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Expression of developmentally defined retinal phenotypes in the histogenesis of retinoblastoma.

Retinoblastoma, the most common intraocular tumor of childhood, is a malignant neoplasm that arises during retinal development. The embryonal cell target for neoplastic transformation is not yet clearly defined. To better understand the histogenetic potential of this tumor, the expression of photoreceptor and glial cell-associated proteins were examined in 22 primary retinoblastomas. Interphotoreceptor retinol-binding protein (IRBP), cone and rod opsins were selected as the photoreceptor specific proteins due to their different temporal patterns of expression during normal retinal development. Neoplastic Müller cell differentiation, and non-neoplastic reactive astrocytes were identified using cellular retinaldehyde binding-protein (CRAlBP), and glial fibrillary acidic protein (GFAP), respectively. Photoreceptor proteins were present in 16 cases and showed different cellular patterns of expression. IRBP and cone opsin were usually abundant. Although rod opsin was clearly identified in eight tumors, its expression was more restricted than either IRBP or cone opsin. This differential pattern of expression, opposite to the normal pattern of photoreceptor gene expression in the adult retina, corresponded to a marked decrease in mRNA for rod opsin. Cone opsin and IRBP colocalized in fleurettes demonstrating that neoplastic human cone cells are capable of IRBP synthesis. Müller cell differentiation was present in 12 of the 16 cases in which photoreceptor proteins were detected. In contrast, GFAP was only present in reactive, stromal astrocytes associated with blood vessels. Our data suggest that the retinoblastoma has the histogenetic potential of the immature neural retinal epithelium which can give rise to both photoreceptor and Müller cell lineages. The differential expression of cone and rod phenotypes in retinoblastoma is consistent with the "default" mechanism of cone cell differentiation.

Blotting, Northern↗

Solitary retinal astrocytoma in a dog.

An intraocular mass from a 13-year old Husky-mix dog was diagnosed as retinal astrocytoma. The mass arose from the ganglion layer of the retina and occupied 50% of the vitreous space. The mass was immunoreactive for neuron-specific enolase, S-100, vimentin, and glial fibrillary acidic protein. The neoplasm had characteristics similar to solitary retinal astrocytomas of humans but lacked the marked vascularity.

Animals↗

Adenocarcinoma arising from congenital hypertrophy of the retinal pigment epithelium.

BACKGROUND: Adenocarcinoma of the retinal pigment epithelium (RPE) is a rare primary malignant intraocular neoplasm. We report a histopathologically confirmed case of adenocarcinoma of the retinal pigment epithelium arising from congenital hypertrophy of the retinal pigment epithelium (CHRPE). METHODS: The clinical features, surgical management, and histopathological features of a melanotic tumor arising from CHRPE are presented. The tumor was excised by transcleral resection. RESULTS: Histopathological and immunohistochemical study of the tumor showed it to be an adenocarcinoma of the RPE. CONCLUSIONS: Adenocarcinoma of the RPE arising from CHRPE is extremely rare. Such a tumor can resemble a choroidal melanoma. In spite of the rarity of this association, periodic monitoring of CHRPE for development of a nodular tumor of the RPE is probably warranted.

Adenocarcinoma↗

Sebaceous gland carcinoma of the eyelid.

We diagnosed sebaceous gland carcinoma of the right lower eyelid in an 82-year-old man. Computed tomography showed the lesion extending from the eyelids anteriorly to the optic foramen posteriorly. With this orbital neoplasm, was an occlusion of the central retinal artery in his right eye. The right orbit was removed surgically and biopsy of the optic foramen region indicated malignant cells. Localized radiation to the right orbit was then initiated postoperatively.

Aged↗