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A Transgenic Mouse Model for Trilateral Retinoblastoma
Joan M. O'Brien, MD;
Dennis M. Marcus, MD;
Rene Bernards, PhD;
James L. Carpenter, DVM;
Jolene J. Windle, PhD;
Pamela Mellon, PhD;
Daniel M. Albert, MD
Arch Ophthalmol. 1990;108(8):1145-1151.
Abstract
• We present a murine model of trilateral retinoblastoma. Ocular retinoblastoma and central nervous system tumors are observed in a line of mice formed by the transgenic expression of SV40 T-antigen. An oncogenic protein known to bind to the retinoblastoma gene product (p105-Rb) is specifically expressed within retinal cells in this model. All animals that carry this genetic alteration develop multifocal retinal tumors. Midbrain tumors are observed in 15% of ocular tumor-bearing animals, and these arise ventral to the cerebral aqueduct at the level of the pineal gland. Both ocular and central nervous system neoplasms are heritable in heterozygous offspring through 10 sequential generations of breeding. Retinal tumors display the gross appearance, invasive properties, light and electron microscopic features, and immunohistochemical staining characteristics of human retinoblastoma. The light and electron microscopic characteristics as well as immunocytochemical features of undifferentiated midline central nervous system neoplasms further correlate with human trilateral retinoblastoma. We postulate an alternative mechanism of retinoblastoma tumorigenesis that involves functional inactivation of retinoblastoma protein locally in the face of an intact retinoblastoma gene locus.
Author Affiliations
From the David G. Cogan Eye Pathology Laboratory, Department of Ophthalmology, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston (Drs O'Brien, Marcus, and Albert); Massachusetts General Hospital, Cancer Center, Charlestown (Dr Bernards); Department of Pathology and Medicine, Angell Memorial Hospital, Boston (Dr Carpenter); and the Salk Institute, La Jolla, Calif (Drs Windle and Mellon).
Footnotes
Accepted for publication April 23, 1990.
This material was presented at the American Ophthalmological Society, Hot Springs, Va, May 21-24,1989, and will be published in 1990 in an expanded form in Transactions of the American Ophthalmological Society.
Reprint requests to Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114 (Dr Albert).
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