear. The anterior chamber was deep and showed light cellular infiltration with Tyndall phenomenon. There was iridal neovascularization originating from the chamber angle at the 6-o'clock position reaching to the pupillary margin. Gonioscopically, brownish-pigmented areas were seen inside the chamber angle from the 5- to 7-o'clock position (Figure 3). The lens showed a nuclear and cortical cataract, the retina was attached, the optic disc appeared normal, and there were some irregularities of the retinal pigment epithelium in the macular region. Because of the suspicion of a recurrent conjunctival malignant melanoma with intraocular extension, a modified enucleation was performed.Figure 1.Clinical photograph of the left eye (October 1991) after sclerokeratoplasty with multilocular recurrence of malignant melanoma of the conjunctiva at the 2- and 6-o'clock positions and from the 7- to 9-o'clock position.Figure 2.Histopathological appearance of conjunctival biopsy specimen (February 1990) with uniform-looking pigmented cells spread within the epithelium and the subepithelial tissue (hematoxylin-eosin, original magnification ×480).Clinical and Histopathological Findings From Biopsy Specimens of Conjunctival Tumors*DateConjunctival FindingsIntraocular FindingsTreatmentClinicalHistopathologicalJun 1989Pigmented tumor (2- to 6-o'clock position)MM. . .Tumor excisionJul 1989Pigmented tumor (2- to 5-o'clock position)Scarring conjunctiva with inflammation. . .Tumor excisionFeb 1990Multiple pigmented tumors (4- to 7-o'clock position) involving plica and limbus (6- to 8-o'clock position)MM with acquired melanosis. . .Lamellar keratectomy and sclerectomy with tectonic lamellar keratoplastyJul 1990Pigmented tumors involving the fornix (5- to 8-o'clock position)MM with acquired melanosis. . .Tumor excision, radiotherapy, 45 GyMar 1991Multiple pigmented tumors (12- to 3.5- and 5- to 8-o'clock position)MM with acquired melanosis. . .Tumor excision, radiotherapy, 40 GyOct 1991Multiple pigmented tumors (2-, 6-, and 8-o'clock position)MM. . .Tumor excision, radiotherapy, 44 GyApr 1992Multiple pigmented tumors (5- to 7.5-o'clock position)MM with acquired melanosis. . .Tumor excisionOct 1992Pigmented tumor involving the limbus (4- to 7-o'clock position)MM with acquired melanosis. . .Tumor excision, radiotherapy, 46 GyJun 1993Pigmented tumor (6-o'clock position)MM with acquired melanosis. . .Tumor excision with cryotherapyOct 1993Pigmented tumor (3- to 6- to 8-o'clock position)MM with acquired melanosis. . .Lamellar keratectomy and sclerectomy with lamellar sclerokeratoplastyMay 1994Multiple pigmented tumors (4- and 7-o'clock position)MM with acquired melanosisInflammation, pigmented tumorModified enucleation*MM indicates malignant melanoma; ellipses, none.Figure 3.Gonioscopical photograph of the left eye (May 1994) showing the chamber angle from the 5- to 7-o'clock position with neovascularization on the iris surface and in the chamber angle, with brownish-pigmented areas inside the chamber angle.HISTOPATHO
JAMA Ophthalmology – American Medical Association
Published: Apr 1, 2000
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