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This report analyzes 92 malignant melanoma cases from a series of 12,383 consecutive autopsies. There were 12 cases of primary ocular malignant melanoma, 76 cases of primary nonocular malignant melanoma, and 4 cases of unknown primary site. Subjects with malignant melanoma were characterized by age, sex, and race; metastatic disease patterns as well as survival periods were analyzed. In the 12 ocular malignant melanoma cases, tumor size, cell type, and survival were considered. Discussion is concerned with the distribution of primary tumor site, the location of metastatic lesions secondary to malignant melanoma, and survival in cases with primary ocular malignant melanoma.

A case of metastatic tapioca melanoma of the iris in a 12-year-old girl is reported. The patient had heterochromia, a red painful eye, and was treated for iritis with secondary glaucoma. In the course of 5 months iris lesions with the clinical appearance of tapioca pudding developed, and biopsy disclosed a melanoma. The eye was immediately enucleated, and pathological examination showed a melanoma with predominantly epithelioid-type cells which had infiltrated the angle, the posterior chamber, and the surgical wounds. Conjunctival extension was noted 10 months after enucleation, and regional lymph node metastases were found 4 months later. Previously reported cases are reviewed and compared with the present case.

The authors sought S-100 protein in sections cut from formalin- or glutaraldehyde-fixed, paraffin-embedded tumors from the eye and other sites by an immunoperoxidase technique, using rabbit antibovine S-100 protein antiserum, swine antirabbit serum, a peroxidase-antiperoxidase preparation, and amino-ethylcarbizole as developer. Tissue from 26 intraocular tumors was examined, the pathologic diagnosis being unknown to the investigators at that time that the tests were performed. Thirteen of 16 malignant melanomas of the choroid contained S-100 protein (81%), while only one of seven retinoblastomas contained S-100 protein (14%). One cutaneous malignant melanoma metastatic to the eye contained S-100 protein, while none of three metastatic nonmelanocytic, nonneural tumors contained the protein. These results are similar to our findings with nonocular tumors, in which 56 of 56 cutaneous melanomas contained S-100 protein (100%) and only 1 of 51 nonneural, nonmelanocytic tumors contained S-100 protein (2%). S-100 protein may be useful as a marker for ocular malignant melanoma.

A case of isolated optic nerve metastasis from breast carcinoma is described. Intraocular tumor was discovered three months following a radical mastectomy and was confined to the optic nerve head and distal optic nerve. Following X-irradiation treatment, central retinal vascular occlusion, rubeosis, and neovascular glaucoma developed during the fifteen months before enucleation. The clinicopathologic features of this case are discussed and the literature reviewed.

S-100 protein is a 21,000 dalton acidic calcium-binding protein present in ocular melanomas and some normal ocular tissues. Ocular fluids and extracts of ocular tumours were examined by a sensitive radioimmunoassay that could detect less than 5 ng of S-100 protein in minute volumes of fluid. Three ocular melanoma biopsy specimens had S-100 protein at levels between 25 and 1300 ng/ml, comparable to that found in a cutaneous melanoma biopsy specimen (1000 ng/ml). (SI conversion: ng/ml = microgram/l.) Six melanoma culture lines had 1000 to 125,000 ng/ml. Four lymphoblastoid cultures had less than 2 ng/ml, and three colon carcinoma cultures had 180 ng/ml. Subretinal fluid from 23 melanoma-containing eyes had 10 to 76,800 ng/ml. Lesser amounts were found in eyes with small, anteriorly located, lightly pigmented tumours. Vitreous from 3 melanoma-containing eyes had 10,000 to 11,000 ng/ml. Vitreous obtained from three eyes during tractional retinal detachment repair had 500 to 1600 ng/ml, and vitreous obtained at necropsy from six normal eyes had 2 to 120 ng/ml. Aqueous from six melanoma-containing eyes had 10 to 30 ng/ml, levels not significantly different from those observed in three normal eyes (80-120 ng/ml). This approach provides new insight into the interaction of ocular tumours and adjacent ocular fluids and may, with more specific tumour markers, have diagnostic applications.

Vitreous cylinders, a rarely discussed ophthalmologic entity, were studied in two clinical cases and in five enucleated eyes. The cylinders were found in four cases of ocular toxoplasmosis, one case of vitreous hemorrhage complicating a malignant melanoma, and one case of pars planitis and one of uveitis, both of unknown causes. Results of scanning electron microscopy in one specimen suggested that cylinders are formed by condensation of vitreous collagen fibers. We emphasize the nonspecific nature of this interesting vitreous finding.

The incidence and morphologic features of coronal adenomas, benign epithelial tumors of the ciliary processes, were studied in 500 autopsy cases. Macroscopically, coronal adenomas are white, globular, often cystic in appearance, of limited growth potential, and found only on the ciliary processes. Microscopically, they contain convoluted sheets or tubes of nonpigmented epithelium, between which are found varying amounts of amorphous eosinophilic, PAS-positive, extracellular material. Coronal adenomas were present in 153 (31%) of 500 cases, were bilateral in 23 (15%), and were thus present in 176 (18%) of the 1,000 eyes examined. Two clinically important complications of coronal adenomas were found, sectoral cataracts and misdiagnosis as iris tumor.

Corneal intraepithelial neoplasia (CIN) is the term applied by some authors to the spectrum of disease ranging from mild dysplasia to carcinoma in situ. Such lesions usually are associated with dysplastic or neoplastic processes at the limbus or adjacent conjunctiva; isolated corneal dysplasia is rare. Clinically, CIN appears as a geographic, gray, translucent thickening of the epithelium with fimbriated or scalloped borders and lesions often contain scattered white dots. We report a case of intraepithelial neoplasia limited to the cornea that had the unusual clinical appearance of a white plaque, which prompted the misdiagnosis of a calcific scar. Histopathologic examination of the debrided tissue revealed intraepithelial neoplasia and marked hyperkeratosis as the cause of the opacification.

The clinicopathologic features of retinal or optic disc astrocytomas are discussed based on eighteen reported cases. In addition, a new case of solitary retinal astrocytoma in a patient without other stigmata of phakomatosis is described. The tumor was prominently vascular and caused total exudative retinal detachment. It doubled in diameter over the seven months prior to enucleation, which was dictated by the occurrence of neovascular glaucoma. The astrocytic nature of the lesion was confirmed by immunohistochemical techniques (glial fibrillary acidic protein stain) and by electron microscopy.